ORTHOPEDICS

e d i t e d b y

Mikl6s Szendr6i

Semmelweis Publisher
�

@ www.semmelweiskiado.hu

B u d a p e st , 2008
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Editor and authors

Instru ctor in orthopaedi cs : Pro! Tibor Vizkelety, M.D., Ph.D . , D . S c

Orthopaedi c Department
Semmelweis University, Budapest
Pro! Kalman Szepesi, M.D., Ph.D., D.S c
Orthopaedi c Depar tment
University of Debre cen, Hungary
Language master: Dr. David Durham
Translator: Dr. Andras Vajda, M.D.
Orthopaedi c Department
Semmelweis University, Budapest
George Szoke M.D., M . S c. (Oxon), Ph.D.
Head of Pediatri c Orthopaedi c Department
Department of Orthopaedi cs
Semmelweis University, Budapest
Tamas de Jonge M.D., Ph.D.
Head of Orthopaedi c Department
Markusovszky Hospital, Szombathely, Hungary
Photographer Peter Kovacs
Orthopaedi c Department
Semmelweis University, Budapest

. © Mikl6s Szendroi, 2008

ISBN 978 963 9656 93 2

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Editor:

Pro! Mikl6s Szendroi, M.D., Ph.D., D.Se.

Head of Orthopaedi c Department
Semmelweis University, Budapest

Authors:

Pro! ArpQd Bellyei, M.D., Ph.D., D. Se.

Orthopaedi c Department
University of Pe cs, Hungary

Pro! Tamas Bender, M.D., Ph.D.

Poly clini c of the Hospitaller Brothers of St. John of God, Budapest

Laszl6 Bucsi, M.D., Ph.D.

Head of Orthopaedi c Department
St. George Hospital, Szekesfeherv ar, Hungary

Associate Pro! Zoltan Csernatony, M.D., Ph.D.

Head of Orthopaedi c Department
University of Debre cen, Hungary

Pro! Laszl6 Hangody, M.D., Ph.D., D.Se.

Head of Orthopaedi c Department
Uzsoki Hospital, Budapest

Pro! Tamas !llb, M.D. , Ph.D., D. Se.

Head of Orthopaedi c Department
University of Pe cs, Hungary

Jeno Kiss, M.D. , Ph.D.

Head of Orthopaedi c Department
St. John Hospital, Budapest

Katalin Kolto, M.D.

Radiologist
Orthopaedi c Department
Semmelweis University, Budapest

Pro! Janos Kranicz, M.D., Ph.D.

Head of Orthopaedi c Department
University of Pe cs, Hungary
Prof. Lajos Kullmann, M .D ., Ph.D.
Medi cal Rehabilitation Institute, Budapest

Associate Pro! J6zsejLakatos, M.D., Ph.D.

Orthopaedi c Department
Semmelweis University, Budapest
Associate Pro! Ferenc Mady, MD., Ph.D.
Orthopaedi c Department
Semmelweis University, Budapest

Associate Pro! Adam Mester, MD., Ph.D.

Radiologist
Radiologi c Department
Semmelweis University, Budapest

Pro! Tamas Meszaros, MD., Ph.D.

Orthopaedi c Department
University of Szeged, Hungary

Pro! Gyula Poor, MD., Ph.D.

Head of Rheumatology Department
Semmelweis University, Budapest

Pro! Miklos Szendroi, MD., Ph.D., D.Sc

Head of Orthopaedi c Department
Semmelweis University, Budapest

Pro! Kalman Toth, MD., Ph.D.

Head of Orthopaedi c Department
University of Szeged, Hungary

Peter Pal Varga, MD.

Head of National Spine Centre, Budapest

Pro! Tibor Vizkelety, MD., Ph.D., D.Sc

Orthopaedi c Department
Semmelweis University, Budapest
 Contents

Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . XVII

1. History and subj e ct of orthopedi cs (Tibor Vizkelety) .

2. Stru cture and development of bones (Zoltcm Csernatony) . 5

2 . 1 . Stru cture of bones . . . . . . . . . . . 5
2 . 1 . 1 . The composition of bone . 5
2 . 1 .2 . The bone tissue . . . . . . 5
2 . 1 .3 . Bones as organs . . . . . 7
2 . 1 .4. The development and growth of bones . 7
2.2. Biologi cal adaptation of bones . . . . . . . 7
2 . 3 . Hormones regulating the bone metabolism . . . . . 9

3. Bone healing after various types of fra ctures (Mikl6s Szendroi) . . . . . . . 11

3 . 1 . Traumati c fra ctures . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
3 .2 . Fra cture healing. Types and stages. Factors influen cing fra cture healing 11
3 . 3 Pathologi cal fra cture healing. Pseudo-arthrosis . 13
3 .4. Pathologi cal fra ctures . 14
3 . 5 . Stress fractures . . . . . . . . . . . . . . . . . 16

4. Examination methods in orthopedi cs (Katalin Kollo, Adam Mester, Tamas Meszaros) 17

4. 1 . Case history (anamnesis) . 17
4.2. Examination. . . . . . . 19
4.2. 1 . Inspe ction . . 19
4.2.2. Palpation . . . 19
4.2 . 3 . Alignment, axial deformities of the limbs. 20
4.2.4. Examination of the j oints . . . . . 20
4.2 . 5 . Range of motion of j oints (ROM) . 24
4.2.6. Assessment of mus cle power . 25
4.2.7. Assessment of mus cle tone . . . . 25
4.2 . 8 . Limb length assessment . . . . . . 25
4.2.9. Measurement of limb circumferen ce 27
4.3 . The standing position and the gait 27
4.3 . 1 . Standing position 27
4 . 3 . 2 . Gait . . . . . . 28
4 .4 . Limping . . . . . . . . . . . 30
4 . 5 . Neurologi cal examinations . 31
4.6. Laboratory tests . 31
4.7. Arthros copy . . 32
4 . 8 . Histology tests . 32
VIII � Co n t e n s

4 .9. Imaging modalities . . . . . . . . . . . . . . . . . . 32

4 . 9 . l . X-ray tests, spe cial X-ray investigations 32
4.9.2. Mus culoskeletal ultrasonography : 35
4 . 9 . 3 . Isotope pro cedures 36
4.9.4. CT s canning . . . . . . . . . . . 37
4 . 9 . 5 . MRI . . . . . . . . . . . . . . . . 38
4.9.6. Invasive and interventional radiology . 38

5. Surgi cal therapy i n orthopedi cs (Tamas Meszaros) . 41

5 . 1 . Mus cle - tendon operations . 41
5 .2 . Fas cia surgery . . . . 42
5 . 3 . Bone operations . . . 42
5 .4. Operations on j oints . 42
5 . 5 . Arthroplasty . . . . . 44
5 . 6 . Operation on nerves . 47

6. Conservative treatment pro cedures (Zoltan Csernatony) . 49

6 . 1 . Immobilization . . . 49
6 . 2 . Mobilization . . . . 51
6 . 3 . Non-weight-bearing . 51
6.4. Irradiation . . . . . . 51
6 . 5 . Laser therapy . . . . 51
6 . 6 . Pharma ceuti cal treatment . 51

7. Physiotherapy (Tamas Bender) 57

7 . l . Exercises . . . . . . . . . 57
7 . 2 . Immobilization, mobilization, exer cises 58
7 . 3 . Massage . . . . . . . . 59
7.4. Ele ctrotherapy . . . . . . . . . . . 59
7 . 5 . Ultrasound treatment . . . . . . . . 60
7 . 6 . Pulsating ele ctromagneti c treatment 60
7 . 7 . High-frequen cy treatment 60
7 . 8 . Cryotherapy . . . . . . . . 61
7 . 9 . Balneotherapy-health spas 61
7 . 1 0. Compresses and pa cks . . 62

8. Manual therapy, acupun cture ( Tamas Bender) 63

9. Amputation - artifi cial limbs (Lajos Kullmann) 65

9 . 1 . Lower limb amputation . . . . . . . . . . . 65
9 . l . l . Indi cations of lower limb amputations 65
9 . l .2 . Surgi cal te chnique of amputations 66
9 . l .3 . Criteria of proper stump . 66
9 . 2 . Lower limb prostheses . . . . . . . 68
9 . 2 . 1 . Temporary prostheses . 68
9.2.2. Final prosthesis . . . 69
9 . 3 . Upper limb amputation . . . . . . 70
9.4. Upper limb prosthesis appli cation 70
9.4. 1 . Cosmeti c prostheses . 71
 Co n t e n s � IX

9.4.2. Me chani cal prostheses . . . . . . . . 71

9.4. 3 . Prostheses powered by external force . 72
1 0. Orthoses, applian ces for mobility (Lajas Kullmann) 73
1 0. 1 . Lower limb orthoses . . . . . . . . 74
1 0. 1 . 1 . Ankle - foot orthoses . . . . . 74
1 0. 1 .2. Knee orthoses . . . . . . . . 74
1 0. 1 .3 . Knee - ankle - foot orthoses . 74
1 0. 1 .4. Hip orthoses . . . . . . . . . 74
1 0. 1 .5 . Hip - knee - ankle - foot orthoses 75
1 0.2. Upper limb orthoses . . . . . . . . 75
1 0. 3 . Spinal orthoses . . . . . . . . . . . . . . . 76
1 0. 3 . 1 . Lumbosa cral orthoses . . . . . 76
1 0. 3 .2 . Thora co-Iumbo-sa cral orthoses 76
1 0. 3 . 3 . Cervi co - thora co - lumbo - sacral orthoses . 77
1 0.3 .4. Cervi cal orthoses . 77
1 0.4. Preventive sport applian ces . 77
1 0. 5 . Walking aids . . . . . . . . 77
1 0. 5 . 1 . Walking sti ck . 77
1 0. 5 . 2 . Crut ch . . . . . 77
1 0. 5 . 3 . Walking frame . 78
1 0. 5 .4. Wheel chair . . . 78

1 1. Orthopedi c shoes and insoles (Kalmim T6th) . 81

1 1 . 1 . Shoes and orthopedi c shoe 81
1 1 .2. Shoes for children . . 82
1 1 . 3 . Shoe and sole insoles . . . 83

1 2. Normal growth, growth disturbances (Kalman T6th) . 85

1 2 . 1 . Physiologi cal growth . . . . . . . . . . . . . . 85
1 2 .2. Growth disturban ces . . . . . . . . . . . . . . . 86
1 2 .2. 1 . Treatment options for growth disturban ces 87
1 2 . 3 . Limb deformities . . . . . . . . . . . . . . . . . . . . 88

13. Bone dysplasias and developmental anomalies (Tibar Vizkelety) . 89

1 3 . 1 . Frequently o ccurring osteo chondrodysplasias . . . . . . . 90
1 3 . 1 . 1 . Skeletal dysplasias with predominant metaphyseal involvement . 90
1 3 . 1 .2 . Skeletal dysplasias with predominant epiphyseal involvement . 92
1 3 . 1 .3 . Skeletal dysplasias with maj or involvement of the spine . . . 92
1 3 . 1 .4. Dysostosis multiplex, complex carbohydrate storage diseases 93
1 3 . 1 .5 . Skeletal dysplasias with de creased bone density . . . . . . . 93
1 3 . 1 .6. Skeletal dysplasias with in creased bone density . . . . . . . . 95
1 3 . 1 .7 . Skeletal dysplasias due to the disorganized development of bone
constituens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97

1 4. Metaboli c diseases of the bone (Laszl6 Bucsi) . 101

1 4. 1 . Osteoporosis . . . . . . . . . . . . . . . 101
1 4.2. Osteomalacia (mollities ossium) . . . . 1 05
1 4.2. 1 . Ra chitis (ri ckets, avitaminosis D) 1 06
x � Co n t e n s

1 4.2.2. Vitamin D resistant ra chitis (Fan coni syndrome, phosphate

diabetes) . . . . . . . . . . . . . . . . . . . . . . . . . 1 08
1 4 . 3 . Hormonal metaboli c bone diseases . . . . . . . . . . . . . . . . . 1 08
1 4 . 3 . 1 . Hyperparathyroidism (Osteodystrophia fibrosa cysti ca
generalisata, von Re cklinghausen ' s disease) . . . . . . 1 08
1 4 . 3 . 2 . Hypogonadism (Adiposogenital dystrophy, Frohli ch ' s disease) . 1 09
1 4. 3 . 3 . Cushing' s disease . . . . . . . . . . . . . . . . . . . . . . . . . 1 09

15. Developmental anomalies o f the extremities (Tibor Vizkelety) . . . . . . . . . . . . 111

1 6. Neuromus cular diseases (Tibor V izkelety) . . . . . . . . . . . . . . 1 15

1 6. 1 . Spinal dysraphisms, myelodysplasia . . . . . . . . . . . . . . . 1 15
1 6. 1 . 1 . Meningo cele, myelomeningo cele, myelodysplasia. 1 15
1 6.2. Cerebral palsy (infantile cerebral paresis, Little disease) . . . . 1 18
1 6. 3 . Poliomyelitis (poliomyelitis anterior a cuta, Heine-Medin disease) 1 22
1 6.4. Developmental anomalies and diseases of the mus cle system . 1 23
1 6.4. 1 . Congenital mus cle absen ces . . . . . . . . . . 1 23
1 6.4.2. Mus cular dystrophy . . . . . . . . . . . . . . . 1 23
1 6.4. 3 . Myopathies due to inflammation, polymyositis . 1 25
1 6.4.4. Myositis ossifi cans progressiva (fibrodysplasia ossifi cans
progressiva) . . . . . . . . . . . . . . . . . . . . . . 1 25
1 6.4.5 Arthrogryposis (arthrogryposis multiplex congenita) . 1 25
1 6.4.6. Congenital laxity of the joints . . . . . . . . . . . . 1 26

1 7. Tunnel syndromes (Zoltim Csernatony) . . . . . . . . . . . . . . . . . . . . . . . . 1 27

1 8. Disorders of tendons, tendon sheaths, bursae, fas ciae and ligaments

(Zoltan Csernatony) . . . . . . . . . . . . . . . . . . 135
1 8 . 1 . Disorders o f the tendons . . . . . . . . . . . . . 135
1 8 .2. Illnesses o f the paratenon and the tendon sheath 136
1 8 . 3 . Diseases o f the bursae . 1 36
1 8 .4. Illnesses of fas cia. . . . 1 37
1 8 . 5 . Disorders of ligaments . 137

1 9. Diseases of joints (Zoltan Csernatony, JozsejLakatos, Tamas Meszaros,

Gyula Poor, Miklos Szendroi) . . . . . . . 1 39
1 9. 1 . The stru cture of the joints . . . . . . 1 39
1 9.2. The biome chani cal aspe cts of j oints . 1 40
1 9. 3 . Arthritis . . . . . . . . . . . . . . . 1 40
1 9. 3 . 1 . Autoimmune lo comotor diseases . 1 40
1 9. 3 . 1 . 1 . Rheumatoid arthritis . . 141
1 9. 3 . 1 .2 . Polysystemi c auto immune diseases affe cting
the lo comotor organs . . . . . . . . . . . . . 1 44
1 9. 3 . 2 . Seronegative spondarthritis . . . . . . . . . . . . . . . 1 44
1 9. 3 . 2 . 1 . Spondylitis ankylopoeti ca ( ankylosing spondylitis) 1 44
1 9. 3 .2 .2 . Other forms of seronegative spondarthritis (SNSA) . 1 47
1 9. 3 . 3 . Juvenile chroni c arthritis . 1 48
1 9. 3 .4. Crystal-indu ced arthritis 1 49
1 9. 3 .4. 1 . Gout . . . . . . . 1 49
 Co n t e n s � XI

1 9. 3 .4.2. Other forms of crystal indu ced arthritis 151

1 9.4. Arthropathies . . . . . . . . . . . . . 151
1 9.4. 1 . Neurogeni c arthropathy . 151
1 9.4.2. Hemophili c arthropathy 1 52
1 9. 5 . Performing surgery in arthritis . . . . 1 53
1 9.6. Synovial chondromatosis . . . . . . 1 58
1 9. 7 . Pigmented villonodular synovitis (PVNS) . 158
1 9. 8 . Arthrosis (osteoarthritis) . . . . . . . . . . 1 59
1 9. 8 . 1 . Alterations in various parts of the joint . 1 60
1 9. 8 .2 . Alterations in the arti cular endings of bones . 1 62
1 9. 8 . 3 . Transformations in the non-loaded zone, osteophytes . 1 62
1 9. 8 .4. Changes in the arti cular capsule 1 63
1 9. 8 . 5 . Symptoms . . . . . . . 1 63
1 9. 8 . 6 . Treatment of arthrosis . . . . . 1 64

20. Bacterial infe ctions of bones and joints (JozseJLakatos) 1 65

20. 1 . Osteomyelitis . . . . . . . . . . . . . . . . . . 1 65
20. 1 . 1 . A cute hematogeni c osteomyelitis . . 1 65
20. 1 .2 . Chroni c osteomyelitis . . . . . . . . 171
20. 1 .3 . Spe cial forms of primary chroni c osteomyelitis 1 72
20. 1 .4. Spe cial forms of inflammatory bone alterations . 1 74
20.2. Purulent arthritis . . . . . . . . . . . . . . . . . . . . . . 1 77
20.3 . Bone and joint tuberculosis . . . . . . . . . . . . . . . . . . 1 79
20.3 . 1 . Coxitis tuberculosa (tuber culosis of the hip) . . . 1 82
20. 3 . 2 Gonitis tuberculosa (tuberculosis of the knee-j oint) 1 83

21. Tumors of the mus culoskeletal system (Miklos Szendroi) . 1 85

2 1 . 1 . General . . . . . . . . . . . . . . . . . . . . . . . . 1 85
2 1 .2. Primary bone tumors . . . . . . . . . . . . . . . . . 1 92
2 1 .2 . 1 . Histologi cal classifi cation of bone tumors . 1 92
2 1 .2.2. Bone forming (osteogeni c) tumors . . . . . 1 92
2 1 .2.2. 1 . Benign bone forming tumors . . . 1 93
2 1 .2.2.2. Malignant bone forming (osteogeni c) tumors 1 94
2 1 .2.3 . Cartilage-forming tumors . . . . . . . . . . . 1 99
2 1 . 2 . 3 . 1 . Benign cartilage-forming tumors . . 1 99
2 1 . 2 . 3 . 2 . Malignant cartilage-forming tumors 20 1
2 1 .2.4. Bone marrow tumors . . . . . . . . . . . 204
2 1 . 2 . 5 . Giant cell tumor of bone. (osteo clastoma,
"brown tumor of bones") . 206
2 1 .2.6. Other rare bone tumors . 207
2 1 .3 . Bone metastases . . . . . . 208
2 1 .4. Tumor-like bone pro cesses . . . . . . 212
2 1 . 5 . Soft-tissue tumors . . . . . . . . . . 215
2 1 . 5 . 1 . Some soft-tissue tumors of orthopedi c signifi can ce 218

22. Reflex dystrophy (Sude ck ' s syndrome) (Zoltim Csernatony) . . . . . . 225

XII � Co n t e n s

23 . Asepti c bone ne crosis (Arpad Bellyei) . . . . . . . . . . . . . . . . . . . . . . . . . 229

23 . 1 . Childhood bone ne crosis (j uvenile osteo chondrosis, juvenile osteo chondritis,
juvenile osteo chondrone crosis) 229
2 3 . 2 . Adult asepti c bone ne crosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 1

24. Diseases of the spine (Tamas Illes, J6zsejLakatos, Peter Pal Varga) . 235
24. 1 . Biome chani cs, fun ctional anatomy, fun ctional units of the spine . 235
24.2. Examination of the spine . . . . . . . . . . . . 236
24.2. 1 . Assessment of the cervi cal spine . 237
24.2.2. Assessment of the lumbar spine 240
24.2 . 3 . Radiologi cal assessment . . . . . 242
24. 3 . Classifi cation of deformities of the spine . . . 245
24. 3 . 1 . Chara cteristi cs of deformities in one plane . 245
24.3 . 1 . 1 . Fun ctional hyperkyphosis 246
24.3 . 1 .2 . Fun ctional hyperlordosis . . . . . 247
24.3 . 1 . 3 . Stru ctural kyphosis . . . . . . . . 247
24. 3 . 2. Spinal deformities 3 dimension: S coliosis . 253
24. 3 . 2 . 1 . Fun ctional (nonstru ctural) s coliosis 253
24. 3 .2 .2 . Stru ctural s coliosis . . . 253
24.4. Congenital abnormalities . . . . . . . . . . . . 267
24.4. 1 . Spondylolysis, spondylolisthesis . 267
24.4. 2 . Sacralization, lumbarization 272
24.4. 3 . O ccult spina bifida . . . . . . . . 273
24.5 . Other diseases . . . . . . . . . . . . . . . . . 274
24. 5 . 1 . Baastrup syndrome (interspinous arthrosis) 274
24. 5 . 2 . Sacrum a cutum . . . . 274
24. 5 . 3 . Co ccygodynia . . . . . . . . . 274
24. 6 . Degenerative disorders of the spine . . . . 274
24. 6 . 1 . Degenerative spinal disorders 275
24.6. 1 . 1 . Degenerative pro cess of the dis c . 275
24.6. 1 .2 . Dis c prolapse, hernia . . . . . . . 275
24.6. 1 .3 . Degenerative spine instability . . 276
24. 6 . 1 .4. Clini cal examination of degenerative spinal
disorders . . . . . . . . . . . . . . . . . . . 277
24. 6 . 1 . 5 . Conservative treatment of a herniated dis c and
degenerative spinal stenosis . . . . . . . . . . 28 1
24.6. 1 . 6. Surgi cal treatment of degenerative spinal disorders 28 1
24. 7 . Spinal changes in osteoporosis . 282
24. 8 . Tumors . . . . . . . . . . . . 284
24. 8 . l . Primary tumors . 284
24. 8 . 2 . Metastases . . . 285
24.9. Inflammations of the spine . . 286
24.9. 1 . Spondylitis tuberculosa (Pott ' s disease) 286
24. 9 . 2 . Pyogeni c spondylitis (spinal osteomyelitis) 288
24. 9 . 3 . Spondylodis citis . . . . . . . . . 29 1
24. 1 0. Chest deformities . . . . . . . . . . . . . . . 292
24. 1 0. 1 . Pe ctus ex cavatum (funnel chest) 292
24. 1 0.2. Pe ctus carinatum (pigeon chest) . 294
 Co n t e n s � XIII

25. Disorders of the ne ck and the shoulder girdle (Jeno Kiss) . . . . . . 295
2 5 . 1 . Fun ctional anatomy and biome chani cs of the shoulder girdle . 295
25.2. Examination of the shoulder girdle . . . . . . . . . . . . . . 296
2 5 . 3 . The congenital and a cquired disorders of the of the shoulder girdle 297
25.4. Disorders of the shoulder . . . . . . . . . . . 3 02
2 5 . 5 . Other painful shoulder conditions briefly . . . 307
25.6. Glenohumeral instability, shoulder dislo cation 308

26. Disorders of the elbow and forearm (Jeno Kiss) . . . 311

26. 1 . Fun ctional anatomy of the elbow and forearm 311
26.2. Assessment of the elbow . . . . . . . 311
26. 3 . Signifi cant developmental anomalies . . . . . 311
26.4. A cquired disorders of the elbow . . . . . . . . 313
26. 5 . Volkmann' s is chemi c contra cture of the forearm and the hand . 317

27. Disorders of the hand and the wrist (Ferenc Mady) . 319
27. 1 . Fun ctional anatomy of the hand and the wrist . 319
27.2. Congenital anomalies of the hand . . . . . . . 32 1
27. 3 . A cquired hand disorders . . . . . . . . . . . . 322
27. 3 . 1 . Avas cular ne crosis of the carpal lunate bone
(Kienb ock ' s disease) . . . . . . . . . . . . . 322
2 7 . 3 . 2 . Cyst and pseudoarthrosis of the s caphoid bone 323
27.3 . 3 . Osteoarthritis (OA) of the wrist and the hand 324
27.3 .4. Inflammatory pro cesses . . . . . . . . . . 325
27.3 . 5 . Ganglioni c cysts of the wrist and the hand . . 326
27.3.6. Dupuytren ' s contra cture . . . . . . . . . . . 327
27.3.7. Tumors and tumorous conditions in the hand 328

28. Hip disorders (Arpad Bellyei, J6zsejLakatos, Mikl6s Szendroi) 329

28. 1 . Pediatri c hip disorders . . . . . . . . . . . . . . . . . . . 329
2 8 . 1 . 1 . Normal development of the hip joint . . . . . 329
2 8 . 1 .2. Congenital dislo cation and dysplasia of the hip 33 1
2 8 . 1 .3 . Osteo chondritis capitis femoris juvenilis (Perthes ' disease,
Legg-Calve-Perthes ' disease) . . . . . . . . . . . . . . . . . 342
2 8 . 1 .4. Slipped capital femoral epiphysis (SCFE, epiphyseolysis capitis
femoris juvenilis, coxa vara adoles cent) 347
2 8 . 1 .5 . Coxa vara infantum ( congenital) . . . . . . . 350
2 8 . 1 . 6 . Symptomati c coxa vara . . . . . . . . . . . . 35 1
2 8. 1 . 7 . Growth disturban ces of the proximal femur . 352
2 8 . 1 . 8 . Transitory arthritis coxae (transitory hip j oint inflammation) 352
2 8 . 1 .9. Juvenile a cetabular protrusion . . . . . 353
2 8 . 1 . 1 o . Snapping hip, external coxa saltans . . 354
2 8 . 1 . 1 1 . Inward o r outward rotation of the legs 35 4
2 8 . 1 . 1 2 . Limb shortening . . . . . . . . . . . . 355
2 8. 1 . 1 5 . Congenital hemiatrophy and hemihypertrophy . 358
28.2. Hip disorders i n adults . . . . . . . . . . . . . . . 358
2 8. 2 . 1 . Fun ctional anatomy . . . . . . . . . . 358
28.2.2. Deformities o f the hip - prearthrosis . 359
28.2.2. 1 . Valgus hip, coxa valga . . . 361
XIV � Co n t e n s

2 8 . 2 . 2 . 2 . Varus hip, coxa vara . . . . . . . . . . . . . . . . . . 362

2 8 . 2 . 2 . 3 . Antetorsion and retrotorsion of the hip (coxa antetorta
and coxa retrotorta) . . . . . . . . . . . . . . 362
2 8 .2.2.4. Other prearthroses . . . . . . . . . . . . . . . . . . 363
2 8 . 2 . 2 . 5 . Surgical treatment of prearthrotic conditions . . . . . 363
2 8 . 2 . 3 . Idiopathic femur head necrosis (aseptic, avascular femur head
necrosis) . . . . . . . . . . . . . . . . . . . . . . . . . . . 365
2 8 .2.4. Coxarthrosis (hip osteoarthritis, arthrosis deformans coxae) . . 368

29. Disorders of the knee (Laszl6 Hangody, Mikl6s Szendroi) 375

29. 1 . Structure and functional anatomy of the knee . 375
29.2. Congenital developmental disorders . . . 377
29.2. 1 . Congenital knee dislocation . . . 377
29.2.2. Genu recurvaturn . . . . . . . . . 377
29.2 . 3 . Developmental disorders of the patella . 377
29.3 . Repeated patella dislocation and subluxation . . . . 378
29.3 . 1 . Habitual dislocation of the patella . . . 378
29.3 . 2. Recurrent dislocation and subluxation of the patella . 378
29.3 . 3 . Luxatio patellae congenita . . . . . 379
29.4. Developmental disorders of the menisci . . . . 379
29. 5 . Developmental disorders of the tibia and fibula . 379
2 9 . 5 . 1 . Tibia, fibula aplasia, hypoplasia 379
2 9 . 5 . 2 . Congenital tibia pseudarthrosis . 379
29.6. Axial deformities . . . . . . . . . . . . . . . 3 80
29.6. 1 . Genu varum - crus varum . . . 380
29.6.2. Tibia vara epiphysarea (Blount' s disease) . 381
29.6. 3 . Genu valgum - crus valgum . 381
29.6.4. Torsion o f the tibia . . . . . . . . 3 82
29.7. Aseptic osteochondroses around the knee . . . 3 82
29.7. 1 . Osteochondritis dissecans genus . 3 82
29.7.2. Osteochondritis of the tibial tubercle
(Schlatter-Osgood' s disease) . . . . . 3 83
29. 7 . 3 . Adult aseptic femur condyle necrosis (Ahlb ack' s disease) . 3 84
29. 8 . Knee contractures . . . . . . . . . . . . . 385
29.9. Fluid accumulation (effusion) i n the knee . 385
29. 1 0. Cysts i n the knee region . . 3 86
29. 1 0. 1 . Ganglion . . . . . . . . . . . 3 86
29. 1 0.2 . Meniscus cyst . . . . . . . . 386
29. 1 0. 3 . Popliteal cyst (Baker' s cyst) . 387
29. 1 0.4. Bursitis praepatellaris . . . . 387
29. 1 1 . 1 . Collateral ligament injuries . 388
29. 1 1 .2 . Chronic collateral ligament insufficiency . 388
29. 1 1 . 3 . Cruciate ligament injuries . 38 9
29. 1 1 . 5 . Meniscal tear . . . . . . . . . . . . . . 391
29. 1 3 . Chondromalacia patellae . . . . . . . . . . . . . . . 392
29. 1 4 . Chondropathies, osteoarthritis (arthrosis) of the knee 393

30. Disorders of the foot (Janos Kranicz, Kalman T6th) . . . . 399

3 0. 1 . The functional anatomy and biomechanics of the foot 399
 Co n t e n s � xv

30.2. Foot deformities in childhood . . . . . . . . . . . . . . . . . . 400

30.2. 1 . Congenital foot deformities, developmental abnormalities 400
30.2.2. Pes equinovarus congenitus (congenital club foot) . 40 1
3 0 .2 . 3 . Pes adductus . . . . . . . . . . 405
3 0 .2.4. Pes excavatus . . . . . . . . . 406
3 0 . 2 . 5 . Pes calcaneovalgus congenitus 407
30.2.6. Accessory ossicles on the foot . 408
30 . 2 . 7 . Tarsal coalition . . . . . . . . . 408
3 0 . 2 . 8 . Osteochondrosis calcanei (apophysitis calcanei) 409
30.2.9. Osteochondrosis ossis navicularis pedis (K6hler' s I disease) 409
30.2. 1 0. Ostechondrosis capitis metatarsi 11. (K 6hler' s 11 disease) 410
3 0 . 3 . Foot deformities in adulthood . . . . . . . . . . . . . . . . . . 41 1
3 0 .3 . 1 . Pes planus (pes planovalgus, flat foot, fallen arch) . 41 1
3 0 . 3 . 2 . Hallux valgus, metatarsus I. varus 414
30 . 3 . 3 . Hallux rigidus . . . . . . . . . . 417
3 0 . 3 .4. Diseases o f the 5th ray . . . . . 417
30 . 3 . 5 . Diseases o f the sesamoid bone . 418
3 0 . 3 . 6. Metatarsalgia . . . . . . . . . 418
3 0 . 3 . 7 . Digitus malleus (hammertoe) . . 419
3 0 . 3 . 8 . Morton's neuroma . . . . . . . 419
3 0 . 3 . 9 . March fracture (stress fracture, fatigue fractures
on the metatarsals) . . . . . . . . . . . . . . . . 420
30. 3 . 1 0. Achillodynia (paratenonitis of the Achilles tendon) 420
30 . 3 . 1 1 . Haglund heel . . . . . . . 420
30.3 . 1 2 . Calcaneus spur . . . . . . 420
30.3 . 1 3 . Arthrosis of the foot joints 42 1
3 0 . 3 . 1 4. Tarsal tunnel syndrome . 42 1
3 0 .3 . 1 5 . Diabetic foot . 422

References . . 423
 Preface
One of the most dynamically developing
fields of medicine is orthopedics. Products of
high technology have become part of our ev­
eryday practice. One of the most successful
operations is hip- and knee joint replacement
using endoprostheses. More than 90 per cent
of the devices last longer than 1 0 years in the
patient. A separate branch of science
tribology deals with their wear-out proce­
dures. The development of technology has en­
abled us to introduce arthroscopic techniques
for joints like the knee, shoulder, hip, ankle
and wrist. By this minimal invasive surgery
we can remove menisci, replace the anterior
crucial ligaments in the knee and refix the
labrum in the shoulder joint. In tumor surgery
limb saving procedures are more and more fa­
vored, which however requires the evolution
of reconstruction surgery, i.e. development of
modular type tumor endoprostheses systems.
The implementation of modem imaging tech­
niques, like CT, MR and PET examinations
have made diagnostic procedures more pre­
cise and accurate, which is crucial for surgical
planning.
This book is intended to fill the need for
up-to-date information on disorders and dis­
eases treated by orthopedic surgeons and re­
lated physicians. The numerous illustrations,
photos taken of patients and removed surgical
specimens, MRI and CT pictures as well as
drawings have been carefully selected to max­
imize their benefits in pointing out orthopedic
principles and concepts and serve the better
understanding of diseases.
M. Szendroi, MD, PhD, DrSc.
 T i b o r Vf z k e l e t y
1. History and subject of orthopedics
Orthopedic conditions are probably basi­
cally as old as mankind itself. Neolithic skele­
tal remains for instance show changes due to
tuberculotic spondylitis. Tuberculotic gibbus
or coxitis, clubfoot and degenerative disor­
ders are often to be seen on Egyptian mum­
mies. Throughout history achondroplastic
dwarfs have frequently been the subject of il­
lustrations, and one picture suggests that epi­
demic poliomyelitis was not unknown. In an­
cient Baghdad, in Persia surgical and orthope­
dic patients were treated in separate wards in
hospitals as long ago as 1 000 BC.
In Hippocrates ' book on the joints we may
read about congenital clubfoot, hip disloca­
tion and the treatment of spine curvatures, and
his statements remain essentially today. Hip-
Fig. 1.1.
Fabricius ab Aquapendente: Opera Chirurgica
pocrates was perfectly familiar with the con­
genital hip dislocation.
Following Hippocrates, exercises were
proposed by Celsus for the treatment of spinal
deformities in the 1 st century AD . In the 2nd
century Galenus created the nomenclature for
the names of spinal curvatures : kyphosis,
lordosis and scoliosis.
In the Middle Ages, similarly to surgery
the treatment of the musculoskeletal diseases
was the task of healers and blacksmiths. This
played a certain role in the predominance of
mechanical treatment methods, enforced cor­
rections and fixations and by the 1 6th century
the field of the mechanical orthopedics was
established. The first and most noteworthy
representatives of this trend were Ambroise
Pare ( 1 5 1 0- 1 590) and Hieronymus Fabricius
ab Aquapendente ( 1 5 3 7- 1 6 1 9), who de­
scribed the origin of congenital clubfoot, hip
dislocation and torticollis and proposed vari­
ous treatment procedures. The book by
Fabricius ab Aquapendente illustrates a con­
struction of metal frames to fix and correct all
parts of the body and the j oints (Fig. 1 . 1 .) .
The term ' orthopedics ' was first used by
Nicolas Andry ( 1 65 8- 1 742) in his book titled
"L 'Orthopedie ou I 'art de prevenir et de
corriger dans les enfans des difJormites du
corps" (Orthopedics, the art of preventing and
correcting bodily deformities in children)
published in 1 74 1 . The picture in this book il­
lustrating a growing curved tree fixed to a
pole in an effort to make it erect has become
the symbol of orthopedics (Fig. 1 .2.). The
word ' orthopaedia' is of Greek origin created
according to Andry is originated from the as­
sociation of words orthos (straight) and
paidos (child). The book was also published
not only in French, but also in English and in
German.
2 � 1 . H i st o ry a n d s u bject of o rt h o p e d ics
&: � __ retl
._.
Fig. 1.2.
The symbol of orthopedics from And ry's book
With this name, the new specialty of or­
thopedics was born, and clear proof of its ex­
istence and development was the founding of
the first specialist orthopedic institution of the
world which was established in Orbe, Swit­
zerland in 1 779 by Jean Andre Venel
( 1 740- 1 79 1 ) . That institution, which may be
considered most sophisticated even today
provided the patients with medical treatment,
aftercare, a supply of appliances and educa­
tion.
During many decades orthopedic care
tended primarily to concentrate on fixation,
mobilization by means of various appliances,
or manual correction (the mechanical trend)
(Fig. 1 .3.).
The dynamic trend, involving the utiliza­
tion of exercises also has a long history. The
first book, titled "Medicina Gymnastica . "
by Francis Fuller was published in 1 705 and
was reprinted 9 times. The term "orthopedie"
in Andry book likewise refers to the impor­
tance of exercises for the development of a
straight posture. These exercises took the pa-
Fig. 1.3.
Scu ltety 1666: appliance to correct spinal curvature
tients to the open fields, adding the healing ef­
fects of the open air and the sunshine. In the
early of 1 9th century, Ling in Sweden intro­
duced medical exercises, for the active and
passive mobilization of certain parts of the
body. Accepted into practice under the name
of "Swedish gymnasty" and the essence of
these procedures is made use of in modem
times.
Surgical procedures were resorted for the
correction of contractures and deformities, es­
pecially in cases where mechanical solutions
had failed. The sequence of evolution of sur­
gical treatment was first closed interventions,
followed by subcutaneous tenotomies and fi­
nally open-exposure operations . The tech­
niques of asepsis and antisepsis introduced by
Semmelweis and Lister played an important
role in this development.
The early surgical interventions were ob­
viously the work of the bone setters, who
could successfully treat certain conditions,
and who handed on their art through many
generations.
Revolutionary changes in the performance
of mechanical and surgical treatment fol­
. . lowed the introduction of plaster of Paris
casts, first used by Dutch physician Mathysen
in 1 85 1 and applied extensively ever since.
Thus the mechanical, functional and sur gi­
cal approaches for the treatment of orthopedic
conditions developed in parallel. In recent de-
 1. H i st o ry a n d s u bject of o rt h o p e d ics
cades, the changes have conside rably accele r­
ated and at p resent pe rhaps the most inten­
sively and dynamically developing b ranch of
medicine. The p rog ress in science ove rall has
naturally played a g reat role in this. The
achievements in anesthesiology and intensive
care now pe rmit successful extensive and
timely surgical p rocedu res that we re ea rlie r
impossible.
The development is basically proceeding i n
two directions:
• The prevention of diseases, and the early
diagnosis and treatment, leading to the
prevention of permanent deformities;
• Utilization of the available technical
achievements in the therapy.
Medicine is most effective if disease is
p revented when the rapy is unnecessa ry. The
best example in o rthopedics is the int roduc­
tion of vaccination Salk and Sabin, which has
eradicated poliomyelitis epidemics in those
count ries whe re it is used systematically.
Well-o rganized hip sc reening can p revent
hip dislocation and dysplasia, fo r early t reat­
ment can solve it without any residual defo r­
mity.
Besides the developments in su rge ry and
anesthesiology p rog ress in othe r p rofessions
has also played an impo rtant role in orthope­
dics. One good example is metallu rgy, with
the p roduction of various new alloys, with
special mechanical p rope rties, which have re­
sulted in the manufactu ring of app rop riately
shaped endop rostheses and implants of excel­
lent quality which g reatly p romote successful
surge ry.
The re have simila rly been g reat develop­
ments in the field of tissue adaptive plastic
mate rials, whe re the new techniques have rad­
ically changed the system of traditional
limb- replacing p rostheses.
� 3
Following the discove ry of x- rays and the
int roduction of radiology, which was a revo­
lutiona ry step in o rthopedic diagnostics, the
cu rrently applied imaging p rocedu res also re­
flect impo rtant imp rovements both in the di­
agnostics as well as in the surgical t reatment.
The int roduction of a rth roscopic diagnos­
tics and su rge ry too has also resulted in revo­
lutiona ry changes in o rthopedics.
O rthopedic activity sta rted in Hunga ry
ve ry early. Some 50 yea rs afte r the establish­
ment of the fi rst o rthopedic institution in
Switze rland by Venel, Agoston Schopj Merei
founded a p rivate "Pesti Orthopedical P rivate
Institute" in 1 836 .
Modem su rgical o rthopedics was int ro­
duced by Gyula Dollinger. In a textbook pub­
lished in 1 942 J Kopits states that "the p res­
ent gene ration of physicians owe thei r thanks
to Dollinger fo r thei r o rthopedic knowledge".
A definite change was the inclusion of o r­
thopedics into the cu rriculum of the medical
school, when the O rthopedic Clinic of Buda­
pest Unive rsity was established in 1 95 1 . The
subj ect of o rthopedics being desc ribed b riefly
as the specialty of medicine dealing with p re­
vention , t reatment and resea rch of diseases of
the musculoskeletal system.
In Hunga ry, simila rly as in Ge rman­
speaking a rea t raumatology and o rthopedics
sepa rated from gene ral su rge ry, which was in
cont rast with the situation elsewhe re in Eu­
rope.
T raumatology deals with acute inj u ries,
including polyt rauma, while o rthopedics
t reats post-t raumatic conditions, axial defo r­
mities, congenital and inhe rited systemic
bone diseases, limb developmental deficien­
cies, inflammato ry diseases of bones and
joints, musculoskeletal tumo rs, etc . Natu rally
howeve r, the re a re conside rable ove rlaps in
the activities of these two p rofessions. Finan­
cial conside rations and effo rts to confo rm to
the p ractice in Eu ropean Union will ce rtainly
result in common t raining and ultimately the
union of the two specialties.
 Zo lta n Cse r n a t o n y

2. Structure and development of bones

2.1. Structure of bones
The bones have a numbe r of functions :
they suppo rt the body, thei r rigidity playing
role in maintaining the shape of the body.
They fo rm the joints with thei r ca rtilaginous
ends, the j oints a re fixed by ligaments. They
se rve as leve r a rms around joints; the j ointed
bones are moved and at the same time stabi­
lized by the muscles via the tendons . They
p rotect vital o rgans and include a significant
part of the hemopoetic system. Finally they
are impo rtant as mine ral rese rves in the cal­
cium and phospho rus metabolisms of the o r­
ganism. Summa ry: the bones play p rotective,
mechanical and metabolic roles in the body.
2.1.1. The composition of bone
Chemically all bones have almost identi­
cal composition. Roughly 1 13 of the adult
bone consists of wate r. D ry matte r content of
fat free bone tissue contains 65% ino rganic
mate rial (mainly hydroxyapatite), while 3 5 %
i s o rganic (chiefly collagen). The bones ac­
counts fo r holds about 99% of the calcium
content of the body.
2. 1.2. The bone tissue
Histologically bone is st ructu red by cells
and a mat rix. The cells which comp rise 1 -5%
of the ove rall mass a re responsible fo r the bio­
logical p rope rties, the mat rix accounting fo r
the mechanical p rope rties. The inte rcellula r
mat rix, and specifically the osteons equips the
adult bone with excellent mechanical quali­
ties.
Mac roscopically the bone tissue can fo rm
compact (substantia compacta) o r spongious
(substantia spongiosa or cancellous) mate ri­
als. Since the compact fo rm mainly c reates
the co rtex of the bones, it is often refe rred to
as co rtical.

1 -,.I�... 1 central canal

2 central canal
----

7
8 3 Havers- lamella
9
2
4 vessels and nerves
3

10 5 osteocyte
6 Volkmann-canal
7 basic lamella

4 -kmf:l��� 8 cemen- line

9 Havers-canal
6 ----+'!I-I...u �=f-- ll
5 --III-'IIII+H"
10 connecting lamella
11 bone trabecules

Fig. 2.1.
Histological structure of human bone
6 � 2 . St r u ct u re a n d d eve l o p m e n t of b o n e s

The basic s truc tural elemen t of subs tan tia
compac ta is the o s teon (Havers sys tem). This
is a thick-walled tube tha t includes a narrow
canal con taining, concen tric pipes 5 - 1 0 �m
thick (Fig. 2 . 1 .) . Small plum- s tone-shaped
holes loca ted sparsely in the walls of the
os teons serve to accommoda te the real bone
cells ( o s teocy te). These holes are connec ted
via Volkmann canals, which are no t sur­
rounded by lamellar sys tems. The lamellae
tha t form the wall of the os teons con tain colla­
gen fibers, which may be loca ted longi tudi­
nally, crosswise or in spirals. Calcium crys tals
are connec ted to the collagen fibers, the orien­
ta tion of the collagen fundamen tally de ter­
mining the loca tion of the crys tals. The irreg-
ular spaces be tween the "comple te" osteons
are filled wi th the remnan ts of os teons .
One of the mos t importan t phenomena ex­
hibi ted by bones is their permanen t res truc tur­
ing. In 1 year 5 - 1 0% of the os teons, and up to
20% of the cancellous bone in adul ts undergo
res truc turing.
Cancellous bone does no t display os teon
s truc turing: i t con tains fine lamellae and tubes
enclosing a complica ted cavi ty sys tem.
The s truc ture of bone is perfec tly adap ted
for i ts role in the body. The alignmen t and spi­
raling of the os teons ma tch the main
load-bearing lines of the bone, they ru n ap­
proxima tely parallel to the longi tudinal axes
of the bones.

�\ 9 1 epiphysis
2 physis (growth plate)
2.......:: 7_=-\

3 tl'-'ori �§&7� 3 apophysis

4 metaphysis
10 5 diaphysis
4 \\�o;'1 I 6 metaphysis
7 physis
11 8 epiphysis
12 9 joint ca rtilage
10 spongiosa (cancellous bone)
13
1 1 periost
12 cortical bone
13 vessel
5 1 .... 1 14
14 med ullar cavity
15 periost
16 nerve
15 17 vessel
18 joint cartilage

/
16
6
17

(
7

8 , \ ( 18

Fig. 2.2.
Structu re of a long tubular bone (femur)
 2 . St r u c t u r e a n d d eve l o p m e n t of b o n e s
2.1.3. Bones as organs
The bones are built up of bone tissue con­
taining cancellous and compact bone in vari­
ous distributions and with complicated geom­
etry. Morphologically we may distinguish
flat, cubic, cavital, short and long tubular
bones. As regards their function we may refer
to permanent load-bearing (the lower limbs
and the spine), occasional load-bearing (the
upper limbs) and non-load bearing (the facial
skull) bones.
The nomenclature of the tubular bones is
based on the growth plate, the physis
(phyestar to grow, Gr). The part between
=
two physes is the diaphysis, the part in the vi­
cinity of physis is the metaphysis, and the
ends form the epiphyses.
The protruded bone - ends that have their
own growing plate and serve for the origin
and attachment of muscles are the apophyses
(Fig. 2.2).
2.1.4. The development and g rowth
of bones
Development of bones starts from the em­
bryonic connective tissue, the mesenchyma
when the embryo is 2 months old. This pro­
cess requires a good supply of blood and oxy­
gen. There are three different developmental -
histological forms depending on the mechani­
cal environment:
I. Desmal ossification
In locations subj ected to pulling forces a
preliminary ossifying focus is created con­
taining connective fibers. The ossification
starts from the so called ossification centers.
2. Chondral ossification
In locations subjected to compressive
forces, a preliminary focus is created that con­
tains cartilage, which is a rough miniature
replica ofthe final bone. The vessels enter this
cartilaginous tissue and trigger the ossifica­
tion process via osteoblasts.
� 7
3. Primary angiogenic ossification
This process occurs in areas lacking me­
chanical forces. It is characteristic for in­
stance, in the spaces between the skull bones.
No precursor tissues are present, the bony de­
velopment starts with the invasion of vessels.
In the growth plate in the vicinity of the
end of the bone, the cartilaginous cells prolif­
erate, and on the opposite side the chondral
ossification process continuously progresses.
By the end of the growth the growth plates are
ossified and closed. The whole procedure is
regulated by hormones. The bones thicken by
transversal growth. In the inner, cambium
layer of periosteum covering the bone, bony
apposition progresses via osteoblasts, while at
the inner surface of the tubular bone the
osteoclast activity leads to bone resorption.
This is the reason why the proportions of tu­
bular bone are protected during the growing
period (see Chapter 1 2. ) .
2.2. Biological adaptation of bones
The composition, histological structure
and shape of the bones are influenced not only
genetically, but also by various mechanical
factors .
As living tissue, bone responds to chang­
ing external circumstances, and especially to
compression and traction forces with perma­
nent restructuring, i.e. remodeling. For opti­
mal load distribution the bony trabeculae
thicken so as to correspond to the compres­
sion - traction force alignments creating the
traj ection lines. In this way, minimal material
is required for the maximal bearing force.
If the alignment of the load changes, e.g. in
cases of different collodiaphyseal angles of
the femoral bone, then the traj ection lines of
the bony trabeculae also change (Fig. 2.3.
a-b).
As Julius Wolfformulated in his law: "The
persistence or transformation of the shape of
the skeleton is influenced by the forces acting
on the bone." In other words : remodeling ad­
aptation of bone allows the musculoskeletal
8 � 2 . St r u ct u re a n d d e ve l o p m e n t of b o n e s

system to adjust most appropriately to re­

peated utilization (Fig. 2.4. a-c. ) .
The law of Jansen states that "The sensi­
tivity of the organism to external stimuli is
proportional to the intensity of growth". As
concerns the musculoskeletal system this
means, that in response to a certain degree of

b
a

Fig. 2.3.
Traction and compression trajection lines in the fem­
oral neck.
a. Normal trajection lines in the normal hip joint.
b. Alteration of trajection lines in a subluxated,
valgus femoral neck.
Fig. 2.4.
a. Lytic bone destruction in the calcaneus of an
8-year-old boy (arrows).
b. Following cu rettage the cavity was filled with ho­
mologous bone chips.
c. After 3 years, full restructuring and remodeling is
apparent: a normal trajection bone structure match­
ing the loading conditions has been restored.
 2 . St r u ct u re a n d d eve l o p m e nt of b o n e s
damage, the younger the organism, the more
severe the deformity. At the same time the re­
generative capacity is larger.
The law of Roux pronounces : "The ade­
quate stimulus for bone formation is the pres­
sure". This law is supplemented with impor­
tant elements by the law of Schultz-Arndt:
"Small stimuli increase the living processes,
large ones decrease them and huge ones ter­
minate them". This may be illustrated by the
behavior of hyaline cartilage cells: their me­
tabolism increases during a normal cyclic
load, but increase of the load above a certain
limit results in the demise of the cells.
According to the law of Pommer-Braus:
"Bone is mechanically firm, but physiologi­
cally plastic". This is of significance in the
pathophysiology of fractures (see Chapter
3 . l ).
These laws provide phenomenological de­
scription of the biomechanical responses of
the skeletal system to certain stimuli (hor­
mones, injuries and loading). The background
of the phenomena at a cell - tissue level in­
volves the mechanism of the internal or struc­
tural remodeling. The main point is that the
o
UV- beam
I
vit. D
Ag. 2.S.
Regu lation of the ca lcium metabolism
� 9
transformation of bones is effected by activa­
tion - resorption - formation.
2.3. Hormones reg u lating the bone
metabolism
A number of hormones and vitamins helps
in regulate the bone and calcium metabolisms
(Fig. 2.5.).
Parathormone (in the parathyroid gland)
increases the calcium resorption and phos­
phate excretion in the kidney. It enhances the
osteoclast activity in the bones. It indirectly
supports calcium absorption in the gastroin­
testinal tract. All these effects result in an in­
creased serum calcium level and a decreased
serum phosphate level. Pathologically high
parathormone levels cause transformation of
the bone, creating cystic lesions (osteo­
dystrophia fibrosa cystic a generalisata -
von Recklinghausen 's disease, see Chapter
1 4.).
Calcitonin (in the thyroid gland C cells) is
an antagonist of parathormone. It inhibits the
activity of osteoclasts, increases the number
--TSH�
�
c-.ce
ACTH STH
1
10 � 2. S t r u ct u re a n d d eve l o p m e n t of b o n e s
and function of osteoblasts, which results i n a
decreased serum calcium level. The secretion
of this hormone is regulated by the serum cal­
cium level. Its effects described above and its
pain-killing action is utilized in the therapy of
osteoporosis.
Somatotropic hormone (STH, in the ante­
rior pituitary lobe) is responsible for the
growth of the bones, until the epiphyses are
closed. Its elevated secretion (pituitary
adenoma) before the closure of the growth
plates causes gigantism; after that period it
causes extensive thickening of the bones and
overgrowth of the acral areas (acromegaly).
Sex hormones, such as testosterone play im­
portant roles in the increased growth during
puberty; in this period changes in the propor­
tions of the somatotropic and sex hormones
are manifested in a characteristic condition:
slipped capital epiphysis, epiphyseolysis
capitis femoris (see chapter 2 8 . 1 ). The de­
creased estrogen secretion in women in the
postmenopausal period results in osteoporo­
sis.
An elevated adrenocorticotropic hormone
(ACTH) secretion stimulates the function of
the adrenal cortex, leading to a calcium defi­
cit. The increased secretion (or therapeutic ap­
plication) of corticosteroids also results in a
decrease in bone mass, since it inhibits the ab­
sorption of calcium in the bowels, increases
the secretion of calcium in the kidney and
ends in secondary hyperparathyroidism. High
dose steroid administration inhibits osteoblast
activity.
Active metabolites of vitamin D (e.g.
1 -25-dihydroxy-vitamin D) increase the ab­
sorption of calcium and phosphate both in the
kidney and in the bowels. They mobilize the
calcium ions in the bone and inhibit the secre­
tion of parathormone in the parathyroid gland.
Overall vitamin D participates in bone forma­
tion, and it is therefore a basic drug in osteo­
porosis. Vitamin is of significance in collagen
synthesis. A deficiency of vitamin C causes
pathological disturbances in osteoid forma­
tion.
 Mi kl6s Szen d r6i

3. Bone healing after various types of

fractures

3. 1. Traumatic fractu res weight on hislher leg) are features leading to

the suspicion of a fracture.
In the event of a sufficient degree of trauma,
when the stress on a bone exceeds its Radiological findings. Anteroposterior
strength, the continuity of the bone sub­ and lateral radiographs reveal the discontinu­
stance is interrupted and a fracture occurs. ity of the bone with or without displacement.
The lesion is called a fissure if there is a frac­
ture gap in only one of the cortices. In some
Etiopathology. There are many classifi � a­
cases, if several bones are proj ected onto each
tions for fractures. From the aspect of the sIte
other (pelvis, shoulder girdle, spinal facets,
of action of the force, the fracture is said to be etc.), a eT-scan is required. If a ligament le­
direct if it occurs at the site of stress; and indi­
sion is suspected, an MRI is required.
rect if it occurs at some distance from the site
of the stress.
Treatment. The earliest possible and pre­
As concerns the pathomechanism, we dis­
cise reduction and fixation of the fracture is
tinguish bending, torsion, compression, sn:ain
important for reestablishment of the circ� la­
and stress fractures. Bone is weakest agamst
tion of the soft tissues. The type of fixatlOn
torsional stresses, followed by distraction and
can be external (casting with plaster of Paris,
compression forces. Special fracture types in
or use of an external fixateur) or internal : ei­
children are epiphyseal plate fractures and
ther on the surface or in the medullary canal of
plastic deformities. This involves bending of
the bone. There are three categories of fixa­
the long bones, mostly the fibula and the ulna,
tion stability: I . positional stability (pinning
which often accompanies the greenstick frac­
or plaster immobilization), 2. mobilization
tures of the tibia and the radius.
stability (the most types of plate fixation) and
Fractures can be classified according to
3 . weigh-bearing stability (some intra­
the shape and the direction of the fracture li!le:
medullary fixations) . In cases of rib fracture,
transverse fractures, oblique fractures, spIral
chest bone fracture, clavicle and vertebral
fractures, longitudinal, Y-shaped, fragmented
body fracture spontaneous healing may occur
and comminuted fractures.
and stable fixation is not necessary.

The patient' s history is usually typical

permits conclusions concerning the direction
3.2. Fract u re healing.
of the stress, the shape of the fracture and the
possible accompanying injuries. Types and stages.
Factors influencing fracture healing
Clinical features. Definitive signs are the
deformity of the limb with crepitus and patho­
logical mobility at the site of the frac �re. !he Fracture healing i s a complex process regu­
presence of pain, hematoma and an Impaired lated by neural and humoral factors.
function (e.g. the patient is unable to put
12 � 3 . B o n e hea l i n g aft e r v a r i o u s ty p e s o f fract u res

Direct bone healing (primary callus) develops
if the fixation is stable (osteosynthesis with a
plate or an intramed u l lary device), if the gap
between the broken fragments is minimal
and the fragments fit well with adequate
compression, and if the blood supply is satis­
factory. Fracture healing is said to be indirect
if the compression between the broken frag­
ments is low and it is the naturally developing
callus which p rovides the stability (plaster im­
mobilization).
Stages of fracture healing (Fig. 3 . 1 .) :
Stage 1 : Hematoma / inflammatory phase.
Bleeding from the injured longitudinal
endosteal and periosteal vessels leads to a
hematoma.
Stage 2 : Subperiostal-endosteal cell pro­
liferation. An intercellular matrix is formed,
which is mostly composed of fibrous-like tis­
sue, but immature cartilage may also be pres­
ent. This matrix surrounds the broken frag­
ments .
Stage 3 : Callus formation. Stem cells
transform to osteoblasts, osteoclasts and
chondroblasts which participate in the forma-

�
��
1

2
a: stage 1:

3 2: periosteum
1: muscle

3: fracture-hematoma

��p:" �J :
4: medullar cavity
5: cortical bone
6: necrotic dead bone
b: stage 2:
1: fracture hematoma
2: subperiosteal cel l proliferation

"'::>Siri �
3: endosteal cell proliferation
c: stage 3:
1: net- like new bone formation
a::2'§ id eC �Q� 3 2: subperiosteal cell proliferation

b
3: fracture hematoma
d: stage 4:

b:��
1, 3: net- like new bone
2: lamellar bone

00 0
� 4: bone marrow

0 0 <:'0 ",;",: .;-" 0 >�01 3

e: stage 5:
1: recanalisation of intramedullary canal

� "" 1

11!L: ,0 4

Fig. 3.1.
Stages of fracture healing
 3 . B o n e he a l i n g aft e r va r i o u s ty p e s of f r a ct u r e s � 13

tion o f immature net-like bony tissue. The cal­ 3.3 Pathological fracture healing.
cium salt content increases at this site and this
will be able to fix the broken bones; it also
Pseudo-arthrosis
gives X-ray signs.
Stage 4 : Consolidation. The net-like bony The d uration of fracture healing depends on
tissue transforms to mature bone tissue due to factors such as the bone type, the fracture
the activity of the osteoblasts. site within the bone (metaphysis or diaphysis),
Stage 5 : Remodellation. The spindle the type of fracture, the age and the general
shaped callus fills the intramedullary canal condition of the patient. Long bones heal
and the gap between the broken fragments. A within 2-4 weeks in infants, within 4-6 weeks
in children but only within 8- 12 weeks in
huge callus develops in children, or if the gap
adults.
is wide, or if there is marked displacement or
if the fixation is insufficient and micromo­
tions occur. The remodellation of the callus The healing of cancellous bone fractures
starts soon, but its duration can vary widely; (in the metaphyseal area) is quicker and better
this process sometimes takes years. since the spongy bone structure provides a
greater surface and quicker penetration for the
The healing of osteotomies or other pro­ immature cells and tissue elements of the
cesses on the bones is equivalent to primary early callus.
bone healing since the internal fixation is usu­
ally stable. In certain cases external fixation is
applied.
Post-traumatic growth disturbances are
common in children. Overgrowth of the af­
fected bone is often seen, due to the stimulat­
ing effect of a trauma or fracture on the neigh­
boring growth plates. If the blood supply of
the growth plate is impaired (a trans­
epiphyseal fracture, a slipped epiphysis, sep­
tic conditions affecting the growth plate, etc.)
shortening of the affected bone may occur.
The younger the patient, the more significant
the expected limb length discrepancy.
The remodellation of post-traumatic bony
deformities is typically seen in children. Axis
deviations and rotational deformities may im­
prove within certain limits . The younger the
child the greater the remodelling capacity.
This usually happens during the first two
years following fracture healing. After pu­
berty (at the age of 1 0- 1 1 in girls, or 1 2- 1 3 in
boys) significant remodelling is usually not
expected.

a b

Fig. 3.2.
Hypertrophic (a) and atrophic (b) pseudo-arthrosis in
the tibia
14 � 3 . B o n e he a l i n g aft e r v a r i o u s ty p e s of fract u res

If the duration ofthe bone healing is mark­ osteogene tic capacity. Septic pseudo-arthro­
edly longer than the average duration of the sis necessitates debridement and stabiliza tion.
given fracture type, delayed callus formation
is the case. This is usually caused by unstable,
insufficient fixation. 3.4. Pathological fractures
P seudo-arthrosis (false joint) is the term
used to describe the condition when after 8
months bony fusion has not occurred yet and
Pathological fractures occur in weakened
the pathological movement between the bro­
bones or at the sites of bony lesions due to
ken fragments is still present. There is no
minor traumas.
osteogenetic activity between the fragments.
The cause is commonly the instability, bu t
pseudo-arthrosis may also develop because of
mechanical factors, such as too rigid fixation, Table 3.1.
improper reduction or soft tissue between the Common disorders leading to pathological fracture

1.
fragments; or because of biological factors :
impaired blood supply, septic condition, bone Generalized osteopenia
defects or non-vital bone fragments at the a. decreased levels of osteoporosis
fracture site. bone minerals osteomalacia

b. endocrine disorders hyperpa rathyreoidism

Various types of pseudo-arthrosis may be
Cushing's disease
distinguished: hyper trophic, atrophic and sep­
hyperthyroidism
tic pseudo-arthrosis (Fig. 3.2.).
c. metabolic disorders of Paget's disease
The clinical features include pain, the bone dystrophies osteogenesis imperfecta
edema, swelling, pathological mobility (not in osteopetrosis
all cases) and inability to put weight on the af­ hypophosphatasia
fected limb. renal osteodystrophy
fibrous dysplasia
Radiology. Hypertrophic pseudo-ar thro­ (polyostotic form)
sis is characterized by the "elephant-leg"
X-ray sign. The narrow gap between the bro­ d. medication steroids
influencing the bone cytostatic drugs
ken fragments is filled with fibrous and carti­
metabolism
lage tissue. In atrophic pseudo-arthrosis, the
broken fragments are thin and round without 2. Bone defects
any signs of bone formation. The circulation a. tumor- li ke lesions juvenile bone cyst
of the bones is insufficient. aneurysmal bone cyst
non-ossifying fibroma
Treatment. This depends on the cause of eosinophyl granuloma
the pseudo-arthrosis. The hypertrophic type is
commonly caused by mechanical factors (in­ b. benign bone tumors enchondroma
stability in most cases). Proper stabilization giant-cell tumor
and compression usually leads to the fusion of c. malignant primary osteosarcoma
the fracture. In the atrophic type stabilization bone tumors (teleangiectatic type)
alone is not sufficient. Surgical intervention is Ewing's sarcoma
required. The fibrous tissue must be removed
d. osteolytic bone renal carcinoma
from the pseudo-arthrosis and autologous
metastases lung carcinoma
bone grafting is then necessary to increase the
 3 . B o n e hea l i n g aft e r v a r i o u s t y p e s of f r a ct u res
a Clinical features. Sudden pain without
b adequate trauma. Crepitus and an inability to
Fig. 3.3.
a) Metastasis in the right femur subtrochanteric area
in a case with non-operable l u ng carcinoma. There is
a lytic lesion in the lateral cortex (arrows), with a
threatening fracture there.
b. Status after intramedu llary nailing in order to pre­
vent the fracture.
� 15
Etiopathology. Different causes of in­
creased bone fragility are listed in Table 3 . 1 .
The status ofa threatening bonefracture i s
the condition when a pathological fracture has
not yet developed, but the quality of the bone
and the extent of the lesion suggest that this
will probably be the case after a minor trauma.
The assessment of a threatening bone frac­
ture is not easy because of the considerable
number of the influencing involved factors : Is
the affected bone is a weight-bearing one or
not; is the site of the defect central or cortical?
What is the quality of the margin of the lesion,
is there is a sclerotic border? Pathological
fracture are threatening in the case of pure
bone lesions; and in metastases, if these mea­
sure more than 2 . 5 cm or half of the diameter
of the bone and are eccentrically localized in
the cortex of a weight-bearing bone (Fig. 3.3.
a, b).
walk (if a lower limb is affected).
Radiology. This depends on the etiology.
Diffuse osteoporosis or osteopenia may be
present in generalized bone diseases. Lytic
bony lesions with fracture lines may be ob­
served in local processes.
Treatment. This depends on whether the
fracture has occurred or threatening; whether
the etiology is known or not. In case of a
threatening fracture in generalized disorders,
the choice of treatment is medication alone
and elimination ofthe cause of the osteopenia.
In local lytic bony lesions, a number of
factors should be taken into consideration. In
children, even in the case of huge cysts (juve­
nile bony cysts of the humerus) the fracture
can be just a bony impression without any dis­
placement due to the thick periosteum. Thus,
the treatment is immobilization of the arm in a
Desault' s bandage (arm-to-body bandage) for
a few weeks. After healing a decision must be
made concerning aspiration of the cyst or
open surgery and curettage. In fractures in­
volving cysts affecting the lower limbs
16 � 3 . B o n e h e a l i n g aft e r v a r i o u s t y p e s of fract u res
(weight-bearing bones) a fixation is necessary
with respect to the growth plates. In solitary
bone metastases, the lesion must be totally re­
moved and the defect should be repaired. In
multiple lesions, even in threatening frac­
tures, palliation is required (fixation with
intramedullary nails, plates, prostheses etc.).
3.5. Stress fractu res
These are partial or com plete fractures due to
chronic or repeated overloading of a bone.
Synonyms: march fracture, fatigue fracture.
Etiopathology. The first reported cases
related to soldiers, who had developed fatigue
fractures in the metatarsal bones (march frac­
ture). Cases were then published which in-
Fig. 3.4.
Fatigue fracture in the 2nd metatarsal bone with a
huge ca llus.
volved athletes following extreme physical
activity : stress fractures. Sometimes it may
occur after a single episode of overexertion
(dancing, runni ng, trekking). In 95% of the
cases observed fatigue fractures develop in
the lower limbs or in the pelvis . They are most
commonly in the tibia and in the metatarsal
bones, and with somewhat lower frequency in
the femoral neck and the pelvic bone.
Clinical features. Severe pain is experi­
enced initially, possibly causing an inability
to walk. The pain persist even at rest. In time
the pain decreases spontaneously and the pa­
tient feel no pain during the normal daily ac­
tivity, but only in response to repeated exer­
cises. The symptoms may last for months.
Radiology. A fissure is observed in the
cortex in a typical case. Less frequently, a
complete transverse fracture may be seen with
some periosteal reaction and usually a huge
callus formation (Fig. 3.4.). It is sometimes
difficult to detect a fissure within the callus. A
CT scan may be helpful.
Treatment. In most cases conservative
treatment leads to complete healing. Suspen­
sion of the sporting activity, administration of
NSAIDs and rest are necessary. Immobiliza­
tion of the limb is sometimes unavoidable.
Promising results have been reported from the
application of low-intensity shock-wave ther­
apy. After healing, the patients are usually
able to resume their previous sporting activity
at the same level.
 Kata l i n Kol lo, Adam Mester, la mas Mesza ros
4. Examination methods in orthopedics
4.1. Case history (anamnesis)
The examination of orthopedic patients al­
ways begins with the case history and a de­
tailed interview relating to the current com­
plaints.
The family history. Information is sought
from the patient or possibly the family mem­
bers as to the occurrence of any inherited dis­
ease in the family. This may be significant as a
number of musculoskeletal disorders and dis­
eases are inherited, and even the process of in­
heritance is known.
The patient' s personal history. In cases
of children it is important to know if the
mother or the embryo was exposed to any
noxious effect during the pregnancy. Prema­
ture birth or any other problems during deliv­
ery could be an indication of perinatal nervous
system damage.
Other features that are possibly of impor­
tance are the birth weight and the times at
which the various stages in the motion devel­
opment were attained, i.e. the sitting up,
standing up and independent gait. In female
patients the times of the starting and cessation
of the monthly periods may be useful data in
many in view the hormonal relation to the
bone metabolism.
As regards the diagnosis, the most signifi­
cant data are the current orthopedic com­
plaints and symptoms of the patient. It is im­
portant to learn the time of onset and circum­
stances of the current complaints of the pa­
tient. If the disorder has been present since
birth, it is most likely a congenital problem. It
is important to know, when the patient or the
relatives first noticed the symptoms, for the
occurrence of many diseases is linked to a cer­
tain age (e.g. osteochondroses, some types of
scoliosis), and it must be determined, if the ill­
ness is chronic or acute? It is of particular rele­
vance to explore the circumstances of the on­
set, e.g. does the patient associate hislher
problems with a specific injury, sudden j erk
or some other event? Although only a few or­
thopedic conditions can be associated with
trauma, the overwhelming maj ority of pa­
tients consider some injury to be the start of
and also the reason for their disease. As injury
or overload can certainly mean initiation of
the symptoms but without necessarily playing
a decisive role in the illness. As an example,
the pain caused by a bone tumor is often asso­
ciated by the patient with some trauma, sug­
gesting to the patient that this particular injury
was the cause of the disease.
It is important to learn, whether the patient
has detected fever, shivers or other symptoms
relating to inflammation. The sequence of on­
set of the symptoms furnishes a picture of the
progress of the illness. Information must be
obtained regarding the earlier operations and
their effects that may be relevant to the pres­
ent disease, and also regarding the patient ' s
opinion o f the success o r failure o f the opera­
tion.
Complaints of orthopedic patients should
be classified as follows :
� Deformity. This refers to an abnormal
shape or size of the body or parts of the
body. It results in complaints in the maj or­
ity of the cases, but it sometimes causes
only esthetic, cosmetic problems . A high
proportion of deformities develop during
life (e.g. scoliosis, funnel chest), and
18 � 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ic s
therefore it is important to learn the time of
onset, which often differs from the time it
is first observed. Parents often note only
the increase in a deformity, which stresses
the importance of screening tests for the
early detection of deformities.
� Pain. A very significant symptom in the
orthopedic diseases is the musculoskeletal
pain. The patient seeks medical help
because of the pain. Pain sensitivity is
individual, and the intensity of pain is
regulated by a number of factors. In
infants and children, pain is manifested by
the avoidance of using the given limb, i.e.
restriction of movement. The pain is most
often located in the affected part of the
body. In other cases, e.g. in conditions
relating to nerve compression, the pain is
experienced distant from the pressure
area, in the receptor zone of the sensitive
nerve (referring pain) . The diseases of
j oints, postural deformities of the feet may
also cause pain which presents elsewhere
(e.g. knee pain caused by osteoarthritis of
the hip, femoral pain caused by flatfoot) .
In a number of cases the patient senses the
pain in one spot or one well-defined area,
but in other cases the pain is diffuse, in
some cases the location of the pain may
change. Pain related to movement,
weight-bearing is very likely to originate
from the joints and to be of musculo­
skeletal origin, while pain independent
from motion may accompany other
diseases of bones. Pain related to j oint
illnesses may be relieved or cease in a
certain position (most often in the
functional midposition) of the j oint,
whereas the intensive pain of tension type
related to inflammation or tumors of the
bones are independent of the position of
the body. The nature of the pain may be
sharp, stabbing, dull, splitting or oftension
type. If it is accompanied by paresthesia or
other sensitivity changes, consideration
should be given primarily to nerve
pressure. A circulatory disturbance may
be suspected if the above symptoms are
accompanied by changes in color and
temperature of the given part of the body
or the limb.
� Swelling. Pain may draw the attention of
the patient to the swelling of some body
part, but the swelling may be painless.
Pathological effusion in peripheral joints
causes fusiform swelling of the area of the
joint. It is important to determine if the
swelling involves one or more joints. The
quantity of joint fluid is usually well
observed by the patient, who can give
valuable information on this subject. The
first symptom of malignant bone and soft
tissue tumors is swelling, which is usually
progressIve.
� Atrophy. When this arises, patient notices
that one or more limb or part of a limb is
becoming thinner. The reason for this is
the atrophy of the musculature. It is
necessary to establish, when this process
started, how rapidly it has progressed and
whether it is accompanied by a loss in
strength. It is essential to differentiate
between the situations when the patient
noticed signs of paralysis (loss of
function), or only muscle atrophy related
to deficient activity (after plaster fixation,
pain, joint contracture or ankylosis) .
� Restriction of range of motion. One very
frequent complaint is when the patient
feels that certain motions are restricted, or
that certain movements have become
impossible, or only with the help of
supplemental motions. For instance the
patient can no longer flex the elbow, and is
therefore unable to reach his/her face, or
can not move up or down to stairs, etc.
� Gait disturbances, limping. Diseases of
the lower limbs can be accompanied by
walking difficulties, which may be
manifested by shortening of the walking
distance and difficulties in climbing stairs.
The patient explains those difficulties in
terms of pain and fatigue experience
during walking. It is important to know
when the limping started. If the patient has
limped since childhood, congenital or
pediatric illness may be suspected; if the
limping started only some days earlier, the
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s
problem may b e acute. Patients often
mention that they limp only occasionally,
following long tiring walks . Limping is
often noticed by the relatives, and not by
the patient.
� A limb length difference causes comp­
laints almost exclusively in the lower
limbs. Limb length difference occurring
and progressing in childhood may reflect a
growth deficit of the limb (or overgrowth
of the other limb), but may also be
considered joint contractures (especially
in hip joints).
4.2. Examination
For an accurate assessment of the condi­
tion of a patient it is essential to perform a full
and detailed examination.
Important observations can be made while
the patient is undressing or dressing. It should
be noted which movements are painful, ab­
normal, and how certain tasks are performed
(e.g. compensatory motions during removal
of stockings).
The body height and weight are recorded
in all cases.
The examination is divided into the fol­
lowing phases.
4.2.1. Inspection
The undressed patient is inspected from
every direction while standing, and while
walking, which provides information regard­
ing the patient' s constitution, the state of
nourishment, and any visible shape changes
and deformities. Even mild muscle atrophy or
swellings should be subjected to careful ob­
servation. Thorough attention should be paid
to the col or of the skin, changes in color
(moles, brown spots, inflammation, redness,
cyanotic color related to vein congestion,
etc.), and visible changes in the superficial ve­
nous system (dilated, static veins) . Scars are
to be explored with special care. Linear scars
indicate cuts or surgical interventions. Wide,
� 19
more or less retracted scars point to secondary
wound healing, and comparatively long sup­
purative processes. Changes in the hair and
nails are often helpful guides to find the right
diagnosis.
4.2.2. Palpation
The temperature of a given area may be
felt by palpation. In this case the back of the
hand is to be preferred as a receptor, since its
heat perception is better than that of the palm.
The examiner' s hand is placed lightly on the
area to be examined and the temperature there
is compared with that of the environment.
When limbs are palpated, this test must be
comparative. It is important to determine if a
given area is painful or whether pain can be
provoked by applying pressure. A cool or cold
feeling of a limb or part of it may lead to the
suspicion of a circulatory problem. Apart
from the temperature, a wet (perspiration) or
dry skin may also be of significance. The skin
temperature is measured exactly with the ap­
propriate equipment.
Palpation can give information on the state
of the tissues, collection or loss of fluid. The
palpation is initially performed lightly and su­
perficially to determine the state of the skin
and subcutaneous tissue (fat) . During this ex­
amination the pathways of the superficial
veins, the pulse of the arteries, and the re­
gional lymph glands are palpated. Nodules
and glands palpable in the subcutis may draw
the attention to benign fat lumps (lipomas),
connective tissue lumps (fibromas) and lumps
originating from the sheaths of peripheral
nerves (neurofibromas) . It must be carefully
observed if these lumps are painful.
The muscles, tendons, deeply-located
nerves, bones and joints are examined by
stronger, deeper palpation. Palpation may fur­
nish information on the state of the muscles
(flaccid, spastic). Myalgic nodules are often
palpable in the substance of muscles the so
called, and the region of origin of the muscles
can also be painful. In case of inflammation
the tendon sheaths are swollen and painful to
20 � 4. Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics

pressure. Cartilaginous - bony apposlhons � valgus: the longitudinal axis of a distal part
and tumors are characterized by being immo­ of a limb deviates laterally from the
bile above the bony basement and are usually proximal part of the limb in the frontal
hard to palpation. plane. In the elbow and knee some degree
of valgus is normal.
� varus: the longitudinal axis of a distal part
of a limb deviates from the proximal part
4.2.3. Alignment, axial deformities
of the limb in the frontal plane, in the
of the limbs direction of the midline of the body.
� recurvated: the distal part of a limb deviates
The alignment of limbs is observed by in the extensive extension direction
comparison in a neutral position. (sagittal deformity).
If the longitudinal axis of a distal part of a � antecurvated: a sagittal deformity opposite
limb corresponds to the axis of the proximal to manner to recurvated one with
axis, the classification is a 0 degree (normal) anteriorly convex curvature of the limb;
axis. The different situations are denoted by � torsion (rotation) position: rotation of parts
expressions as follows (Fig. 4. 1 .) : of limbs around the longitudinal axis.

The extent of an axial deformity is charac­

terized by the angle of the axes. The measure­
ment is made during the physical examination
of the patient, but accurate evaluation requires
the use of x-ray films. The reasons for axial
\. -' deviations are the curvature of the bones of
the limb, or more rarely the deformity of the
joint surfaces and loosening of the ligaments.
The alignment of the lower limbs is deter­
mined in with the patient in a standing posi­
tion as well, and its extent is evaluated.

4.2.4. Examination of the joints

Before examination of the joints the
a b
broader area of the j oint is thoroughly in­
Fig. 4.1. spected with respect to changes in shape,
Genu valg u m (a), varum (b) and recu rvatu m (c) deformities, swelling.

35-45�• .
O'

45 : ..
,� 5R:
�
---
.. . ..
(
..
':
� :�
O'

'\ �,. .45'

,,'
2!2lS
60_80 : .
...
.�
O'

: ...

-
.
. . . 60-80 ·

.
-*-, , , . . . . .
- � ' , � , ' ...
.
, "

' .:.. ' � :

b
. c
.
a

Fig. 4.2.
Flexion of the cervical spine, forward - backward (a), lateral (b) and rotation (c)
 4. Exa m i n a t i o n m e t h o d s in o rt h o p e d i c s � 21
o· o· 30 .
�, 3"
· ·

I. . � �
o. . . ..

2" b
� o· 30 · _40 ·
:--; '

Fig. 4.3.
Forward flexion of the trunk, with hip flexion (a), backward flexion of the trunk (b), lateral flex ion of the trunk
(c) and rotation (d)
150- 170 ·

o·
70·
b

o·

135 ·
_0·
_,, 40-60 ·
-- o ·

d e

Fig. 4.4.
Abduction and elevation of the shoulder (a), external rotation over 90 degrees with rotation of the shoulder

and internal rotation in the neutral position of the shoulder (e) and i n 90 deg rees of abduction (f).
blade (b), Flexion and extension of the shou lder (c), flexion and extension in the horizontal plane (d). External
 22 � 4 . Exa m i n at i o n m e t h o d s i n o rt ho p e d i cs

90 °

_ 0°
- - -' - 80- 9 0 ° 80 -90 °
10 °

Fig. 4.5.
Flexion and extension of the elbow (a), supination and pronation of forearm (b)

_ 35 -60 °

"
00
0°
\3
--- � �- -- -- ��
..
�: : :
'. ... ... - _ ... ... - .
: : : ' 90 0 o·
\!J;;;; �
" 50 - 60 ° , 100 ° , 90 °

a c

0°

25-30 : 3 0 - 40 °

b c

Fig. 4.6.
Extension and flexion of the wrist (a), u l na r and radial deviation (b). Flexion of the metacarpophalangeal and
interphalangeal joints measured by angle and by the d istance of the fingertip (c)
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s � 23

130- 140 ·

�mn'. o·

o·
90 ·

:
��
2 .
o·

_____ 30-40 0
4 0 - 5 0 · ----....-,-
-'

,
,,'
80 .
\ :
:
, ,

, ..

30 - 45 ·

d e

Fig. 4.7.

in extension (c) and flexed to 90 degrees (d), external and internal rotation in extension (e) and flexed to 90
Flexion and extension of the hip tested in the supine (a) and the lateral position (b). Abduction and add uction;

degrees (f).

_ - - - - 5 - 10 ·
• • �ooO: :: : : : : :::�� �- � �- � _ ....-0.

1 2 0 - 150 ·

Fig. 4.8. Fig. 4.9.

Flexion and extension of the knee. Flexion and extension of the a n kle.

Fluid collections in superficially located rection of the other hand which senses the
joints with a loose capsule may expand the flow. Ballottement of fluid accumulation in
capsule. Depending on the quantity fluid may joints covered by thick musculature cannot be
be demonstrated in a j oint, if the examiner detected by this method.
places both hands on the j oint, and exerts a Bursae surrounding j oints, sometimes
sudden pressure is elicited with one hand. connected to their cavity may also be palpated
This causes any fluid present to flow in the di- in forms of various elastic lumps.
24 � 4. Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
0°
,
"
:-';\ a
a k-i
16 ° 20 °
A B
Rg7" 4.10.
Eversion and inversion of the foot (A), pronation and supination (8)
4.2.5. Range of motion of joints
(ROM)
The range of motion of a joint is described
in the main planes of motion.
First the passive movement is tested. This
always starts with the examination of the
symptomless joints in the opposite limb.
Movement of the j oint may be painful. If so, it
is important to record, which direction and
which part ofthe ROM causes pain for the pa­
tient. Pain is most common at the endpoints of
the range of motion, but it may be present
throughout the entire motion. The patient re­
acts to pain elicited by motion with a defen­
sive muscle action.
The ROM of a j oint motion may differ
from individual to individual under normal
circumstances. The ROM in children is gener­
ally larger than in adults . The outcome of the
ROM test is evaluated relative to the normal
values for the joints on the other side.
The ROM is determined by the angle in
the main motion planes assessed with a
goniometer. The extent of the motion is re­
corded for the neutral (0 degree) system. The
ROM of the spine and fingers can be ex­
pressed in centimeters . The ROMs of individ­
ual j oints are displayed on fig 4.2 to 4. 1 0. Ex-
35 °
" .
/1 '. -;-Y <r< ,1 0°
' a � a
,
,
tension in excess 0° can be denoted as
hyperextension. ROM of a particular joint in a
single plane may also be recorded by that
starting at one end and passing through 0° to
the other end, e.g. the abduction - adduction
of the hip is 1 0° - 0° - 1 5 ° .
A t the passive ROM o f a joint the values
may differ from the normal (average) in two
ways:
- ROM exceeding the average
- ROM decrease (contracture).
When ROM is greater than the average, it
is important to learn, if this is the case in every
direction or only in one particular plane, and
whether this phenomenon is present in one or
more joints. If many j oint exhibit an excessive
ROM, joint laxity is likely, which is often a
symptom of connective tissue disease. If the
direction or extent of motion is abnormal, this
is called pathological movement. The etiol­
ogy usually involves tearing or stretching of
the limiting ligaments. A joint ROM decrease
(contracture) may occur in one or more direc­
tions of movement. The extent of the
contracture is characterized by giving the two
endpoints . E.g. if the elbow ROM in flexion ­
extension is 3 0- 1 50 degrees, this means that
the extension is limited, lacking the range be-
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s
- - - - - - - - - - - - - - - - -
Fig. 4.11.
Thomas test to detect flex ion contracture of the hip.
tween 0-30 degrees. Since the mo tion of the
forearm s tops in flexion, in this case the con­
di tion is called a flex ion con trac ture. If the
flexion is limi ted, ex tension con trac ture is
presen t.
For the de tec tion of a flexion con trac ture
of the hip, tes t technique of Thomas is
employed. When maximal flexion is a ttained
on the uninvolved side, the o ther thigh will lift
up from the examining table. The angle mea­
sured from the plane of the table is the ex ten t
of the hip flexion con trac ture (Fig. 4. 1 1 .).
Accordingly the con trac tures by types are
flexion, ex tension, abduc tion, adduc tion, ex­
ternal and in ternal ro ta tion con tractures.
The causes of con trac tures :
- scarring o f the skin,
- cons tric tion, paralysis of muscles,
- changes in the j oin t and capsule.
A to tal loss of joint mo tion (ankylosis) de­
velops when a bony union or connec tive tis­
sue adhesion preven ts movemen t. In cases of
ankylosis, the posi tion of the j oin t mus t be de­
termined.
The ac tive mo tion of a join t is assessed to
es tablish the ROM when the patien t uses his /
� 25
her own muscula ture. I t is eviden t tha t the ac­
tive ROM can no t be larger than the passive
one. I t is essen tial to observe any possible oc­
casional compensa tory or supplemen tary
movemen ts.
A reduc tion or to tal loss of ac tive mo tion
is a consequence of weakness or lack of the
func tion of the muscle(s). The ac tive move­
men t is evaluated to es tablish which func tions
of the muscles are diminished or missing.
Pain related to the func tion of the muscle (e.g.
tenosynovi tis) may also cause a decline in the
ac tive ROM.
4.2.6. Assessment of muscle power
The power of synergic or individual mus­
cles may be classified by physical assessmen t.
The muscle power is graded from 5 to 0:
5: normal power (full function even against
g reat resistance),
4: muscle function only against limited
resistance,
3: muscle function against the action of
g ravity,
2: muscle function only in the absence of the
action of g ravity,
1: only fibrillation is visible,
0: total paralysis.
Muscle func tion may be analyzed VIa
elec tromyogram (EMG) assessmen t.
4.2.7. Assessment of muscle tone
The resis tance of the muscle agains t pas­
sive s tre tching (muscle tone) may be normal
(normo tonia), decreased (hypomyo tonia) or
increased (hypermyo tonia).
In flaccid myo tonia, s tre tching in the di­
rec tion opposi te to the muscle func tion is
easy, in case of hypermyo tonia, i t is difficul t.
4.2.8. Limb length assessment
The real leng th of a limb is agreed by con­
sensus to be the dis tance be tween the easily
palpable bony elemen ts. Limb leng th mea-
26 � 4. Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics

•
\t
"
"
"
"
"
"
"
"
"
"
,,
, ,

If J¥- �
, ,
, ,
, ,
, ,
, ,
, ,
,

: '

15 cm

T I--Q--\ j /�
---- - -------

25 cm
Fig. 4.12.
Leg length measurement between the a nterior iliac
spine and the inner - outer a n kle. Comparative
measu rement of the circumference at identical dis­
tances from fixed points.
surements are performed with the limbs in ex­
tended position; or if this is impossible, the
limbs are positioned symmetrically and the
measurement is made by using intermediate
points .
The real length of up per limbs is measured
between the acromion and the styloid process
of the radius . The intermediate point may be
the lateral epicondyle of the humerus.
The real length of lower limbs is measured
between the anterior iliac spine and the medial
ankle . The intermediate point may be the me-
Fig. 4.13.
� t
Apparent shortening and lengthening of the lower
limbs.
Above: Abduction contracture of the right hip; when
lower limbs are parallel, this limb will be longer. The
child is compensating by bending the knee. Below:
Adduction contracture in the right hip. When lower
limbs are parallel, the right limb will be shorter. The
child is compensating by bending the opposite
knee.
dial epicondyle of the femur or the patella
(Fig. 4.1 2.).
Apparent shortening or lengthening is pri­
marily a sequel of joint contractures or of
ankylosis of the lower limb joints . If the lower
limbs are parallel, in case of adduction
contracture of the hip the limb will be shorter,
in the event of abduction contracture it will be
longer (Fig. 4.1 3.).
Functional shortening: series of shoe
raises are placed under the patient' s footwear
with the patient in standing position. The pa­
tient is then asked to indicate the height at
which he/she feels that the limbs are of the
same length and loaded equally. The length
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
discrepancy detected in this manner i s called
the functional shortening. This is the elevation
is needed to correct the limb shortening (e.g.
by wearing an orthopedic shoe), since the op­
timal limb function is ensured in this way. In
cases of intact spine the functional shortening
is 0,5- 1 ,5 cm less than the real or apparent
shortening, this can be compensated by lateral
compensatory curvature of the lumbar area.
4.2.9. Measurement of limb
circu mference
An important element in the patient as­
sessment is the objective circumference mea­
surement of limbs. Theoretical\y any part of
the limb could be selected for comparative
circumference measurements, however it is
advised to perform this in accordance with an­
atomical parameters .
Changes occurring in the substance of a
muscle are best measured in the muscle belly
area (the mid third of the arm, the proximal
third ofthe forearm, 1 5 cm above the tip ofthe
patel la, the proximal third of the calf, etc.).
Joint circumference measured to evaluate
the intra- and periarticular swellings .
The results of circumference measure­
ments are recorded either as absolute values
or as differences in cm-s, the site of the mea­
surement also being noted (e.g. 1 5 cm above
the tip of patel\a). Since this assessment is
comparative, it must be performed with the
muscles in the same state of contraction, and
in the identical section of the limbs (Fig.
4.12.).
4.3. The standing position
and the gait
4.3. 1. Standing position
In the standing position the body is sup­
ported by the lower limbs and is at apparent
rest. The entire weight is loaded onto the sur­
face of the sole that is in contact with the
ground. The support points of the feet, the
� 27
Fig. 4.14.
S u pported areas of the feet.
soles and the surface between them form the
support surface (Fig. 4.1 4.). The relatively
stable position of the standing body is ensured
by the musculature that is involved in support­
ing the posture. The loading line of the body
then passes through the middle of the support
surface. In a resting, balanced standing posi­
tion, the loading line is in the midline of the
body; looking from the side, it runs from the
middle of the ears through the shoulder, hip
and knee j oints and reaches the supporting
surface in front of the ankles (Fig. 4.1 5.). The
muscular function ensures that the centers of
gravity of given organs (partial centers of
gravity) match the loading line. The center of
gravity of the part of the body above the lower
limbs (the head, the trunk, the upper limbs) is
located in the lesser pelvis in front of the sec­
ond sacral vertebra.
In various postures, the loading line re­
mains within the supporting surface, but is
transferred anteriorly or posteriorly (Fig.
4.1 6.). In these positions the body is still bal­
anced, but maintenance of this balance re­
quires more extensive and intensive use of
muscles compared to the normal, comfortable
standing.
The muscle power applied is dependent on
the posture, e.g. a position of "military atten­
tion" requires about 20% more energy than
28 � 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
Fig. 4.15.
Loading line in the case of a balanced
posture.
the normal posture. A fully relaxed standing
demands even 1 0% less energy, since the liga­
ments play a maj or part in maintaining the
posture. During standing the neural system
guides the posture and coordinates the muscle
function. Important roles are played by the
proprioceptive, vestibular and neck position­
ing reflexes and the optical information to­
gether with the extrapyramidal system.
4.3.2. Gait
During walking, the entire body moves
continuously forward through alternating mo­
tion of the lower limbs. The decisive element
in the gait is the movement of the lower limbs,
which is accompanied by the well-coor­
dinated motion of the trunk, upper limbs and
head. These motions are characteristic of the
Fig. 4. 16.
Changes in loading line in different postures.
gait. It is possible to differentiate normal,
esthetically spectacular, or special, abnormal,
but still not pathological gait patterns. During
walking the body is first displaced from its
"stable" standing position, and then regains
the lost balance, this process occurring repeat­
edly. The gait may therefore be defined as a
process in which the balance of the body is
lost and then regained.
This is a cyclic process, certain phases fol­
lowing each other repeatedly in an identical
fashion. This includes the repeated periodic
motion of each individual limb and the coor­
dinated periodic motion of the two limbs to­
gether. In gait analysis, the basic unit is the in­
terval between identical positions. A step is
taken to mean the period of motion of the limb
from one heel strike till the next heel strike.
The gait cycle is characterized by timing and
length.
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s � 29

Phases of the gait

The step is divided by definition into two

phases:
• stance phase
• swing phase

The stance phase lasts from the moment

the heel touches the ground until the foot lifts
off the ground. The swing phase lasts from
moment of the lift-off until the heel again co­
mes into contact with the ground. During
walking at a comfortable pace 60% of the cy­
cle is in the stance, and 40% in the swing
phase.

The stance phase

In the stance phase, the body weight is
loaded onto the supported limb, the trunk con­
tinuously progresses and the foot is supported
on the ground (Fig. 4.1 7.).
This phase can be divided into 3 stages :
- heel strike : The hip is in flexion, the
knee in extension, the ankle in plantar
flexion.
- rolling: Gradually the entire sole
touches the ground, while the trunk is
continuously progressing above the
foot. As soon as the trunk overtakes the
ankle j oint, the heel gradually lifts off
the ground and the load is transferred to
the anterior part of the foot.
- lift-off: As the result of the active
extension of the limb (this corresponds
to the plantar flexion on the foot) the
foot lifts off the ground.
Fig. 4.17.
Process of the stance phase.
of the distal part ofthe limb the trunk proceeds
continuously above the limb (Fig. 4.1 8.).
The swing phase may be divided into two
stages :
- limb shortening,
- limb lengthening.
At the start of the swing phase, the limb is
behind the trunk and swings forward due to
the flexion motions of the limb. While passing
below the trunk it gradually becomes longer
due to the extension of the knee, and the foot
goes into plantar flex ion and prepares for the
heel strike.

Even before the heel strike a braking mus­

cle function is initiated, which increases from
the moment of heel strike and lasts till the
trunk reaches a position above the ankle joint.

The swing phase

From the moment of lift-off the weight of

the trunk is loaded onto the opposite limb and
the swinging limb proceeds forward above the Fig. 4.18.
ground. Together with the sweeping motion Process of the swing phase.
30 � 4 . Exa m i n at i o n m e t h o d s i n o rt h o p e d ics
Because of the differences in the time peri­
ods of stance and swing phases there are mo­
ments during the gait, when both limbs are
supported: one is still standing, whereas the
other already in the stance phase; this is re­
ferred to as a double stance.
The gait is assessed both on flat surface
and on stairs . If possible, the patient should
walk without appliances (a stick or cane), so
as to allow an assessment of the ability to walk
without walking aids.
At the beginning of the assessment, the pa­
tient walks at a spontaneous pace, and the
character of the gait, the step length and the
width of the gait are observed.
It is important to assess if the nature of the
gait is different during fast or slow walking,
what new component appears in the motion.
In cases involving a minor hip flexion
contracture for example the patient is able to
walk normally with short steps, but ifthe walk
is accelerated and the increase in step length
requires full extension, a marked limp may be
detected.
For the measurement of step length the pa­
tient walks steadily and slowly, and the dis­
tance between the sites of two heel strikes is
determined on both sides. The absolute dis­
tance is validated, since the step length will
obviously be identical bilaterally.
To assess the motion of one lower limb
relative to the other, the term step distance is
used. To assess this, - while the patent is step­
ping forward in a standing position, the dis­
tance between the heels or the distance be­
tween the heel of the forward-stepping foot
and the front point of the foot left behind
should be determined. Assessment is carried
out bilaterally. The result always refers to the
foot positioned forward.
The walking width is the distance between
the paths of the two lower limbs. The distance
between the points at which the heels hit the
ground is used to measure this. Increased
walking width improves the safety of walk­
ing, so it may be considered as an indicator of
an imbalanced gait. A decreased walking
width is not necessarily normal, in of bilateral
adduction contracture of the hip the lower
limbs approach each other, and may even
cross each other.
4.4. Limping
When the gait is disturbed, so that the mo­
tion of the lower limbs is no longer sym­
metrical, or the motion cycles of the lower
limbs differ, the patient is said to limp. The
extent of a limp can vary from the barely no­
ticeable form to a severe gait disturbance.
The reason for a limp may be:
� pain,
� ROM restriction of the joints,
� weakness or loss of muscle power,
�
limb shortening,
� joint instability.
A protective limp may occur if any joint or
loaded area in a lower limb develops pain in
response to loading or moving. It is a general
feature of a protective limp that the stance
phase becomes shorter on the affected side, as
does the swing phase on the opposite side.
Typically, the patient loads the involved limb
in a protective constraint position (e.g. abduc­
ted hip). The shorter stance phase results in a
decreased ROM of the joints.
A restriction of a joint ROM results in a
limp, if the ROM needed for gait is involved.
A mild restriction of joint motion may not re­
sult in a limp during slow walking; the limp
may be observed only during fast walking
with long steps.
The total loss of joint motion (ankylosis)
always causes limp, the nature and extent of
which are mainly dependent on which joint is
involved (hip, knee, ankle, etc.) and in what
position.
A limp resulting from weakness or paraly­
sis of the muscle power originates from the
impaired function of the muscles involved in
the gait. If the abductor muscles of the hip
j oint (m. gluteus medius and minimus) are un­
able to support the pelvis in stance phase, be­
cause of muscle weakness, the pelvis will sag
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
Fig. 4.19.
Loading force exerted on the hip, when the center
of gravity is above the swinging limb (S center of
= information on the location (nerve or
gravity, T body weight, R load exerted on the
= =
femoral head, M abductor force).
=
on the opposite side (Trendelenburg ' s sign,
Fig. 4.19.).
Shortening of a limb may also produce a
limp, the extent of which is approximately
proportional to the degree of shortening.
Compensation of shortening is possible by
lateral bending of the lumbar spine, with low­
ering of the pelvis on the side of the shorter
lower limb and/or a flexion (equinus) position
of the foot.
In the event of joint instability a limp de­
velops since the support is insecure in the
stance phase. Walking both up and down
stairs must also to be observed, since the nec­
essary j oint motions and functions differ in
these two cases.
4.5. Neurological examinations
A number of orthopedic conditions are
caused by neurological system impairments;
� 31
in other cases, musculoskeletal changes pro­
duce neurological symptoms.
Sensitivity, reflexes and motor functions
are routinely assessed. As regards the sensor
function attention is paid to the differentiation
of the segmental (dermatomes) and peripheral
nerve-type sensitivity changes (see anatomy) .
The assessment of motor function also re­
quires a knowledge of the segmental and pe­
ripheral nerve supply. The proprioceptive and
foreign and vegetative reflexes are evaluated
and interpreted too in the knowledge of the re­
flex arch.
Special neurological examinations
In the diagnosis ofnerve and muscle disor­
ders assessment of the reactions to electrical
stimuli is a basic procedure.
� Electromyography (EMG): Qualitative
analysis of the electrical activity yields
muscle) and type of the pathology.
� Electroneurography (ENG): Measure­
ment of the speed of conduction in
peripheral nerves may be used to
determine the location and type of the
neural damage.
� Progression neurology: Early and repea­
ted special neurological examinations per­
mit the differentiation normal or impaired
psycho-senso-motor development of the
newboms and infants. Recognition of the
early signs of damage of the neural system
ensures a possibility for successful early
complex support (motion and cognitive
therapy) .
4.6. Laboratory tests
Qualitative and quantitative changes in
various blood components help to diagnose
many orthopedic conditions. Acute inflam­
matory diseases increase the number of white
blood cells (WEC), while chronic inflamma­
tion rather cause increase in the number of
lymphocytes, the sedimentation rate increases
32 � 4 . Exa m i n at i o n m e t h o d s i n o rt h o p e d ics
in both cases. Changes in the serum proteins
components (gamma-globulin increase) can
be precisely monitored by separating the pro­
tein fractions (electrophoresis). A greatly ele­
vated sedimentation rate without WBC in­
crease may signal a malignancy. C reactive
protein (CRP) is a good monitor of inflamma­
tion.
Streptococcus infection is accompanied
by an increase in the level of anti-strepto­
lysine titer (AST) produced by the organism.
In the diagnosis of rheumatoid arthritis, it is
vital to find the rheuma factor (e.g. the latex
test).
Regarding serum electrolyte tests, the se­
rum Ca and P levels are the most common
monitors, of the changes in the Ca and P me­
tabolism. This metabolism can be monitored
only by means of lengthy loading tests, which
reveal not only numerical changes, but also
the uptake of Ca and P and their excretion in
the urine.
Of the enzyme tests increased level of
alkalic phosphatase (related to osteolytic pro­
cesses), and increased creatine - phospho­
kinase level (progressive muscular dystro­
phy) are to be mentioned.
An increased serum urate level is an im­
portant indicator in the diagnosis of gout.
Apart from the basic urine tests, the detec­
tion of a number of materials characteristic of
some metabolic disease can be of orthopedic
significance (e.g. alkaptonuria, mucopoly­
saccharidoses ) .
A n impaired neurohumoral regulation i s
present i n some orthopedic conditions, and
therefore special hormone tests are performed
in these cases.
Cerebrospinal fluid tests are needed for
certain differential diagnoses, chiefly the cel­
lular elements, protein and sugar levels are
tested.
To clarify the nature of the disease, tests of
the synovial fluid can be of great help.
Normally, j oint drainage is clear, transparent,
straw - yellow, mildly viscous fluid, with a
low count of leukocytes and a low protein
content. It may become bloody following a
j oint injury, or a fracture of the epiphyses. It
becomes cloudy in purulent arthritis and the
agent can be cultured from it.
Cultures or animal inoculation can con­
firm tuberculotic arthritis. Bacteriological
tests can identify the agent and its antibiotic
sensitivity.
4.7. ArthroscoPl
This is a diagnostic and therapeutic
method; mainly the inner aspects of the
wider-spaced joints are examined. Arthro­
scopy is used most often in connection with
problems of the knee, and (in decreasing fre­
quency) the shoulder, hip elbow, ankle.
4.8. Histology tests
If the tests mentioned above do not satis­
factorily clarify the nature of the changes
causing the complaints of the patient, a speci­
men biopsied from the area in question is sub­
j ected to histological analysis. The specimen
can be obtained via a small operation or by
percutaneous trocar biopsy. Among others,
histology is essential to decide if a tumor is
malignant or benign (see also chapter 2 1 ) .
4.9. Imaging modalities
4.9.1. X-ray tests, special X-ray
investigations
Imaging procedures play extremely im­
portant roles in the diagnosis, and follow-up
of various conditions and in the evaluation of
the outcome of the treatment in orthopedics,
in musculoskeletal diseases. At least one im­
aging procedure is utilized in 80-90% of the
cases, and often a number of different types of
tests are involved.
A variety of imaging procedures have
been developed to depict different normal or
pathological conditions. The referring physi­
cian must be familiar with the possibilities to
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
request the test most appropriate for provision
the most information. The sequence of tests
should be planned with regard to the clinical
picture and the diagnostic possibilities. In un­
certain cases, consultation with the radiologist
should be initiated. It is customary to start
with traditional x-ray. The use of imaging
procedures is important from the aspect of ef­
ficacy, but the costs are far from negligible.
Certain methods may subj ect the patient to a
higher radiation load (e.g. CT), which can
possibly be avoided or reduced by careful
planning.
The various imaging methods :
� Traditional x-ray examination :
- Subjects : Bones, j oints : shape, Size,
position, axis,
- motion ! / fluoroscopy, functional films /
- structure of the bones and extent of the
pathology:
- entire skeleton
- one j oint or bone
- one part of a bone
� Traditional x-ray test using contrast:
- Arthrography, fistulography, angio­
graphy, an rarely lymphography.
The other imaging methods yield more in­
formation:
� ultrasonography: soft tissues, superficial
bone surfaces, vessel supply
� Isotope tests : bone scintigraphy, single
photon emission computed tomography
(SPECT), positron emission tomography
(PET), rather function (osteoblast activity,
blood supply, localization), than
morphology, and are usually capable to
reveal extremely early changes : e.g. the
dissemination of diseases.
� CT (computer tomography) :
- Structure of bone and cortical de­
struction, the border of pathology,
- shape and structure of soft tissues;
- with the use of contrast material: vessel
supply, CT myelography is possible
- 3 dimension image reconstruction
� 33
� MRI (magnetic resonance image) :
- detailed structure of soft tissues and the
medullary cavity, vessel and nerve
supply, refers to histological structure
Traditional x-ray examination is the
first choice in musculoskeletal, orthopedic
diseases. It frequently supplies sufficient in­
formation for the diagnosis, or determines the
order and sequence of further tests. Even the
negative result is important, since certain
changes can be excluded.
At least bidirectional views of the area in­
volved are needed (Fig 4.20.), so as to deter­
mine the position of the changes in 3 dimen­
sion. If this is not enough, supplementary
views are used. To differentiate between mi­
nor pathologic and normal conditions, consid­
ering the nature of orthopedic diseases (limb
length discrepancies or systemic illnesses),
comparative views are necessary.
The technical quality of the x-ray film is
very important even in the simplest cases, so
that certain changes are not overlooked. In
musculoskeletal diseases functional views are
b
a
Fig. 4.20.
a. Antero-posterior view of the left hip: THR in a
good position
b. The lateral view reveals, however, that the tip of
the stem protrudes from the bone backwards.
34 � 4 . Exa m i n at i o n m e t h o d s i n o rt h o p e d ics

Fig. 4.21.
Fu nctional image: 12-year old boy; anteflexion during standing (a), normal position during standing (b) and
retroflexion (c) pictu re of lumbo-sacral area; spondylolisthesis at LV. vertebra. Slippage of LV. vertebra mea­
sured in various positions exceeds 5%, the spine is unstable.

often needed for the detection of spinal insta­
bility and the exact localization of joints in
different ROM), and they are essential in the
planning for corrective operations (Fig.
4.2 1 .) .
cance of these test has lessened, since direct
and indirect eT and MR with contrast mate­
rial provide much more information, the pro­
cedures are simpler and complications arising

Traditional fluoroscopy provides the a b

only means of examining the position ofjoints
and bones during motion in an unloaded or
loaded state. Essential information can be ob­
tained by fluoroscopy from vague sublux­
ations, instability, mobile loose bodies of
j oints, pseudoarthroses, and implants sus­
pected of loosening. In certain cases overlap­
ping areas and bones concealed by implanted
metal ware can be simply differentiated, and
their 3 dimensional location determined.
Provocation tests may also to be employed: on
application of pressure to the bone instabili­
ties can be visualized.
In certain orthopedic conditions special
films may be necessary both in children and
adults (Fig. 4.22.).
Fig. 4.22.
Osteochondroma originating from the inner surface
Tomography is used only exceptionally,
when eT is not possible, e.g. ifthe presence of
of the shoulder blade and progressing in the direc­

a large metal implant rules out a eT test.

Traditional musculoskeletal x-ray tests
involving the use of contrast medium : In the
era of modem imaging techniques the signifi-
tion of the ribs.
a. Conventional a-p x-ray examination leads to the
suspicion of a calcified area i n the aspect of the
scapula.
b. In the projected x-ray examination of the scapula
the attached osteochondroma is clearly visible.
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s
from the contrast material are less frequent.
These tests are feasible, when the modem im­
aging procedures can not be performed, or are
not available.
Arthrography: This is used to detect bod­
ies of not-bony material in the j oints. Menis­
cus ruptures, labrum deformities, j oint cap­
sule tears, pathologic swellings and ruptures
of joint cavities and bursae, synovial abnor­
malities and changes in the joint cartilage are
well detectable.
Fistulography: The draining sinus is
filled with contrast medium. At present this is
used only if the ultrasonography can not un­
ambiguously reveal the extent and shape of
the sinus and its relation to the bone and j oint.
Myelography: contrast injected into the
liquor space to diagnose space reducing pro­
cesses of the spinal canal. In the presence of
eT and MR it is used extremely rarely.
Angiography : The blood vessels are visu­
alized using contrast medium. This is used
only rarely as a diagnostic tool. Selective or
super selective angiography is performed �s
part of the therapy, e.g. in preparation for sur­
gery to stop the blood supply of tumors, in
embolization, or to promote the application of
Fig. 4.23.
Ultrasonography image: Swelling of the dorsum of
the foot in a 68 year old female. The tendon of
extensor dig. longum muscle has already diminished
(1,6 mm), and is surrou nded by fluid. Tendinitis,
peritendinitis.
� 35
drugs in limited areas (regional chemother­
apy).
Lymphography: The lymph vessels are
filled with contrast medium. This technique is
out of date.
The clinical examinations lead to the con­
dition being classified into a certain group of
diseases after which a decision is made as to
the best choice of imaging procedures, and
when and in what sequence they should be
carried out.
4.9.2. Muscu loskeletal
u ltrasonography:
X-ray techniques only provide limited
possibilities for the checks on soft tissues. The
visualization of calcifications is convincing,
and maj or soft tissue swellings can also be es­
timated, but the soft tissues investigations re­
quire ultrasonography. This method is highly
dependent on the technique, and requires
skill. It has the advantages that no ionizing ra­
diation is involved, it is simple and can be
used repeatedly useable, and it can replace
painful or costly examination (positioned
x-ray test, MR). During motion, functional
tests furnish additional information, similarly
as for fluoroscopy during bone screening.
Further ultrasound-guided interventions can
be performed with high level of accuracy (e.g.
biopsy).
Besides 2D pictures with color-Doppler
and power-Doppler tests the major and minor
vessels, the circulation, and the curves of ves­
sels can also be detected.
Frequent indications:
� Trauma: partial or complete rupture of
ligaments, tendons, muscles, and the
possibility ofjoint instability; in children a
slipped capital epiphysis, and the sus­
picion of greenstick fracture, blood
collections following trauma.
� Suspected fluid accumulations: bursitis,
tendonitis (Fig 4.23.), arthritis, purulent
36 � 4. Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
Fig. 4.24.
3 phase bone scan (courtesy of dr. Gyorke). I n all three phases an increased isotope uptake is seen in the dis­
tal metaphysis of the right femur.
Jomt inflammations, hemophilic bleed­
ings. Abscesses, sinuses, hematomas can
be detected.
� Swellings, inflammations: these may be
of various origins. Swelling can be caused
by inflammation, a soft tissue tumor, or
fluid collections. Information can be
obtained on the vascularity, relation to
large vessels and the environment of the
latter.
� Available cartilage and bone surfaces :
the thickness, structure, IllJunes of
cartilage can be tested; loose bodies are
generally detected. Hip j oint dysplasia and
dislocation in infants can be diagnosed in
age of 4-6 weeks. The efficacy of treat­
ment can also be tested. The available
surfaces of bones, and possibly the
thickenings of the periosteum may be
visible (callus, bleeding, pus, tumor) .
� Peripheral nerves can be located with
high resolution equipment, these should
be examined in tunnel syndromes or
compression symptoms of other origin.
4.9.3. Isotope procedu res
The most frequent isotope test procedure
is bone scintigraphy (bone scan). The most
frequently used radioactive materials are
complex phosphate molecules.
The efficacy of investigations of changes
localized in a given region of the body can be
greatly enhanced by three phased bone scinti­
graphy. In this procedure, during the intrave­
nous application of a radioactive phosphate
complex the lesion in question is placed be­
low the center of the viewing field of the
gamma camera, and serial exposures are made
(Fig. 4.24.). In cases of osteomyelitis, arthri-
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d i c s � 37

tis, bone tumors containing hypervascularized

areas and metastases this is the most sensitive
procedure, facilitating an early diagnosis most
effectively. The test is not specific for either
malignancy or inflammation. It rather indi­
cates whether there are any bone changes or
the number of sites at which changes are de­
tected (metastasis, polyarthritis).
There may be a discordance between posi­
tive clinical signs and a negative bone scan
morphological imaging result. In these cases
bone marrow scintigraphy may be performed
as a supplementary test.
Isotope testing is common, but the PET
(positron emission tomography) is not yet ev­
erywhere available in Hungary. Most often
the 1 8fluoro-desoxy-glukose (FDG) PET is
used, this is the only method that differenti­
ates between inflammation and a tumor in­
duced metabolism increase. PET : 1 8 F-timidin
and l l C-timidin tests, can visualize DNA and
the cell cycle, and have higher specificity.
4.9.4. Cl scanning
The most appropriate of the many tech­
niques of computer tomography must be se­
lected in order to answer a particular clinical
question. For a fast test (helical CT) so as to
avoid dilution of the contrast medium in
blood, high-contrast visualization of ves­
sel/not-vessel structures is necessary. For the
more accurate imaging of morphological de­
tails slower but more extensive data collec­
tion, with pictures of very high quality
Fig. 4.2S.
HRG, high resolution G: osteochondritis dissecans
at the posterior joint su rface of the talus. The well
visualized bone trabecu lar system and the dissected
piece have not been displaced but are already de­
marcated by a semilunar sclerotic margin.
(HRCT: high-resolution CT) are required
(Fig 4.25.).
One of the most recent technical advances
is the multislice MDCT (multi detector row
CT). This system provides very fast imaging
with extremely thin slices ( 1 mm) of very
large body volumes. Diagnostic angiography
can be avoided, because even the fine vessel
structure in a tumor can be precisely visual­
ized. MDCT has restored arthrography to the
arsenal of diagnostic tools. It has been intro­
duced in doubtful MRI cases as a supplemen­
tary test for the evaluation of intraarticular
trauma cases (knee meniscus, shoulder
labrum injuries).

Table 4.2.
Signal intensities of different materials in MRI
TI-weighted T2-weighted Proton-density Fat suppression

strong no signal strong weak no signal strong weak strong no signal

signal signal signal signal signal Signal
fat water water fat bound free water water fat
water
Gd air air fat
calcium calcium
iron iron
38 � 4 . Exa m i n at i o n m e t h o d s i n o rt h o p e d i cs
4.9.5. MRI
In the process of MRI, which depends on
the traditional spin echo (SE) triggering and
acquisition parameters the distribution and
binding qualities of hydrogen are visualized
via differences in signal intensity (Table
4.2.).
Detailed additional measurements can be
carried out by gradient echo (GRE) tech­
niques to evaluate cartilage material and the
matrix. Sequences are included to separate the
fat and water signals and to be able to decide if
the vertebral lesion is caused by metastasis or
osteoporosis (Fig. 4.26).
MRI tests as well as CT examinations can
involve the intravenous inj ection of contrast
material. The faster measuring possibilities of
modem MRI equipments have resulted in new
advantages. During dynamic contrast exami­
nations many images are generated in one
second, and records can be obtained from des­
ignated sites. This is mostly important in
cases of recurrent or residual tumors.
Fig. 4.26.
MRI image of increased T2 signaling from Ll II. vertebral body deformed because of foreign tissue (metasta­
sis).
The relations of bones, surrounding soft
tissues and possible skip lesions can be visual­
ized in cases of known tumors . A knowledge
of the involvement of major vessels, the
subcutis and the skin is also essential. For the
differential diagnosis of swollen lymph
glands T2 contrast media have been devel­
oped. These contain USPIO: ultra small par­
ticle iron oxide, which accumulate in nor­
mal-structured lymph glands, but not in meta­
static lymph glands.
4.9.6. I nvasive and interventional
radiology
Angiography and angio-intervention
The traditional image intensifying catheter
angiography has been replaced by MDCTA
(multi detector CT angiography). The catheter
introduced into any vessel is still used if it has
a some therapeutic value.
The ideal method of tissue sampling is
open surgical biopsy. With the help of an im­
aging method (most frequently ultrasono-
 4 . Exa m i n a t i o n m e t h o d s i n o rt h o p e d ics
graphy) fine needle aspiration biopsy (FNAB)
can be performed, or a tissue cylinder can be
obtained by means of thick needle (a core nee­
dle or a thru-cut biopsy).
In special cases of pain-killing (anti­
inflammatory treatment by the means of
intra-articular injections, imaging techniques
may be required. An example is inflamed
osteoarthritic changes in the facet j oints of
spine, when an image intensifier and contrast
material control may aid the accurate expo­
sure.
An ultrasound guided percutaneous
puncture may be utilized to administer local
anti-inflammatory injections, or more re­
cently to treat a fresh rupture of Achilles ten­
don; for the repair of local wound, adhesives
can be applied, so that open surgery may be
avoided in lucky cases.
� 39
Vertebroplasty is a new, fashionable mo­
dality of intraosseal intervention performed
with a thick needle. Under an image intensi­
fier the metastatic or osteoporotic compressed
vertebral body is exposed from trans­
peduncular approach. A thick path is drilled
and contrast material mixed with bone cement
is inj ected slowly with a proper device under
high pressure.
Thermo-ablation procedures are thermo­
destructive procedures directed by imaging
devices. The most frequent such procedure at
present is for the treatment of osteoid
osteoma. It is also used in cases of painful
bone metastases. In non sequestrated disc pro­
lapses laser thermocoagulation of the nucleus
pulposus is mainly dealt with by neurosur­
geons.
 Ta mas Mesza ros

5. Surgical therapy in orthopedics

Orthopedic disorders may be treated surgi­
cally or by conservative methods. The likeli­
hood of surgical interventions has increased
in the recent decades due to new surgical pro­
cedures, and the advances in operative safety
(antibiotics, developments in anesthesiology).
However although operative procedures pre­
dominate, conservative methods remain im­
portant, and they are still used extensively.
The surgical procedures applied in ortho­
pedics are classified into the following types:
5. 1. Muscle - tendon operations
Tenotomies are performed to solve joint
contractures caused by muscle shrinkage. The
tendon of the muscle is lengthened in most
cases by Z tenotomy : it is cut half longitudi­
nally, a the pathological state of the j oint is
corrected, and the parts of the tendon are then
united by stitches in the shifted position (Fig.
5.1.) . Following the operation a plaster cast is
applied in the corrected position for 3 -4
weeks.
Certain muscles having a large apo­
neurosis can be lengthened by incising trans­
versely the aponeurosis or in a V shape (m.
gastocnemius, biceps femoris, semimembra­
nosus recession). Tenotomies are carried out
most often from an open exposure (open
tenotomy), but in selected cases a trans­
cutaneous procedure is done with special
sharp device (tenotome) (subcutaneous
tenotomy). The advantage of open tenotomy is
that it is safe and the optimal extent oflength­
ening can be achieved.
During the transposition of muscles (ten­
dons) different muscles are transferred so as
to be able to replace the weakened or missing
functions in their new position. It is most ap­
propriate to change the path of a muscle by re­
locating its attachment to ensure its altered
function (Fig. 5.2.). Plaster casts are applied

a b c

Fig. 5.2.
Transfer of the tibialis anterior muscle to the second metatarsal
bone: Dissected at its attachment (a), withdrawn to an incision
Fig. S.l. of the leg (b), transferred to the basis of the second metatarsal
Z-shaped tenotomy bone (first cuniform) and fixed in a drilled hole (c).
42 � 5 . S u rg i c a l t h e ra p y i n o rt h o p e d ics
until appropriate firm reattachment (3 -4
weeks), though stimulation ofthe muscle may
be started earlier. Early motion can avoid ad­
hesion of the transferred muscle. An injured
or missing tendon can be replaced by a func­
tionally less significant tendon (e.g. m.
palmaris longus) or a conserved tendon.
Scarring and shrinking of tendon sheaths
may hinder the muscle function, occasionally
it may be painful, longitudinal incision or ex­
cision of the scarred part is therefore per­
formed.
The free motion of the tendon is also en­
sured by early exercise.
5.2. Fascia surgery
Scarring and shrinking of the superficial
fascia obstructs the motion. Accordingly the
incision of tense bands, and the partial or total
excision of the shrunk part of the fascia is per­
formed.
5.3. Bone operations
Operations on bones include the excision
of exostoses or other bony parts (ostectomy),
the removal of centers of inflammation or
other types of pathologic changes (ex­
cochleation), and other routine interventions.
In an osteotomy the bone is cut with a
chisel or saw. The main aims are to correct de­
formities, to relieve pain in degenerative joint
diseases and to correct joint instabilities.
In corrective osteotomies, the required ax­
ial correction is achieved by removing a bone
wedge of appropriate shape (Fig. 5.3). Osteo­
tomy is done linearly in a transverse or
oblique direction, or arched depending to the
aim and the location of the correction. Re­
garding to the direction of the correction,
varus, valgus, rotation, flexion, extension
osteotomies can be differentiated. Multidirec­
tional correction is also possible. Correction
of limb length, with elongation or shortening,
is also accomplished by osteotomies.
Osteotomies performed for joint instabili­
ties have the aim of changing the shape and
depth of the articular cavity so as to improve
the attachment and stability of the joint head
(e.g. pelvis osteotomy in dysplastic hip
joints) .
If it is possible to ensure fast callus forma­
tion, the preferred site of osteotomies is the
metaphyseal area; some other site is elected if
the maximal deformity ( e.g. curve) is else­
where. After the osteotomy, the adequate ad­
aptation of the bone ends ensured over the
largest possible surface and fixation is applied
until callus formation has occurred. De­
pending on the location and type of the
osteotomy the fixation may be internal or ex­
ternal or some combination of the two. Inter­
nal fixation is achieved with metal plates and
screws and other special devices. These usu­
ally result in motion-stabile fixation (osteo­
synthesis), permitting early mobilization. An
intramedullary nail provides loading-stabile
fixation.
External fixation can be achieved with
plaster casts, occasionally plaster slabs or an
external fixation device (fixateur externe).
This is primarily indicated if fast callus for­
mation is expected at the site of osteotomy or
if internal fixation would comprise an unac­
ceptably great intervention.
External and internal fixation are applied
in combination in cases where the fixation
achieved is only motion-stabile, but the oper­
ated limb is to be loaded.
Epiphyseodesis. The epiphysis can be
fixed to the metaphysis by bridging the
growth plate with a metal plate, metal nail,
screw or staple. This can be used to inhibit the
growth fully or asymmetrically.
5.4. Operations on J_oo__in
_t_s ____
The therapy may necessitate joint punc­
ture. Pain related to tension of the capsule can
be relieved by the evacuation of collection in
the joint and the damage to the hyaline carti­
lage can be prevented. Joint puncture is to be
done in sterile and aseptic conditions simi­
larly to the operations .
Arthroscopy can solve certain pathologic
conditions of joints (e.g. removal of loose
 5. S u rg i ca l t h e r a p y in o rt h o p e d ics � 43

a b
Fig. 5.3.
Various forms of correction osteotomies and fixation with a compression device (a) and a self-compression
plate (b).

bodies, meniscus removal). Other therapeutic
uses are on the increase (cruciate replacement,
acromioplasty) .
Arthrolysis involves the release ofthe j oint
from the adhered capsule or the division of fi­
brous adhesions, which can be performed by
either arthrotomy or arthroscopy.
Arthrotomy is the exposure of a j oint cav­
ity. It is employed as an independent modality
to remove loose bodies, but in fact it is the first
phase of all joint explorations .
Synovectomy is the partial or total
exstirpation of the synovial layer of a j oint
capsule. It is done to solve chronic inflamma­
tion of the synovial liner.
Incision of the capsule (capsulotomy) is
done if a joint capsule participates in the per­
sistence of a contracture. This intervention is
44 � 5 . S u r g i c a l t h e r a p y i n o rt h o p e d i c s
limited t o certain joints because of the risk of
dislocation, or to only certain areas of j oints
(e.g. to save the integrity of the collateral liga­
ments) .
Reconstruction and plastic surgery ofjoint
ligaments and capsule. In order to ensure the
normal mechanism ofjoints occasionally with
replacement by a tendon, reconstruction of
elongated or tom ligaments or the capsule, or
some other method may become necessary.
Surgical repositioning. In cases of
subluxation or dislocation, the joint reduction
may be conducted with a visual check-up after
exposure. To ensure the position, plasty of the
extended capsule (narrowing, duplication)
may be performed.
A rthrorisis. The j oint ROM decrease by
producing a bony bumper.
A rthodesis. Joints are operatively stiffened
to relieve pain, to correct a deformity or insta­
bility. The joint surfaces are flattened, and the
limited painful motion is sacrificed in order to
achieve a painless stiff joint which is func­
tional and can be loaded painlessly.
Instability, luxation, subluxation can oc­
cur following damage to joint components or
in diseases causing muscle paralysis. In such
cases arthrodesis of the slack, malfunctioning
joint(s) results in a functional improvement of
the entire limb. This is rarely done because of
the ever greater expensive use of arthroplasty.
In developmental abnormalities and ac­
quired diseases of the spine, various extents of
arthrodesis may be necessary (spinal fusion,
spondylodesis). During arthrodesis the j oint is
often exposed, hyaline is resected and the
bony surfaces are matched and accurately
fixed (intraarticular arthrodesis) (Fig. 5.4.).
The bone ends are held in position by internal
and external fixing methods until bony union
has occurred (3-6 month). As a general rule
arthrodesis is avoided in children, until the
end of growth if possible since it disturbs the
progression of the epiphyses.
Great care must be taken as to the position
of the joint, because a good limb function can
be achieved only if the j oint is stiffened in the
optimal position.
5.5. Arthroplastl'.
This is an operation, in which joint surfaces,
epiphyses are newly formed or replaced, or
epiphyses are removed to improve the mo­
tions of a joint. The aim of the surgery may
also be to decrease or eliminate pain.
Types:
� Plastic resection. Reshaping of the epi­
physes may result in a wider (perhaps
slack) range of motion. An interposed flap
between the epiphyses may improve the
chances of pain-free motion. Resection of
one epiphysis (e.g. resection of the head of
the radius) may improve the joint
movements (resection arthroplasty).
� Endoprostheses.
� The most up-to-date type of arthroplasty,
ensuring the best function involves the
insertion of endoprostheses, manufactured
from tissue-friendly materials to replace a
damaged j oint. In recent decades there has
been an increase in the numbers of the
orthopedic interventions happened
specifically because of the endoprosthesis
operations . Nowadays almost all of our
joints can be replaced by endoprostheses.
� Endoprostheses made of various tissue­
friendly metals (various stainless steel
alloys, titanium and alloys) have excellent
static and dynamic load-bearing pro­
perties . Among the plastics, the ultra high
molecular weight polyethylene
(UHMWPE) has proved best and is used
as an element of almost all endo­
prostheses. Its advantage is its elasticity,
which ensures the elasticity of loading
especially during walking in cases of
lower limb endoprostheses. Its dis­
advantage is wear of the surface, since the
worn particles induce the production of
granulation tissue, bone resorption and
ultimately loosening of the prosthesis.
Bioceramics are excellent tissue-friendly
materials with a high resistance to wear.
 5. S u r g ica l t h e ra p y in o rt h o p e d ics � 45

a b

Fig. 5.4.
Arthrodesis procedures. Knee (a), hindfoot (talocalcanear and Chopart) joint arthrodesis (b), external fixation,
compression following knee arthrodesis (c), intraarticu lar hip arthrodesis fixed with 2 nails (d), ischiofemoral
(Brittain-type) extraarticular hip arthrodesis (e).

They have the occasional disadvantage of - Surface-replacing endoprostheses are

breakage or cracking which limits their
use.
� Different forms of endoprostheses have
been designed for different types of the
joint damage:
employed when only the joint surface
and the close surrounding area are
damaged (e.g. cup prostheses).
- Prostheses replacing the ends of bones
are inserted in cases of extensive
damage.
46 � 5 . S u rg i c a l t he r a py i n o rt h o p e d ics
- Tumor prostheses can be applied if
tumors cause maj or damage in the bones
of a joint.
There are a large number of variants of
endoprostheses with particular areas of appli­
cation. Two basic types may be mentioned:
- In hemiarthroplasty, when only one part
of the j oint is replaced, whereas in total
arthroplasty both joint surfaces are
replaced. In orthopedic diseases total
arthroplasty is usual. Total endopros­
theses should ensure an almost normal
range of motion relative to the original
joint, and in certain j oints the stability of
the joint / limb. A suitable prosthesis is
selected with the regard to the state of
Fig. 5.5.
Hip prosthesis with smooth surface for cemented
use.
the capsule, ligaments and stabilizing
muscles. E.g. if the joint capsule and li­
gament system is damaged, joint
movement and stability is achieved by
limited (constrained) endoprosthesis. In
other cases a free range endoprosthesis
(of non-constrained type) is suitable to
provide motion and stability.
The endoprostheses must match the body
and bone size of the patients. Accordingly se­
ries of each prosthesis type are available and
the proper size is selected. Modular
endoprostheses have recently become popu­
lar, where implants built up from optimally
sized elements are inserted.
It is extremely important, that the elements
of the endoprostheses must be fixed firmly to
the bone yet during the operation. The fixa­
tion method of the prostheses to the bones is
extremely important, depending primarily on
the type of the joint and the structure of the
bone.
The most common technique is the bone
cement fixation (Fig. 5.5.). If the prosthesis
has a special surface, cementless fixation is
possible (Fig. 5.6.). The long-lasting stable
fixation of cementless elements of prostheses
is achieved by the new cancellous bone grow­
ing into the uneven surface of the prosthesis.
This anchor is sometimes only partial, thus to
Fig. 5.6.
Cementless hip replacement removed because of
indifferent reason. The surface has a special, rough
finish, the cancellous bone may grow in to provide
secondary fixation for the prosthesis.

Fig. 5.7.
Cementless hip replacement with a hydroxyapatit
surface.
improve the chances of fixation, various sur­
face designs and biologically active surfaces
(e.g. hydroxyapatit) are used (Fig. 5.7.).
Depending on the circumstances, there
may be naturally a possibility to fix one
element of the total prosthesis with cement,
and insert the other element in a cementless
manner (hybrid fixation) .
Implantation of endoprostheses may allow
pain-free joint motion, but the functional out-
5. S u rg i ca l t h e r a p y in o rt h o p e d i c s � 47
come depends considerably o n the state o f the
muscles (contracture, motor power etc.) and
on the postoperative motion.
A late problem with endoprostheses is
aseptic loosening, which can occur in some
cases a few years after the operation. Bone re­
sorption may occur in the prosthesis - bone
interface, when the prosthesis may work loose
and elicit pain during walking.
The partial or complete exchange of loos­
ened endoprostheses can usually be effected
with special revision prostheses, occasionally
supplemented by a bone transplant. If the in­
sertion of a new prosthesis is technically im­
possible, removal of the loose prosthesis re­
sults in a pseudoarticulation (Girdlestone ' s
state), and the patient will b e ambulatory but
will need a walking aid and his / her life style
will be limited.
5.6. Operation on nerves
Decompression operations are done when
the peripheral nerve becomes compressed,
leading to pain, sensory or motor disturbance.
In the most common situation the lumbar spi­
nal nerves are compressed at the spinal canal
or at the foramen by a prolapsed intervertebral
disc. Several peripheral sections of the nerves
may be compressed by the stricture of muscle,
fascia or bony tunnels (scarring, inflamma­
tion) or tumors.
Denervation. An increased muscle tone
and spasm may be lessened by transection of
the motor nerve.
 Zo lta n Cse r n a t o n y

6. Conservative treatment procedures

In orthopedics, conservative and surgical

treatment modalities are complementary. The
bulk of conservative treatment is included in
physiotherapy, that is the competence of
physiotherapists. Some conservative methods
however, belong among the activities of phy­
SIClans.

6. 1. Immobilization
This may be required to alleviate pain, to a

cure infection processes, for the regeneration b

of tissues injured during operations or acci­
dents.

Bed rest. This is the simplest way to

achieve immobilization. A normal or hard bed
is often satisfactory, but support of the limb
involved (a pillow, Braun frame), use of a
high or low headrest or neck support, or sup­
port of the dorsal or lumbar spine may be nec­
essary. For immobilization of the spine, plas­
ter bed may be used, which is a plaster shell c
fitted to the posterior trunk.

Plaster slab. either the entire limb or part

of it can be immobilized or fixed with a plaster
slab. The advantages of this are, that it is ap­
plied immediately following a fracture or op­
eration, the circulation of the limb can be as­
sessed and the compression of the bandage
can be lessened, the wound is visible and the
limb can be cooled. Synthetic immobilizing
slabs can be prepared or purchased for a simi­
Fig. 6.1.
lar purpose.
Plaster cast for clubfoot in a baby: a felt band is at­
tached over the pressu re areas (a), the lower limb is
Circular plaster. This provides more sta­ covered with soft cotton (b), and a circular plaster is
ble fixation than a plaster slab. Both its appli- applied (c).
50 � 6 . Co n s e rvat i v e t reat m e n t p roced u re s
cation and its use require great caution. The
patient must be warned about the symptoms
of compression (swelling, blue skin, pain,
hypoesthesia) and its dangers (ischemic limb
damages, pressure sores). After lengthy use,
when the soft tissue swelling has subsided and
inactivity muscle atrophy has set in, the circu­
lar plaster cast may become loose, and require
replacement. It is very important is to protect
the skin; insertion of felt over the areas sub­
j ected to pressure (Fig. 6. 1 . a-c) and the pad­
ding.
Special immobilization. Depending on
the body part and indication a number of ap­
pliances are used for full or partial immobili­
zation or support (Fig. 6.2.), which are dis­
cussed in detail in chap . 1 0.
Traction (extension treatment). Relative
immobilization can be accomplished by the
traction of the limbs. In orthopedic traction,
a b
Fig. 6.2.
An air-cast fixation device, used to treat partially torn ligament in the ankle, permitting motion exclusively in
the sagittal plane.
a} Schematic drawing of a lateral ankle ligament tear.
b} Pneu matic ankle cuff (air-cast), a-p view.
c} Lateral view.
mainly soft-tissue traction is employed, when
the traction force necessary for immobiliza­
tion is applied via a rope fixed to the skin by a
cuff or adhesive. This method solves cont­
ractures caused by increased muscle spasm,
the musculature around inflamed painful hip
and knee j oints can be relaxed. A common ap­
plication area is in the hip and knee osteo­
arthritis. In such cases traction with a weight
of 2 kg is advised for 3 hours twice a day.
The other large group involves skeletal
traction, when a Kirschner wire is inserted
into a certain bone (calcaneus, tibial tubercle,
olecranon), and kept under tension by an ap­
propriate device. It has the advantages, that it
does not harm the soft tissues and is more pre­
cise than the soft tissue traction (axis, angle,
force). Disadvantage is that it is invasive,
there is a risk of infection and extensive bed
rest is necessary. It is used only rarely in or­
thopedic practice, chiefly as part of the preop­
erative preparations.
c
 6 . Co n s e rv a t i v e t re a t m e n t p roced u re s
6.2. Mobilization
In cases of restriction of the ROM of
joints, when a muscular spasm or the fear of
the patient maintains the restriction, mobiliza­
tion may be performed under general or local,
regional anesthesia. If the restriction is
arthrogenic, in selected cases the method of
brisement force (forced mobilization) can be
tried. This demands be done great care be­
cause of the risk of vascular or nerve injury or
bone fracture.
6.3. Non-weight-bearing
There are a number of illnesses and condi­
tions, in which full or partial non-weight­
bearing of the lower limbs or the spine is re­
quired.
The lower limbs. To relieve the load-bear­
ing of the lower limbs during walking, appli­
ances can be worn on the body of the patient (a
tuber supporting brace, condyle supporting
orthesis) .
Another method to relieve load bearing
during walking is the use of walking aids (a
walking frame, crutches, stick).
The spine. Total immobilization of the
spine can be achieved only by bed rest (if
necessary, supplemented by longitudinal trac­
tion, a plaster bed, ortheses) .
6.4. Irradiation
In orthopedic therapy x-ray irradiation is
utilized for 3 purposes:
Irradiation of malignant tumors (see
chapter 2 1 ).
� 51
Prevention of heterotopic ossification.
Bone formation in the muscles or around the
joints may occur following hip arthroplasty,
or in hemiplegic patients following damage to
the central nervous system. In high-risk pa­
tients a daily dose of 1 00 R to the areas in­
volved is advised for 3-5 days. In hip surgery
the development of periarticular ossification
can be effectively prevented by non-steroid
anti-inflammatory drugs.
Pain relief. The use of x-ray irradiation
for this purpose is gradually decreasing. In
case of osteoarthritis 50- 1 00 R may be indi­
cated.
6.5. Laser therapy
While high performance lasers of are of
use in surgery, low performance lasers (soft
lasers) are employed for their anti-in­
flammatory, pain-reducing and wound- heal­
ing effects. Their use is based on empirical
knowledge; their mechanism of action has not
been fully elucidated yet.
6.6. Pharmaceutical treatment
Pharmaceutical treatment of value in or­
thopedic therapy for very different indica­
tions. Organotropic (directed to a certain or­
gan), etiotropic (directed to the etiology), lo­
cal, systemic, symptomatic and causative
forms of pharmaceutical treatment are all
used.
Table 6.1. lists those drugs, which may be
considered for the conservative treatment of
orthopedic illnesses, according to their areas
of indication.
 VI
Table 6.1. N
Drugs in orthopedic use .....

Effect group /
'"
Active agent Products Effect mechanism Indication

n
Pharmaceutical
o
:::J
group

III
VI
Drugs on peripheral nerves
:;;:
local anesthetics lignocaine Lidocain 1% inj. reversibly blocking the stimulus elimination tests, local QJ

<
....

III
conduction capabilities of the sensitive anesthesia in surgery
bupivacaine Marcain inj. tissues (nerve fibers), so d isconnecting
....
ro
Marcain spinal heavy inj. the pain
Bupivacain i nj. QJ

3
....

III
:::J
....
articaine Ultracain inj.
Ultracain DS Forte
"0
procaine Procainium HCI 2% inj. o
III
n

c..
c
" nerve damage and injury
vitamins thiamine Vitamin B1 inj., tabl. "vitamine against neuritis , its active
ro
Milgamma N inj. metabolite takes part in carbohydrate
metabolism as a coenzyme VI
Neurobion inj.
Muscle relaxants
central activity guaifenesine Relaxil-G inj. decrease the resting tone of striated conditions causing spastic
(spinal chord) musculature without considerable tone increase of skeletal
tolperisone Mydeton tab!., inj. effect of its voluntary contraction muscles (lumbago,
chloroxazon Myoflexin tab!. contractu res, cramps,
spasticity)
carisoprodol+ Scutamil C tab!.
paracetamol
tizanidine Sirdalud tab!.
baclofen Baclofen tab!.
Lioresal tabl.
Drugs in orthopedic use
Effect group / Active agent Products Effect mechanism Indication
Pharmaceutical
group

peripheral activity mivacurium Mivacron i nj. non depolarizing neuromuscular relaxation of the skeletal
blockers, they competitively inhibit the m uscles, general anesthesia, in
pipecuronium Arduan porampulla effect of acethylcholine on the motor cases of ventillation,
vecu ronium Norcuron inj. endplate intubation

rocuronium- Esmeron i nj.

bromid
succinylcholin Succinyl-Asta inj. lasting depolarization of the motor muscle relaxation in general
Pantolax i nj. end plate, so the stimuli can not reach a nesthesia, muscle relaxation,
the muscle intubation

(j\
Non opiate pain killers
n
o
tramadol Contramal i nj., caps. opiate receptor agonist acting centrally, moderate to severe acute or
but not narcotic chronic pain :::l
ID
Adamol SR ret. caps.
Vl

<
Tramalgic i nj., caps. ....

Cl>
<'
paracetamol Benuron tab!. prostaglandin inhibition by connecting to relieve mild and moderate
ID
r+

Mexalen tab!., supp. to the cyclooxygenase enzyme pain or fever

ID
Cl>
Panadol filmtab!. r+
....

3
Rubophen tab!., supp.

ID
r+

:::l
ibuprofen Nu rofen tab!.
Advil tab!. r+

'0
Algopyrin tab!., i nj. o
ID
nor-
n

c..
aminofenazon
C
Panalgorin tab!.

ID
....
Vl

.....

V1
W
 �
\J1
Table 6.1.
Drugs in orthopedic use ......

Effect group I
0\
Active agent Products Effect mechanism Indication

n
Pharmaceutical
o
:::J
group
'"
Anti-inflammatory drugs ro
<
non-steroid ketoprophen Fastum u ng. by attaching to the cyclogenase joint, tendon or muscle pain, DJ

<
.....

ro
anti-inflammatory Profen id gel, tabl., caps. enzyme they inhibit the prostag landins inju ry, inflammation,
d rugs (NSAID) (and this way the production of prevention of periarticular
.....
ro
flurbiprofen Flugalin drg., kup inflammation and pain mediators). ossification
DJ

3
ibuprofen Solpaflex creme, tabl. .....

ro
:::J
Spedifen granu late
naproxen Apra nax filmtabl. .....
1J
Naprosyn tabl., supp. (3
ro
n

c..
c
indometacin I ndobene gel

ro
'"
I ndometacinum caps., supp.
diclofenac Catafla m tabl.
Diclac gel
Diclofenac Duo caps.
Olfen gel
Voltaren gel, tabl., supp.
methyl-salicylate Gerosan ung.
piroxicam Erazon gel
Feldene gel, caps., supp.
Hotemin creme, caps, inj., supp
phenylbutazon Phenylbutazon creme
Rheosolon tabl.
azapropazon Prolixan caps., filmtabl.
niflumin acid Donalgin caps.
flufenamin acid Mobilisin creme
Table 6.1.
Drugs in orthopedic use
Effect group / Active agent Products Effect mechanism Indication
Pharmaceutical
group
------
---

Anti-inflammatory drugs (cont.)

selective COX2 nimesulid Mesulid tabl., granu lte COX2 is inhibited in a great extent RA, osteoarthritis
inhibitors
meloxicam Movalis tabl., supp.
celecoxib Celebrex caps. COX2 is inhibited selectively i n
therapeutic dose
steroids betamethason Celestone tabl., inj. They inhibit the synthesis of Rheu matoid diseases
Diprophos inj. prostanoids by blocking the
transcription of phospholipase A2
dexamethason Oradexon tabl., inj.
0'1
gene, besides they directly inhibit the
expression of COX2 .
n
methyl- Medrol tabl.
o
These are the most effective anti-
:::J
pred nisolon Depo-Med rol inj.
inflammatory drugs, they effect both
ro
Solu-Medrol inj. In
the acute vascular, and cellular phase
Metypred tabl., inj. :;:
!l)

<
....

ro
pred nisolon Rheosolon tabl.
Pred nisolon tabl.
....
Di-Adreson F Aquosum inj. ro
!l)

3
....

ro
triamcinolon Kenalog i nj.
:::J
....
Polcortolone tabl.
----

'0

o
Agents for improving viscosity ""

ro
n

Cl.
hyaluron acid Hyalgan inj. the viscosity of the synovial tissue OA of knee, hip, shoulder
c
Fermathron inj. increases, viscoinduction, pain relief
ro
follow ""
Synvisc i nj.
In

...

VI
VI
 VI
able 6.1. 0\
Drugs in orthopedic use
.....

Effect group /
0\
Active agent Products Effect mechanism Indication

n
Pharmaceutical
o
::J
group

III
Chondroprotective drugs VI

glycose-amin- DONA it's the natural substrate of the hyaline Degenerative joint illnesses ;:
[lj

<
,....

III
sulphate proteoglycanes, stimulator of their
biosynthesis
,....
hyaline and bone Rumalon inj. stimulator of the regeneration of iD
[lj

3
extract of you ng hyaline ,....

III
::J
animals
,....
chondroitine Condrosulf powder, caps. this is one of the basic elements of the
"0

(3
sulphate hyaline, excellent water absorbent, so
III
n

Cl..
promotes the mechano-elastic qualities

c
of the cartilage

iD
VI
 Ta m a s B e n d e r
7. Physiotherapy
Physiotherapy involves the use of natural en­
ergy (thermal, mechanical, electrical) as well as
chemical effects (e.g. the a bsorption of trace
elements during balneotherapy at medical
spas) in medicine.
The most important physiotherapy methods
are:
�
exercises (mechanotherapy),
�
electrotherapy,
� heat therapy (electrotherapy together with
balneotherapy),
�
mechanotherapy (exercises, massage and
ultrasound treatment),
�
hydrotherapy
�
light therapy
�
balneotherapy.
An advantage of physiotherapy is that it
has hardly any side-effects, but it also has the
disadvantage that its effects are much less un­
derstood than those of drug therapy.
Physiotherapy endeavors to utilize the
procedures objectively, in another words evi­
dence-based medicine is also valid in physio­
therapy.
It has already been mentioned, that both
surgical interventions and conservative treat­
ment are equally important, and the conserva­
tive treatment in orthopedics includes physio­
therapy procedures. Preoperative physiother­
apy is of great significance in improving the
breathing and circulatory capacities, while
postoperative physiotherapy plays an impor­
tant role in maintaining the outcome of the op­
eration, and promoting the avoidance of the
contractures and thrombo-embolic complica­
tions.
7. 1. Exercises
The most important physiotherapy proce­
dures are exercises, which are irreplaceable.
These exercises can be active, when the pa­
tient uses his / her own muscles to carry out
the motion, or passive, when the physiothera­
pist moves the joints . During exercises, move­
ment is also used as a mechanical stimulus.
Exercises are important elements of preven­
tion, treatment and rehabilitation. During as­
sessment of range of motion the cause of any
restriction is evaluated. The causes can in­
clude pain, contracture, fluid collection,
swelling, joint block.
One of the basic tenets of motion therapy
is that pain must be avoided. Exercises can in­
volve one phase, e.g. exercising the agonistic
muscles or two phase, e.g. exercising both the
agonistic and the antagonistic muscles. Dur­
ing isometric exercises, the length of the mus­
cle is unchanged, resulting in a static muscle
load; during isotonic motion, the length of the
muscle is changed, resulting in a dynamic
muscle load.
Individual exercises should be selected, if
possible, and should be pursued as long as the
motion is manually guided. If group exercises
are advised, patients with similar capacity
should be grouped together, similarly as in
underwater exercises.
Active exercises are less risky than passive
ones following surgery, since the patient,
mainly through the onset of pain, is able to
judge the limits of his / her joint.
58 � 7. Phys i o t h e r a p y
7.2. I m mobilization, mobilization,
exercises
Fixation can have multiple aims : restrict­
ing or blocking movements, keeping the limb
in rest, etc. In different disorders, involving
inflammation plaster slab, plastic slab or cir­
cular plaster cast immobilization of the af­
fected j oint can alleviate pain and enhance
healing.
Immobilization is essential after certain
orthopedic operations, to protect the corrected
position of the j oint, e.g. following arthrodesis
or osteotomy. Adherence to the principles of
joint protection is indispensable in motion
therapy. Movements, which have disadvanta­
geous effects, increasing the pain, deformities
and causing negative changes are to be
avoided.
Preoperative exercises serve to increase
muscle power, promoting a successful surgi­
cal outcome. After Chiari pelvic osteotomy,
when the origins of gluteus medius and
minimus muscles are almost completely de­
tached, even if very regular and active exer­
cises are practiced, the hip abductor power re­
turns, and the Trendelenburg sign ceases only
after some 6 months. In cases of hip osteo­
arthritis motion therapy is designed to en­
hance gluteus muscle power. Non-weight­
bearing is achieved very well in the so called
Fig. 7.1.
Passive knee-moving device
(CPM).
Fig. 7.2.
Computer-guided traction
table, with an additional mi­
crowave heating unit.
Traction of either cervical or
the lumbar spine can be
performed.

Schlingel bed with a hanging grating : the re­
sistance is eliminated by means of ribbons in­
corporated into the grating over and at the
sides of the treatment bed.
In cases of osteoarthritis of the knee, the
aim of the exercises is to raise the quadriceps
power together with joint protection; in cases
of ligament instability a supportive brace can
be applied. Protection in cases of the very
common osteoporosis may be provided by ax­
ial loading of the spine. In cases of frozen
shoulder, when contractures are already pres­
ent, passive motion may be applied to the
maximum range, chiefly in the subacute
phase. In the chronic phase self-stretching ele­
vation exercises are ordered.
In cases of prolapsed discs, McKenzie ' s
method i s advised, based o n rearrangement of
the nucleus pulposus-annulus fibrosus. Pas­
sive motion devices (Fig. 7. 1 ., 7.2.). move the
selected joints in preset ranges and frequen­
cies continuously or periodically.
7.3. Massage
Massage is a mechanical stimulus applied
to the muscles, ligaments, soft tissues.
Massage can be performed for hygienic (in
healthy subjects), sport or therapeutic pur­
poses. Its therapeutic forms include : classical
Swedish massage with the following tech­
niques : caressing, kneading, tapping, shaking,
vibration; reflex zone massage; connective
tissue and segment massage.
7.4. Electrotherapy
Electrotherapy, i.e. the use of electricity in
medicine, is a very widespread method. In
musculoskeletal conditions it is used to re­
lieve pain. Electricity may cause hyperemia
(mainly thermotherapy; care must be taken in
cases of acute inflammations ! ) . Treatment is
applied in the form of stable low-frequency
(up to 1 000 Hz) galvanic current, longitudi­
nally placed along a limb (Kowarschik treat­
ment). This has an outstanding pain-killing ef-
7. Phys i o t h e r a p y � 59
fect, e.g. in cases of radiculitis (sciatic nerve
or brachial plexus). The current sensitivity, al­
ways dependent on the patient' s tolerance,
generally is 0. 1 mA/cm2 .
Iontophoresis involves the penetration of
pharmaceuticals into the body through the use
of electricity. The drug in ionic form slowly
penetrates through the skin into the target
area. The active ingredient may enter the cir­
culation, or the synovial fluid e.g. of knee
iontophoresis. This procedure is used for its
local effect, but it may have general effects as
well.
Transcutaneous electric nerve stimula­
tion (TENS). TENS is one of the most fre­
quently used pain-killing methods worldwide.
Its advantage is that the treatment is possible
with portable, battery-powered equipment,
which allows self treatment of the patient at
home. The background of higher-frequency
TENS treatment (up to 1 00 Hz), is the
Melzack-W all gate theory. The theory behind
the lower-frequency (2- 1 0 Hz burst or acu­
puncture-like TENS) relates to the mobiliza­
tion of the endorphins in the body.
Diadynamic current treatment: This en­
tails application of a form of current with two
components: a basic current with 1 -3 mA and
a supplementary dose current. This treatment
is a good pain killer and to a less extent in­
creases the circulation. Considering, that
many orthopedic patients have a metal im­
plant in their body, care must be taken to pro­
tect these such implants from the current.
Medium frequency alternating current:
Medium frequency treatment means therapy
in the range of 1 000- 1 00000 Hz. (Fig. 7.3.).
Apart from the classical interference, a num­
ber of medium frequency treatment modali­
ties are used for pain relief and muscle exer­
cises. In contrast with the low-frequency ther­
apy the medium frequency readily overcomes
the resistance of the skin, does not leave red­
ness, and is effective in the depths. It is used
primarily for pain-killing. Besides traditional
60 � 7. Phys i o t h e r a p y
�oo , I 00 __KLMm
�
\ii\ \i\ '
•
[IJ � -
Fig. 7.3.
I nterstim medium frequency interference treatment
device.
plain electrodes, vacuum electrodes can also
be used.
Stimulation current therapy: This is
used to treat peripheral damages of the mus­
cles in orthopedic and trauma patients, and
also to treat atrophied muscles with a good
nerve supply (muscle exercises). It is applied
most frequently before prolapsed disc sur­
gery, or to treat paralysis of peroneal, tibialis
anterior or extensor hallucis longus muscles
occurring as a surgical complication, or fol­
lowing traumatic nerve damage. The basis of
sophisticated stimulation current therapy is
electrodiagnostics.
The treatment should be repeated twice a
day if possible. Atrophied muscles with intact
nerve supply, e.g. following long bed rest or
plaster removal are stimulated with a rectan­
gular impulse.
7.5. Ultrasound treatment
The sound with a frequency over 1 6000
Hz is called ultrasound; it can be applied for
mechanotherapy. It is generated by an inverse
piezoelectric effect. Equipment generating
sound with 800 kHz-3 MHz is used in physio­
therapy. Ultrasound is conducted relatively
well by bones, but less so by soft tissues, wa­
ter and air. The intensity of ultrasound is mea­
sured in watt/cm2 . It necessitates a mediator
material (e.g. paraffin) . Its physical effect in-
clude the application of selective heat and
micromassage. It also has a biochemical ef­
fect: drugs can be infiltrated through the skin
into the organs (sonophoresis). Its biological
effects include : pain-killing, vasodilatation,
the resolution muscle spasm, e.g. softening
the connective tissue in Dupuytren
contracture. It is applied in hip osteoarthritis
in 3 fields for 3 minutes each, in degenerative
conditions of the cervical or lumbar spine, in
achillodynia or coccygeal complaints. Ultra­
sound treatment has recently been combined
with various forms of current treatments
(diadynamic, TENS, medium frequency treat­
ment), for stimulation of the deeper tissues.
The combined treatment is time-sparing, as
the two modalities may be applied at the si­
multaneously.
7.6. Pulsating electromagnetic
treatment
Views as to the clinical value of this ther­
apy differ. It is used clinically for its
osteoblast-stimulating effect. e.g. to treat re­
flex dystrophy, to enhance callus formation,
in cases of pseudo arthroses. For home use 1 00
Gauss devices with frequency range of 2 -50
Hz are used to deliver therapy, for 20-30 min­
utes.
7.7. High-frequency' treatment
High frequency treatment induces endoge­
nous heat in the body. It is used in the form of
shortwaves (27 MHz, 1 1 meter wavelength).
Shortwave therapy can take place in con­
denser or in coil fields. Modalities can be con­
tinuous or impulsive with proper frequency) .
Heat may alleviate pain in cases of chronic in­
flammations . High-frequency treatment is
usually applied daily for 1 0- 1 5 minutes. Its
use is prohibited in disturbances of heat sensi­
tivity and if there is a risk of bleeding. When
microwaves are used, the eyes (risk of cata­
ract), testicles and the growth plates must be
protected (children are not treated) .

7.S. Cryotherapy
Cold has a pain-reducing effect, which is
produced by blockade of the A-delta and C fi­
bers, reducing the speed of neural connec­
tions. Cryo-packs, the most popular practical
form. The treatment time is 1 0-1 5 minutes,
can be used at home and are easily applicable
to the skin. Cryotherapy can also be achieved
with nitrogen gas, and in a new procedure :
cooled air at minus 40 C degree is directed to
the limb for 3-5 minutes . In orthopedics, the
main application is in acute arthritis, joint in­
flammations, to relieve muscle spasm and for
pain- killing before exercises.
7.9. Balneotherapy-health spas
Balneotherapy is the branch of medicine
that uses the healing effect of medicinal wa­
ters. Its arsenal includes mud therapy and the
internal use of mineral waters . Climatic ther­
apy and cave therapy are further areas of
balneotherapy.
Hydrotherapy is a therapeutic modality
based on the physical properties of water. It is
used in every country of the world, mainly for
the treatment and rehabilitation of musculo­
skeletal conditions . It does not demand ther­
mal water; warmed tap water is suitable. The
therapeutic environment is a complex; the pa­
tient is treated in a given milieu with either
mineral-, or tap water, and the effects of other
physiotherapeutic procedures are also avail­
able, not to mention the effects of environ­
mental and climatic changes .
The physical effects of hydrotherapy are
based on three factors :
- hydrostatic pressure
- buoyancy
- temperature
Mud therapy. Mud has a large heat stor­
age capacity and a low thermal conductivity,
so it is capable of inducing endogenous heat
production (heat delivery is slow, and there is
no risk of skin bum). Chemical characteris-
7. Phys i o t h e r a p y � 61
tics: minerals present in the mud may be ab­
sorbed into the body. Applications : mud pack­
ing ( the treated limb is coated with a 2-3 cm
thick layer of mud), for 1 5-20 minutes, at 42
Co. Mud compress: the part of the body to be
treated is covered with mud heated in hot wa­
ter (only physical effects are expected, this
procedure can be applied at home). Mud lake :
this can be used at 3 8 -42 Co, the heat effect is
more marked than in water at the same tem­
perature. Mud is an efficient medium in heat
therapy, kills the pain and decreases muscle
spasm.
A weight traction bath is an underwater
traction modality. The patient is suspended at
the cervical or lumbar spine in water in a tub,
where his / her feet do not reach the bottom,
and is gradually loaded with weights (applied
to the lumbar area or to the limbs). Axial trac­
tion is applied for 1 0- 1 5 minutes to treat
discopathies, prolapsed discs, and hip or knee
contractures.
Weights are not used in cervical tractions.
Weight of 1 . 5-6 kg can gradually be increased
in lumbar traction, secured to belts applied to
the waist or to the calves. Its use is prohibited
in cases of acute j oint or nerve inflammation,
after spinal fusion or spondylolisthesis.
Subaqua) j et massage: A patient lying re­
laxed in water of indifferent temperature (34
CO), is massaged with a water j et at appropri­
ate pressure (0.5-2 atm) from a distance of 1 5
cm, for a usually 1 5 minutes treatment time.
This procedure is used to relax spastic mus­
cles.
Baths with alternating temperature:
This is used for the treatment of limbs. Two
baths are needed: one containing cool (with
1 4- 1 6 CO) and the other one warm (with 3 6- 3 8
C O ) water. Patient first submerges both limbs
in the warm water for 1 minute, and then for
1 0 seconds in the cool water. This process is
repeated ten times. The warm water causes
vasodilatation, and the cool vasoconstriction.
The carbonic acid bath exerts a marked
effect in enhancing the limb circulation, and
venous return.
62 � 7. Phys i o t h e r a py
Radon bath : radon, a noble gas with a
pain-killing effect, stimulates DNA repair,
and stimulates the immune system; it can be
combined with carbonic acid.
Sulfur bath : This causes vasodilatation,
decreases the sensitivity of the cold receptors,
and stimulates the warm receptors. It has been
assumpted by some that sulfur can penetrate
into the joint cartilage.
7. 10. Com presses and packs
Cold compress is applied for a maximum
of 1 0 minutes . A compresses soaked in 1 4- 1 6
C o cool water to treat acute inflammations. A
compress soaked in 40 Co water is warm com­
press applied for 1 5-30 minutes.
Warming (Priessnitz) compress: A textile
soaked in cool water is applied to the target
area, covered with warm sheets.
Wet-pack: Between the Priessnitz two
layers of a plastic foil is inserted.
Paraffin therapy: Paraffin is a material
softens in response to heat, and its physical
properties are similar to those of mud. It is
usually applied at 60 Co, the target area with
thin brush, and then covered with a dry sheet.
Packs : Packs differ from compresses in
being applied to major body areas. There are
trunk and limb packs, dry or wet. A wet pack
is always covered by a dry one.
Wash-down : This is the gentlest form of
hydrotherapy. Washing-down may be done
with a cool wet sponge to improve the circula­
tion. The average duration is 4-5 minutes. It is
followed by rubbing with a dry cloth. A body
part is usually washed with a rubber hose.
 Ta m a s B e n d e r
8. Manual therapy, acupuncture
Manual therapy and acupuncture have re­
cently become accepted by official medicine
as two methods of natural medicine and are
widely used in orthopedics.
Acupuncture : This technique of healing
has been performed for some 3 000 years and
this is the best-known branch of traditional
Chinese therapy. Various points of the body
are stimulated manually (with needles) or by
means of other devices (a laser beam, or elec­
trically). It is used to relieve chronic pain, and
postoperative pain and in orthopedic condi­
tions umelated to deformities. In recent years
various evidence has been published concern­
ing the effects of acupuncture (e.g. an increase
ofCSF endorphin level). The theoretical basis
of the treatment assumes the presence of me­
ridians in the body and the acupuncture points
are located along these meridians. The major­
ity of the acupuncture points have a diameter
of 0. 5-2 mm. The oriental philosophy of this
treatment is the theory of yin and yang (union
and antagonism) .
Manual therapy: This subdivision of
medicine is concerned with the biomechanics
and reversible changes of the musculoskeletal
system, the diagnostics and therapy primarily
being manual . Chiropractic and osteopathy
(synonyms) and the so called bone setting are
other terms and are also often used both by
doctors to describe manual therapy. The diag­
nostic and therapeutic procedures are used
both by doctors as well as by physiotherapists.
A j oint block of mechanical, neural or
reflectory origin that gives rise to symptoms
(pain in motion, etc.) is eliminated by special
manipulations. It is employed most often to
treat functional disturbances of a spinal seg­
ment. Manual therapy can be dangerous if is
practiced by a person without proper training
in anatomy.
 Laj o s K u l l m a n n
9. Amputation - artificial limbs
Amputation is the removal of parts of the
body. Most frequently tissues, organs or parts
of limbs are excised, usually by a su rgical
method. Exarticu lation is a special mode of
amputation, when the removal is effected
through the joints without cutting bones.
Amputation is generally considered by
both doctor and patient to be a sign of the fail­
ure of the previous treatment. The reason is
the outcome of the conspicuous loss. Never­
theless, amputation is merely an episode in
the usually lengthy treatment procedure, in
the course of some diseases (constriction of
the arteries), trauma (severe injury of a limb),
or developmental abnormalities (dysmelia,
myelodysplasia) . Amputation comprises a re­
constructive operation in this lengthy treat­
ment series, done on a functionless limb
(which otherwise occasionally j eopardizes
the patient' s life). This reconstructive proce­
dure, often together with artificial limb supply
and rehabilitation, eliminates pain, enhances
mobility, and in an ideal case may result in
full quality of life.
9.1. Lower limb amputation
The Hungarian epidemiological data indi­
cate that the conditions requiring amputations
in order of incidence are: blood vessel dis­
eases (>80%; arteriosclerosis obliterans, dia­
betic angiopathy, endarteritis obliterans), ac­
cidents « 1 4%,) primary and secondary tu­
mors « 3%), infections, limb developmental
abnormalities and miscellaneous conditions
« 4%). Amputations relating to blood vessel
diseases are most frequent between the ages
of 50 and 70 years . More than 70% of ampu­
tees are males.
9 . 1 . 1 . I ndications of lower limb
amputations
In blood vessel diseases, Fontaine phases
Ill. (pain at rest) and IV. (gangrene), ifthere is
no chance for reconstruction, amputation is
the treatment of choice. In diabetes cases sig­
nificantly more amputations are done than
necessary. Most of them could be avoided by
well-timed and proper therapy of the diabetic
foot.
Following a severe crush injury of a limb
immediate (primary) amputation is needed, if
there is no chance of reconstruction of the vi­
tality and function of the part ofthe limb distal
to the injury. (Criteria are serious damage of
three out of the five types of tissue: vessels,
nerve, muscle, bone, skin). Primary amputa­
tions performed as distal as possible, and are
to be treated openly. After the wound has set­
tled and the general condition has improved,
the final stump is formed by elective interven­
tion.
If a limb tumor can not be managed by
some other approach, amputation is needed.
In cases of secondaries amputation is also
considered if the tumor elicits unbearable pain
or severely hinders living or patient care.
Patients with chronic inflammation
(mainly osteomyelitis) and with congenital or
acquired abnormalities of the lower limbs of­
ten undergo a number of operations. Amputa­
tion is indicated, when a segment of a limb is
functionally useless or an incurable, treat­
ment-resistant infection is present which rules
out the use of appliances.
In the period of growth, amputation done
on a humerus or leg induces excessive bone
growth relative to the soft tissues. It is sensi­
ble to take this fact into account when the indi-
66 � 9 . A m p u t at i o n - a rt if ic i a l l i m bs
cation and timing of the operation are consid­
ered.
9.1.2. Su rgical technique of
amputations
Preoperatively the proper psychological
and physiotherapy preparation is indispens­
able.
Besides the general operative technique
the following aspects are to be considered:
1. The extent of the wound is controlled by a
well planned incision.
2. No foreign material a re to be inserted
except the sutures.
3. B leeding is careful ly controlled.
4. Bones are severed with a manual saw (a
framed or Gigli saw).
5. A d rain is to be inserted with the exception
of transmetatarsal, transtarsal amputations.
A 14- 16 Ch thick plastic or rubber tube is
used to ensure d rainage.
6. Extensive infections, ischemic necroses
must be exposed and treated open.
7. Wound is to be closed without tension.
Further surgical technical aspects to be
taken into account are related to the nature of
the different tissues. The blood supply of the
skin on the anterior part of the leg and on the
dorsum of the foot is poor. Deep undermining,
destruction must be avoided, the skin edges
must be protected from drying out. If the
subcutis is thick, it should be drained sepa­
rately. In arterial occlusions partial fascia ex­
cision is not done. An atraumatic technique is
especially important with muscles. Cut mus­
cles must be fixed, either to bones (myodesis)
or by stitching the antagonist muscles to­
gether over the bone (myoplasty). Muscles are
fixed by stitching their own fascia. The
periosteum is transected sharply at the level of
the bone cut. The spiky edges of bone are to be
rounded. The level the bone cut is elected so
that the stump can be covered with a proper
muscle layer and the wound above is closed
with no tension. The vessels must be severed
as distally as possible so as to protect the col­
lateral circulation. The bleeders are carefully
checked and controlled. The nerves are cut
high (3-4 cm above the operative field) so as
to avoid their reaching the scar, and the cut
ends are well covered by soft tissue. In cases
of major nerves (e.g. the sciatic) the comitant
arteries are also secured.
9. 1.3. Criteria of proper stump
Special surgical technique aspects come
into consideration at a number of amputation
levels, since they have substantial effect on
the prosthesis application.
A transmetatarsal amputation stump is to
be covered by a plantar musculocutaneous
flap, which has a good blood supply and is
more resistant to load-bearing (Fig. 9.1.).
At the transtibial level, a conventional,
long ( 1 5 - 1 7 cm) posterior fascia-musculo­
cutaneous flap (Burgess type) is approved, be­
tween the proximal and middle third. It is es­
sential to round the tibia edge and cut the fib­
ula 2 cm proximal . Removal of the soleus
muscle is recommended (Fig. 9.2.).
In an ideal case transfemoral amputation is
done between the distal and middle thirds of
the thigh. Myoplasty too may be added by at­
taching the tendons and muscles.
For hip exarticulation, the surgical inci­
sion is made along the inguinal ligament and a
long posterior flap is prepared.
Fig. 9.1.
Technique of transtarsal amputation. (The part to be
excised is shaded).
 9. A m p u t at i o n - a rt i fi c i a l l i m bs � 67

Fig. 9.3.
Dressing and fixing the transtibial amputation stump with a
dynamic knee orthesis.

After transtibial amputation, a flexion

Fig. 9.2.
Posterior flap (Burgess) technique of transtibial am­
putation. (The part to be excised is shaded).
contracture of the knee may develop, as may a
flexion and abduction hip contracture after
transfemoral amputation. These can be pre­
vented by proper positioning of the limb,
proper physiotherapy and the use of dynamic
knee extension equipment (Fig. 9.3.).
Besides stump contracture, a frequent
early complication is thrombosis, which can
be a source of emboli and delay prosthesis ap­
plication.
Amputation is often followed by stump
edema. This may be resolved by applying an
elastic bandage to the stump.
Mobilization commences postoperatively
as early as possible, depending on the state of
the patient,. It starts with breathing and limb
exercises. Exercises of the stump are per­
formed with the use of phantom sensation, en­
suring the acceptance of the prosthesis into
the body scheme. Early exercises and walking

Table 9.1.
Lower limb prostheses
Period Aim Device

From 7- 10th postop. day till the
wound healing
After wound healing till the final
form of the stump is attained
After the final form of the stump,
long term
Maintenance walking stereotypes,
walking confidence
Ensuring the formation of the
stump, and assessment of the
abilities to use an artificial limb
Provision of a walking aid serving
all demands
Temporary prosthesis with
prefabricated pneu matic
components
An easily adaptable, inexpensive
prosthesis (plaster shell, modular
system)
A modern, cosmetical prosthesis
with a good function and durable
quality
68 � 9 . A m p u tat i o n - a rt i fi c i a l l i m bs

are the correct protective measures against

phantom pain.

9.2. Lower limb prostheses

There are three different lower limb pros­
theses, which are employed in different peri­
ods with three different purposes (Table 9. 1 .).

9.2. 1. Tem porary prostheses

If the wound is healing without complica­
tions, use of the first temporary prosthesis
may start, when the stump is no longer tender.
This is the pneumatic temporary prosthesis
(Fig. 9.4.).
After the wound healing, a modular tem­
porary prosthesis is used (Fig. 9.5.).

�:::::::.--.. ( a
Fig. 9.5.
Temporary modular prosthesis with a plaster shell
---I-+--- b for the transtibial stump.

c
Advantages of temporary prostheses:

� early mobilization
� maintenance of the ambulatory ability
� faster stump modelation
� prevention of contracture
� protection of the body scheme
d � decisions related to prosthesis application
� selection of the type of final prosthesis; its
e trial of fitting

Fig. 9.4.
Schematic drawing of a pneumatic temporary pros­
thesis. Legends: a) metal frame, b) inflatable case, c)
strap preventing slipping of the case, d) height ad­
juster, e) rocker bottom.
Prosthesis application is team work. The
members of the team are the surgeon, the re­
habilitation specialist, the appliance techni­
cian and the physiotherapist.

9.2.2. Final prosthesis
This is the final aid with which to provide
a long-term and reliable function and an ac­
ceptable cosmetic outcome (Fig. 9.6.).
It is vital and ifthe answer yes, to select the
proper artificial limb. The following factors
must be taken into account:
a ---+--
c mentioned so far it might be assumed, that
b ---+--F
C --t-+
d ---\-
e -----++
Fig. 9.6.
Schematic drawing of a modular, final pipe-framed
prosthesis for transfemoral stump. Legends:
a) stump bed (case), b) artificial knee joint, c) custom
made pipe frame, d) cosmetic plastic foam lining,
e) ankle adapter, f) artificial foot.
9 . A m p u t at i o n - a rt i fi c i a l l i m bs � 69
� The a g e o f t h e patient. The elderly adapt
with g reater difficu lties, and the chances of
learning problem-free ambulation are less.
� The condition that indicated the
amputation. I n vessel disorders cardiac and
vertebra l vessels are also involved, which
influences the weight- bearing and
cooperation. I n diabetes the general
metabolic changes and other tissue
damages limit the outcome of the artificial
limb use.
� The level of amputation. The hig her the
level, the more energy is required for
ambulation with the a rtificial limb and the
more difficult to control precisely. The
proprioceptive feedback is also lower in
cases of higher amputations.
� The number of amputated limbs. The loss of
both lower limbs significantly increases the
energy consumption of gait. The loss of
one u pper limb disturbs the harmony of
walking since the motion of the lower and
upper limbs is no longer coordinated and
synchronized.
The ability to walk is influenced by the
quality of the stump.
1 . Length of the stump. From what has been
a longer stump would provide a better situ­
ation. However bone-ends in the distal
third of the leg can not be enveloped by
muscles, and the bones in this region are
tender, which may necessitate re­
amputation later. Prosthesis application
can also be difficult when the femur stump
is short « 5 cm below the lesser trochanter,
because of the excessive hip contracture.
2 . Certain conditions are particularly disad­
vantageous, e.g. contractures of the re­
maining joints of the stump, a butt or
pear-shaped stump, a scar adhering to
bone, neuroma, exostosis, infection or ten­
derness, pain of different origin, and sub­
stantial muscle weakness.
Additionally the characteristics of artifi­
cial limbs also effect the success of applica-
70 � 9. A m p utat i o n - a rt i f i c i a l l i m bs
tion. A good fit is required, with well con­
toured supporting surfaces, the shell should
not constrict (to avoid venous stagnation at
the end of the stump), and the proper length
and axial positioning.
From a practical aspect, a femoral prosthe­
sis with a knee lock is partially able to relieve
the load from the other limb, which is usually
also affected by the illness.
During the last decade there have been
substantial technical developments in the
manufacturing of artificial limbs. En­
ergy-storing and returning foot, microproces­
sor-guided pneumatic and hydraulic artificial
knee joints have been devised, which allow
motion very similar to the physiological one .
A silicone stump lining may reduce the load
from the skin, but it may also induce
dermatological problems. Exact stump bed
making is facilitated by computer-assisted de­
sign and manufacturing procedures
(CAD/CAM).
Finally it must be borne in mind, that a
successful gait may be expected only after ed­
ucation in the management of the stump and
the prosthesis, and after mastering the proper
gait pattern with the guidance of the physio­
therapist, using the other supplementary
equipment and assimilating the mental inju­
ries following amputation.
9.3. Upper limb amputation
The indication for upper limb amputation
is most frequently trauma. The loss of fingers,
especially ofthe thumb results in an enormous
loss of function.
Regarding the amputation and artificial
limb supply of upper and lower limbs, there
are two basic differences.
1 . The hand is also a sensory organ. It plays a
crucial role in sensing the outer world (tac­
tile, heat perception), and in maintaining
contact and communicating with the envi­
ronment and fellow human beings. In con­
trast with "useless" amputation levels in
the lower limbs therefore, as concerns the
upper limbs it is important to preserve ev­
ery possible centimeter that possesses
good tactility.
2. An other functional difference is, that the
upper limbs perform various, basically
different activities. Some of them involve
the exertion of great force, whereas others
demand various fine motions which are
very accurately regulated. Some motions
are made with great pulling force, which
could possibly remove the prosthesis from
the stump.
Some general points may be mentioned
here to illustrate the differences involved in
the amputation of upper and lower limbs.
� Tactile areas (mainly on the hands and
fingers should be possibly covered by skin
with preserved sensation.
� I n cases of bilateral transradial amputation,
Krukenberg plasty may be contemplated, if
the proximal half can be retained. This
means separation of the radius and ulna
and their covering with soft tissue. A
grasping ability can be achieved with the
two forearm bones.
� In case of transhu meral amputation the
butt-shaped stump is highly advantageous,
since it may prevent dislodgement of the
prosthesis. Fol lowing transhumeral
amputation in children bone may overgrow
the soft tissues necessitating reamputation.
9.4. Upper limb prosthesis
application
The number of lost limbs, the level of the
amputation and the basic condition all influ­
ence the prognosis of the use of the artificial
limb. Stumps resulting from traumas involv­
ing bums and electrocution are often very ten­
der, and huge scars may develop.
Temporary prostheses are rarely used on
upper limbs. The reason is that temporary
prostheses are less able to replace the lost

functions than are the final prostheses. In chil­
dren with a congenital upper limb deficiency
it is recommended to provide the numerous
functions in a series of steps with increasingly
more capable prostheses.
The final prostheses are produced with
mUltiple aims and with different solutions,
and may be divided basically into three types.
9.4. 1. Cosmetic prostheses
The exclusive aim of a cosmetic artificial
limb is to conceal the loss of the limb. The
cover of the prosthesis fixed to the stump de­
creases the tactile surface. Since the hand is
indispensable in maintaining interpersonal re­
lations, a supply of cosmetic artificial I imbs is
unconditionally necessary.
If a bilateral upper limb amputee has an ac­
tive artificial hand on one side, a cosmetic
a -----;r'T-
c
d ----=-
e -------U
Fig. 9.7.
Anterior and posterior views of a universal functional, mechanical upper limb prosthesis, hook. Legends: a)
shou lder strap, b) humerus support, c) bowden, d) forearm shell, e) dou ble hook.
9. A m p utat i o n - a rt i f i c i a l l i m bs � 71
hand should b e applied on the other side to
load the spine symmetrically.
9.4.2. Mechanical prostheses
Functional upper limb prostheses, i.e. me­
chanical prostheses, are powered by the pa­
tient via the muscles of the stump and/or the
remaining muscles of the shoulder girdle.
They replace the grasping force. There are
two types of artificial hands with an universal
function. One mimics the shape of the hand,
but without fulfilling the silhouette of a cos­
metic hand. It does not provide a fine pinch.
The other type has the shape of a double hook
which can be spread against the closing spring
or rubber band with the muscles of the shoul­
der girdle or elbow. Opening is ensured by
properly positioned straps and bowdens at­
tached to the hooks. This hand is popularly
"""""----\- a
b
c
d
)
72 � 9 . A m p utat i o n - a rt i f i c i a l l i m bs
named as hook. This can be considered as
functional basic artificial hand (Fig 9.7.).
9.4.3. Prostheses powered by
external force
Prostheses may be powered by external
electrical motors. These are known as electri­
cal artificial hands. Their purpose is to pro­
vide grasping with better cosmetic qualities as
compared with mechanical prostheses.
The remained limb muscles switch the
motors on and off, and to operate the controls
allow grasping with the fingers is done by.
The action potentials generated in the muscles
are normally used to control the motors, but in
some cases mechanical muscle motion can
also be utilized.
It is important, that muscle motion can
only switch the motor on and off; speed and
power can not be altered. Hence despite the
great expectations, this artificial hand is of
only a relatively modest functional value. It is
less effective, than the hook for fine motion,
to pinch small obj ects.
To educate the usage of this prosthesis re­
quire more patience than with lower limb
prostheses, because many types of motion
must be mastered. The vocational therapist
plays an even more important role than the
physiotherapist. The task is clearly more diffi­
cult if both upper limbs are lost or even if a
single limb suffers an extensive loss. Psycho­
logical support may be essential.
It must be repeated that prostheses block
the tactility of a major part of the stump. The
stump is covered by a case, which leads to a
number of inconveniences.
The above outlined modest function, and
the need to change the prostheses frequently
in childhood have the consequence that upper
limb amputees use their artificial limbs much
less frequently than do lower limb amputees.
A one-hand life style can be relatively easily
learned especially at an early age.
The situation much more difficult in cases
of bilateral upper limb amputees, but they of­
ten manage by means of simultaneous, coor­
dinated motions of both stumps and through
use of their mouth, occasionaIIy with their
feet.
 Laj o s K u l l m a n n
10. Orthoses, appliances for mobility
Orthoses are externally fitted applia nces
which support or influence the structure of
the neuro - musculo - skeletal system. Their
basic aim is to restore function.
Orthoses are generally used only tempo­
rarily in traumatology, most often in cases of
anatomically intact or surgically recon­
structed limbs, following an operation or an
injury, until full recovery is achieved.
In orthopedic conditions long-term
orthosis use is usually necessary, often ap­
plied to deformed limbs, trunk. In these cases,
it is essential that the orthosis is fitted accu­
rately to the body parts . Further, the devices
must resist deformation, it must maintain its
shape precisely and permanently.
Orthoses can be prescribed for the follow­
ing purposes.
� Lower limb length equalization with a
functional aim e.g. in cases of congenital
limb shortening.
� To provide fixation, support, e.g. in cases
of leg pseudoarthroses, or degenerative
spinal conditions.
� To limit joint motion:
- pathologic joint motions, e.g. knee
hyperextension following paralysis
- unwanted motions (mainly temporarily)
postoperatively, e.g. after cruciate liga­
ment reconstruction
- in case of conditions, such as hip
dysplasia
� To correct deformities, e.g. varus or
valgus malposition of the ankle
� To provide non-weight-bearing, e.g.
destructive processes of the vertebrae, in
Perthes disease
� To replace lost muscle function, e.g. foot
extensors
� To support muscle function, e.g. hand
flexors
In the past few decades there have been
significant changes as regards the materials
and technology used to manufacture the
orthoses.
Modem light metal and plastic orthoses
are noticeably less heavy, they are easy to
clean, they are more esthetic. Some plastics
soften in response to relatively low heat, they
can swiftly be fitted directly to the body. One
drawback of modem materials is that a num­
ber of patients perspire heavily in plastic
sockets . Care must be taken furthermore to
provide the needed firmness .
I t is often inconvenient t o wear the
orthoses, since they act to enforce corrective
effect. In general, they are conspicuous, their
wearers - specially teenagers - tend to conceal
their disorders. Patient compliance with the
use of an orthosis improves if exact instruc­
tions are given regarding the method of use,
maintenance, together with detailed informa­
tion concerning potential problems (e.g. if
some other appliance is also required), the in­
conveniences as well as the functional out­
come to be expected.
Similarly to other appliances, orthoses are
expected to meet the following expectations :
� Provide the expected function fully
� Cause the least possible inconveniences
� Be safe, and not cause injury (e.g. they
must not break)
� Be as esthetic as possible
� Be easy to use and maintain
� Be lasting (adaptation to a new o rthosis can
cause inconveniences)
� Be inexpensive
74 � 1 0 . O r t h o s e s , a p p l i a n ce s fo r m o b i l ity

It is clear that it is unlikely that all of these
expectations will be completely satisfied. It is
therefore especially important to check,
whether the prescribed orthosis meets the
functional aims .
Orthoses may cause complications (e.g.
pressure sores at the supported sites), and ex­
act instructions are to be given as to how to
prevent these, with daily observation. It is
sensible to recall the patients for repeated
checkup.
Brief information on the more important
orthosis types will now be discussed, with the
orthoses grouped by body regions.
extension of the knee which hinders walking,
may be prevented with orthoses, which permit
free flexion. Since resyriction or elimination
of inappropriate movement requires extensive
forces, the orthosis must be long enough to
distribute the load over as large a surface of
the limb as possible (Fig. 1 0.2).
10. 1.3. Knee - ankle - foot orthoses
These are applied in cases of major limb
length differences, muscle weakness, axial in­
stability of the knee, and varus and valgus de­
formities of the knee and ankle.

10. 1. Lower limb orthoses 10. 1.4. Hip orthoses

Immobilization of the hip is indicated in
These will be discussed in some detail be­
cases of hip dysplasia or dislocation. The
cause of their frequent use.
Pavlik harness (see also Chapter 28) is a strap­
ping system attached to the trunk and lower
10. 1 . 1 . Ankle - foot orthoses limbs, and it prevents extension, leaving the
other motions free.
These are partially able to relieve the load Partial immobilization of the hip may also
the ankle and hindfoot j oints. The non­ be necessary in infantile cerebral palsy (sym-
weight-bearing orthoses support the tibia
condyles and the patellar ligament.
When the peroneal muscles are paralyzed,
a peroneal slab can be fitted, attached to the
sole and the posterior part of the calf (Fig.
1 0 . 1 .) . Its lightness is an advantage, but it has
the shortcoming that it often breaks at the an­
kle level. In this event prescription of a circu­
lar orthosis is advised.
Ankle orthoses provide fixation of the an­
kle and hind foot joints and correct their
malalignment. In cases of rigid fixation a
rocker bottom shoe is needed to restore the
lost ankle motions.
In post-traumatic conditions, prefabri­
cated linen or plastic ankle - foot orthoses are
often used in variable sizes, which can pro­
vide relative fixation.

10. 1.2. Knee orthoses

These are used for temporary fixation of �
the knee. Lateral instability can be eliminated, Fig. 10.1.
while the j oint motion is undisturbed. Hyper- Peroneal slab ankle - foot orthosis.
 1 0 . O rt ho s e s , a p p l i a n c e s fo r m o b i l ity � 75

metrically, on both sides), or following dislo­ In cases of simultaneous paralysis of the

cation of a hip endoprosthesis and also in
posttraumatic states.
10. 1.5. Hip - knee - ankle - foot
orthoses
Transfer ofload bearing to the sciatic tuber
is the method to accomplish non weight bear­
ing of the hip joint. This orthosis relieves
weight bearing of the entire limb. It is applied
most often in cases of Perthes disease.
musculature of the lower limbs and the pelvis
(e.g. myelodysplasia), this orthosis may be
supplemented with a pelvic bucket, hip lock
(Fig. 1 0.3.).
10.2. Upper limb orthoses
The applications of the upper limb
orthoses will be mentioned only briefly be­
cause of their infrequent use. Motions and ac­
tivities practised in many cases in a
non-weight-bearing position. Loading can be
relieved with support, suspending devices
and/or orthoses. Trunk - arm orthoses may
also provide the non-weight-bearing of cer­
tain upper limb joints .
For upper limb orthoses modem, light ma­
terials play a decisive role, especially the ther­
moplastic and easily moldable plastics.
A substantial difference from lower limb
orthoses is the frequent use of elastic compo­
nents (plastic slabs, rubber bands, springs).
The reason for this is based on the experience,
that if certain joints are fixed elastically in
proper position, motion of the distal segments
can be more successful (Fig. 1 0.4) .
A frequently used upper limb orthosis is
the shoulder strap . In cases of negligent pos­
ture this assists retroflexion of the shoulders .
It is also applied unilaterally in paralyzed pa­
tients (e.g. after stroke), it may prevent
subluxation of the shoulder (Fig. 1 0.5.).
Functional orthoses (therapeutic devices)
may decrease harmful, symptomatic muscle
tension through use of the proprioceptive
feedback, e.g. an epicondyle pad.

Fig. 10.2. Fig. 10.3.

Knee orthosis, for Hip-knee-ankle-foot
extensor weakness, orthosis with a hip and
recurvation, and to cor­ knee lock for cases of Fig. 10.4.
rect lateral instability. extensive muscle weak­ Wrist- hand orthosis to fix the wrist and support
ness of the lower limb. grasping.
76 � 1 0 . O rt h o s e s , a p p l i a n ce s fo r m o b i l ity

�\ / -------

Fig. 10.5.
Shoulder orthosis to prevent
subluxation.

Fig. 10.6. Fig. 10.7.

Sacroiliac orthosis for degener­ Thoraco - lumbo - sacral plastic
ative spinal disorders. corsette.

The motions are normally based on reflex 10.3. 1. Lumbosacral orthoses

motions and sensory - motor feedback. This
has led to the development of hybrid orthoses,
which combine the features of traditional
orthoses and functional electric stimulation,
the actual motions conducting the further
movements. Their indication is limited and
they are expensive. They may be used only in
cases of very good cooperation.
10.3. Spinal orthoses
These are quite popular in degenerative
spinal disorders. However the extensive use
of spinal orthoses may result in atrophy of the
paravertebral muscles.
These are applied in cases of lumbar pain
or instability. They are made of linen with
various stiffeners (Fig. 1 0.6), but they are also
produced from rigid materials (metal, plastic).
10.3.2. Thoraco- Iumbo-sacral
orthoses
Linen corsertes provide relative fixation.
They are custom made from elastic fabric,
with or without breast support.
Following vertebral fracture, in
Scheuermann ' s disease and occasionally in
cases of inflammatory vertebral conditions,
immobilization of the spine and non­
weight-bearing of the vertebrae is the aim. In
 1 0 . O rt h o s e s , a p p l i a n ces fo r m o b i l ity � 77

these cases plastic trunk corsettes are fitted,

fixing a longer section of the spine. They act
on the basis of three point support. To attain
the desired correction (reclination) an the ex­
act fit on the pelvis is aspecially important
(Fig. 1 0.7) .
In scoliosis, the purpose of spine orthoses
is to achieve active muscular correction. The
orthoses must fit on the pelvis exactly and ap­
ply tender pressure to the curvatures that the
child attempts to circumvent
The manufacturing of scoliosis corsettes
\
Fig. l0.8.
demands excellent professional knowledge
Soft cervical orthosis, so called 5chanz -collar.
and skill. Faulty orthoses are occasionally
produced, the common problems may be:

� no 3 dimensional effect
� no assistance of active correction
� if too tight, or malpositioned on the trunk,
they may impair the breathing or kidney
function
10.3.3. Cervico - thoraco - lumbo -
sacral orthoses
These are indicated in cases of cervical
scoliosis, destructive vertebral diseases or pa­
ralyses.
10.3.4. Cervical orthoses
These serve for the partial or full non­
weight-bearing of the cervical spine. They are
supported on the shoulder, on the occiput and
the mandible. The Schantz collar made of
foam, provides partial fixation (Fig. 1 0.8.).
10.4. Preventive sport appliances
These orthoses are not for treatment, but
rather serve to prevent injuries.
Their use is considered unacceptable for
sportsmen, who may demand to be provided
with an orthosis for relative fixation following
insignificant injuries, so as to be able to carry
on competitive sports.
10.5. Walking aids
The handles of the devices listed below
must be adjusted to the height of the greater
trochanter. Their use must be educated. It is
important that walking with two canes re­
quires a path 70 cm wide, with the crutches
even the path is even wider. On a wet surface
the rubber bottom of the devices may slip, re­
sulting in a fall. On the other hand a carelessly
placed walking aid may disturb moving of
other people causing accidents.
10.5. 1. Walking stick
If a stick is used in one hand, weight bear­
ing of the other lower limb can be diminished
by app . 1 0%. Around 2 5 % of the body weight
can be relieved by using 2 sticks. In cases of
paralysis or imbalance a stick increases the
safety of the gait. With the use of special 3 and
4 legged sticks the safety of gait can further be
enhanced.
10.5.2. Crutch
The full non-weight-bearing of one lower
limb can be achieved by using a pair of
crutches.
There are a few forms in use:
78 � 1 0 . O rt h o s e s , a p p l i a n ce s fo r m o b i l ity

Fig. 10.11.
Wal king frame

rarely prescribed in cases of diseases, defor­

mities, weakness of the hand and wrist.

10.5.3. Walking frame

A walking frame has two handles and four
Fig. 10.9. Fig. 10.10. legs (Fig. 1 0. 1 1 .). It is made in two varieties :
Elbow crutch. Armpit crutch. one is transferred step by step, the other rolls
(perhaps with forearm support). It improves
walking safety superior to that with crutches
and also provides non-weigh-bearing.
� armpit crutch,
� elbow (Lofstrand) crutch,
10.5.4. Wheelchair
� forearm supported crutch.
The basic type is the patient-driven wheel­
In cases of intact upper limbs and a good chair, which is presented in fig. 10.12. The pa­
muscle function the elbow crutch is pre­ tient-driven wheelchair is a backed chair with
scribed (Fig 1 0.9.). Armpit crutch may com­ a foldable frame which has 4 wheels, leg rest
press the axillar nerves causing paresis (Fig. and brake. Two of the wheels are large, they
1 0 . 1 0.). However armpit crutches provide hold the handrims .If the hoops are driven
better walking safety for individuals in poor equally in the same direction and at the same
condition. Forearm- supported crutches are speed, forward or backward rolling may be
 1 0 . O rt h o s e s , a p p l i a n ce s fo r m o b i l ity � 79

e --------fl�

a -----tf"IIlIIII_1!I.
g -----z=.=-<J
---"":::tcl----- b
h ----I/

j
k ------'�_lWPdlt+_Ir�:___J

d
C -------j*-llllr

Fig. 10.9.
Patient driven wheelchair. Legends: a) arm support, b) seat, c) custer, d) leg rest, e) push hand le, f) back, g)
driven wheel, h) hand rim, i) brake, j) tipping lever, k) frame.

achieved. Driving the handrim on one side
can make the wheelchair turn .
The other common type of wheelchair is
the electrical wheelchair. Apart from the com­
ponents listed above, it has a battery-powered
engine. Control (starting, stopping and steer­
ing) is achieved by a joystick fitted on the side
more suitable for the capabilities and requests
of the user.
 Ka l m a n T6t h
1 1. Orthopedic shoes and insoles
1 1.1. Shoes and orthopedic shoe
People living in the area now known as
Europe, have been wearing shoes since pre­
historic times. The average distance walked
by a person during a lifetime is around 200
000 km. A good shoe must ensure comfort,
and the fuIl function of the foot. The main pur­
pose of every shoe is to protect the foot from
heat, wet and injuries, thus good design and
suitable material are very important. The pos­
terior part of the shoe is of key importance.
The position of the heel in the shoe is stabi­
lized by a posterior stiffener. The efficacy of
the lining or other corrective supplements is
only possible in the presence of a weIl­
designed stiffener (Fig. 1 1 . 1 .). Another sig­
nificant task of the heel of the shoe is to sup­
port the foot. The more dynamic the load on
the foot, the more important the proper sup­
port. Selection of the correct height of the heel
"
"
"
"
"
b
a c
Fig. 11.1.
a: A shoe without a stiffener or with a loose stiff­
ener: the heel is in the valgus position.
b: In a shoe without a stiffener or with a loose stiff­
ener, the wedge form correction placed on the sole
is ineffective: the heel is still in the valgus position.
c: A proper stiffener design supports the effect of
correction lifting; when placed on the sole, the
valgus of the heel is reduced.
is also fundamental. Other essential factors
are the width and the alignment of the heel and
the type of materials come into contact with
the ground for a stable touchdown and lateral
stability.
The middle part of the shoe is situated ana­
tomicaIly between the sustentaculum tali and
the middle metatarsal region of the foot. The
basic task of this part of the shoe is to fix the
foot steadily. The structure of the front part of
the shoe is adequate if it aIlows the toes to
move freely. This is only possible if the cap of
the shoe is high enough and extends 5- 1 0 mm
beyond the foot. This activity length" is es­
"
sential in the selection of the shoe. Most fash­
ionable shoes (especiaIly for women) do not
meet the physiological requirements, and this
can cause inflammation, caIlosity, parony­
chia, etc.
An orthopedic shoe is custom-made after
individual size measurement, which is carried
out by an orthopedic shoemaker, who utilizes
a copy of the footprint, the silhouette of the
foot, and the circumferences of specific parts
of the foot. The event of a severely deformed
foot, there is a need for plaster of Paris mold­
ing, in a medium loaded condition, before the
plaster has fuIly set.
An orthopedic shoe must satisfy very com­
plex demands. One of the apparently most
simple is to equalize limb shortening. If the
heel is raised by 1 5 mm, the forward-moving
of the center of gravity causes the roIl-over
turning point to move, and for this reason the
distance between the front of the shoe and the
ground also has to be increased.
The greater the disparity to be equalized,
the higher the sole must be raised in order to
protect the knee and hip stability. If a shoe has
a heel elevation of more than 4-5 cm, there is a
82 � 1 0 . O rt h o p e d i c s h o e s a n d i n s o l e s
need for a rocker bottom. I f the elevation i s
even larger than this, the sole is shaped with a
bent last, resembling a rocking chair.
A rolling sole supports the roll-over, while
it permits certain parts of the foot to rest. The
prescription must specify the largest height of
the rolling sole and (depending on the part to
be rested) the distance from the end ofthe heel
(Fig. 1 1 .2.).
It is sometimes desirable to reduce the in­
tensity in the phase of heel strike, in which
case a buffer heel is employed (Fig 1 1 .3.).
Distribution of the pressure on the anterior
part of the foot is important when deforma­
tions are caused by metatarsalgia or rheuma­
toid arthritis, while in cases of diabetes or
other neuropathic foot conditions there is a
�
a often connected to a walking brace or a walk­
�
b
� c
Fig. 1 1.2.
a: A rocker bottom extending to the whole sole and
with a greater heig ht causes the axis of the leg to
lean back; for this reason, the knee joint comes into
extension and hyperextension. This technique there­
fore is used in cases of patella chond ropathy, for ex­
ample.
b: Usage of a rocker bottom placed u nder the mid­
dle of the foot is advantageous in cases of tarsal
and tarsometata rsal osteoarthritis.
c: A rocker bottom placed at the heads of the meta­
tarsal bones is used in cases of metatarsalgia and
hallux rigid us.
Fig. 1 1.3.
a: A buffer heel in an u nloaded position
b: A buffer heel in a loaded position.
need for complete sole surface contact. In­
structions are necessary for the construction,
and positioning of special loading and
load-relieving points (e.g. for patients with se­
vere rheumatoid arthritis, deformation of the
hand means that the shoe should have a
Velcro or clip fastener rather than laces).
Supplementary therapeutic aids can be in­
corporated into orthopedic shoes (e.g. an in­
step raiser, a Bayer spring, etc.). The shoe is
ing apparatus and therefore the appearance
also changes. It must be remembered that the
wearing of such devices is a burden that
causes lifelong problems for the patient.
1 1.2. Shoes for children
At the posterior part of children' s shoes
there should be a strong stiffener to keep the
heel in a vertical position. Wearing sandals
without a stiffener is inappropriate for young
children. The heel part should incline from in­
side to outside, while the stiffener inside
should extended forward to keep the heel in a
stable position. If the doctor considers that an
otherwise well-prepared shoe with a stiffener
is not sufficient to correct a valgus flat foot, it
is necessary to use a shoe with a supinating
heel that compensates the outward-leaning
(valgus) of the heel. The tasks of the middle
part of the shoe are not to let the muscles of
the sole stretch and to hold the middle part of
the child ' s foot. The anterior part of a child' s
shoe is significant a s concerns the position

and movement of the toes. The inside line of
the big toe is straight until the age of 5 , after
which it deviates from a straight line by only
8- 1 00• The shoe should be at least 5 mm lon­
ger than the foot, so that the toes can be moved
freely. The usage of a built-in insole (except
for some special indications) is improper
since the pad weakens the foot muscles of the
child even more. Naturally, orthopedic shoes
for children are also custom-made after indi­
vidual foot size measurement, with accesso­
ries and special upper and lower part con­
struction ordered by the doctor on the pre­
scription (e.g. with a straight sole axis, and a
pronating heel in cases of clubfoot) .
1 1.3. Shoe and sole insoles
The insole (archer, arch support or insert)
is an orthopedic aid that must be indicated, or­
dered and verified by the doctor while it is be­
ing worn. The suitable insole forms a func­
tional unity with the shoe, and therefore the
form and the material of the insert should be
adapted both to the foot and to the shoe.
1 1 . O rt h o p e d i c s h o e s a n d i n s o l e s � 83
The task of the insole is t o correct and re­
tain the arched structure of the foot in every
ray. Optimization of the distribution of the
weight between the sole and the ground is es­
sential, since the insert transmits the body
weight between the sole and the ground. The
weight distribution is achieved via a pad un­
der the transversal and longitudinal arches.
Adj ustment of the posterior foot j oints and
keeping them in the right position is the task
of the longitudinal arch pad. The pad should
restore the balance of the muscles affecting
the foot. It is important that the transversal
arch is not corrected by the transversal pad:
this takes over part of the loading from the
metarsal heads, thereby relieving the weight
from the metatarsal heads, and dispersing the
weight on the longitudinal arch. The material
of the insole must be easily shaped and
molded, and it should be elastic. A well­
designed size series is essential. Even with the
proper indication, a foot insole prepared with
the above expectations, will be effective for
only a certain period. It is duty of the doctor to
inform the patient about the effects of the in­
sole, and that it will be necessary to change to
a new pad at the appropriate time.
 Ka l m a n T6t h

1 2. Normal growth, growth disturbances

This category of diseases includes g rowth dis­
turbances of a general or a local nature that
occur in childhood and involve a more than
average difference in height or a the length of
the limbs and / or axial deviation. The clinical
pictures of these conditions are somewhat
similar, despite the fact that they may be in­
duced by various endogenous or exogenous
causes or even unknown.
12. 1. Physiological growth
The process of skeletal development be­
gins during the embryonic phase and ends at
puberty with the closure of the growth plate
cartilages. At the end of the second embryonic
month, the cartilaginous skeleton is already
segmented as concerns the main parts, and
thus the preliminary skeletal limbs are already
visible. The primary ossifying center of the
long tubular bones appears in the week 9, al­
though all the primary cores of the tubular
bones develop during the intrauterine period.
The primary ossifying center is in the middle
of the embryonic cartilaginous matrix, (which
will later become the diaphysis), while the
secondary ossifying centers are at the two
endings of the bone (later the epiphysis). The
secondary ossifying centers typically appear
after birth in the long tubular bones (Fig.
1 2 . 1 .),with the exception of the distal femur
and the proximal epiphysis of the tibia.
The growth of the bone itself is due to an
enchondral length gain periosteal bone thick­
ening. The enchondral lengthening (Fig.
12.2.) occurs in the cartilaginous epiphysis
fugue (growth plate) which remains cartilagi­
nous, and therefore remains radiologically
transparent and clearly observable until the
termination of growth. Growth and cell mito­
sis occur in the proliferation zone of the grow­
ing cartilage. Due to the continuous cell mito­
sis the distance between the center of the
epiphysis and the center of the diaphysis
steadily increases, thereby leading to an in­
crease in length. From a biological aspect, it is
crucial that the epiphysis and the metaphysis
have an independent and separate blood sup­
ply till the closure of the epiphysis fugue.
Periosteal growth is a result of the apposi­
tional activity of the osteoblasts originating
from the periosteum, as a consequence of
which the width of the tubular bones in­
creases. Endosteally, within the tubular
bones, e.g. from the medullary cavity, decom-

a -=---#,
c c b
b
d
e -=--.......

e
Fig. 12.1.

age of 16 and full ossification takes place by the age of 23-25. b: A ring-shaped secondary ossification nucleus
a, c, d, e: Secondary ossification of the vertebra. The secondary ossification nucleus appears at around the

appears approximately at the age of 7 and fu lly ossifies by the age of 23-25.
86 � 1 2 . N o r m a l g rowth, g ro w t h d i st u r ba n ces
I
4 Femur
2 Fibula
Fig. 12.2.
Enchondral ossification
1: epiphysis 4: cartilage proliferation zone
2: growth plate 5: cell degeneration zone
3: metaphysis 6: ossification zone
position of the bone substance also occurs re­
sulting in a gradual increase in the diameter of
the medullary cavity.
Both enchondral and periosteal growth oc­
curs in accordance with the biomechanical
principle of the developing cartilage being
perpendicular to the forces acting on it. Any
intrinsic and extrinsic factor disrupting this
principle can lead to a growth disturbance
(e.g. closure of the epiphysis fugue due to
trauma) .
The tactics for the treatment of growth dis­
turbances necessitates the consider of two as­
pects. First the proportions of the growth of
the epiphysis located at the distal and proxi­
mal ends are well established for all long tu­
bular bones (Table 1 2 . 1 .) .
The increase in length is not linear with
aging; in certain periods the growth acceler­
ates. When a particular treatment strategy is
Table 12.1.
Ratio of bone growth
Humerus Proximally 80%, distally 20%
Radius Proximally 25%, distally 75%
Ulna Proximally 20%, distally 80%
Proximally 30%, distally 70%
Tibia Proximally 57%, distally 43%
Proximally 60%, distally 40%
under consideration, therefore the changes in
the pace of growth have to be taken into ac­
count. Then again, there are periods during
normal development (e.g. puberty) when the
ossification ability has difficulty in keeping
up with the pace of growth within the pro­
liferative zone of growth plates. Later on,
when the pace of growth of the proliferative
zone decreases, the ossification can catch up.
On the other hand, if any mechanical injury
should impair this certain area, it may lead to
various diseases (e.g. Scheuermann ' s dis­
ease) . The development of the joint surface
regions located at the ends of the long tubular
bones is governed by the development within
the epiphysis itself.
12.2. Growth disturbances
Growth disturbances basically fall into
one or other of two categories. First, there are
generalized growth disturbances, that affect
all developing bones leading to excessive or
inhibited growth, producing a too tall or a too
short body height. The cause in such cases is
usually metabolic and/or endocrine distur­
bance.
Secondly, there are local growth distur­
bances, where the defect involves a well de­
fined area (may be a bone) . This could be
hypoplasia or hyperplasia, affecting the dis­
eased bone uniformly, while an uneven
growth disturbance involving the inside of the
bone may be due to the involvement of the
 1 2 . N o r m a l g rowth, g r o wt h d i s t u r ba n ces
epiphysis, the meta-diaphysis or the growth
plate. The resulting appearance differs ac­
cording to the location of the deficiency
within the bone . For example, any harmful ef­
fect occurring in the epiphysis will lead to de­
formation of the j oint' s surface (e.g.
osteochondrosis dissecans, Perth es -disease) .
Growth disturbances occurring in the
metaphysis and / or diaphysis generally upset
the growth in periosteal width and may also
lead to a disparity the in axis. However, most
local growth disturbances involve damage
caused in the germinate layer of the epiphysis
fugue.
Some local growth disturbances may be
highlighted from an etiological aspect:
1 . Vascularization disturbances: This group
includes aseptic osteochondroses and
osteochondro-necroses which can be
linked to blood supply disorders of un­
known origin. A discussion of these for
diseases according to age and localization
is presented in the appropriate chapters .
2. Infections: Due to hematogenous spread
during childhood, osteomyelitis is mainly
localized in the metaphysis, close to the
epiphysis fugue, which disrupts the devel­
opment of the epiphysis. In childhood
osteomyelitis commonly affects the femur
epimetaphyseal region. For example, if
this is situated medially, then (due to the
growth disturbance here), the unaffected
lateral epiphyseal segment will relatively
overgrow, creating a varus deformity. The
younger the affected patient, the larger the
axial deviation may be, and a limb length
discrepancy may occur as well.
3 . Tumors: These rarely cause growth distur­
bances, but this chance should be consid­
ered nonetheless.
4. Trauma: Growth disturbances caused by
childhood inj uries can be divided into two
groups : direct and indirect. The growth
disturbance is indirect if the inj ury does
not affect the growth plate, but impairs the
blood supply of the growth zone, thus pro­
ducing a growth disturbance. A growth
disturbance is direct, when an injury af-
� 87
fects the cell-division zone of the growth
cartilage resulting in a bridging callus de­
velopment. A bridging callus obstructs the
growth of a particular zone in the growth
region and, depending on its location and
the relatively greater growth of the other
growth zones, leads to axial deformities
(e.g. if it is medial and anterior, it leads to
recurvation and varus; if it is in a lateral or
central position it leads to valgus). If the
damage causes closure of the whole
growth plate, it will lead to a shortening of
the limb.
5 . Growth disturbances caused by iatrogenic
procedures:
a) Post surgery: Growing cartilages may
be damaged when bone injuries suf­
fered during childhood are treated. If
the fracture does not affect the epiphy­
sis fugue, great care must be taken to
use such fixation technique which
avoids transepiphyseal fixation.
b) Damage caused by x-ray: x-ray irradia­
tion used in the treatment of malignant
tumors in childhood may cause growth
disturbances. For example x-ray irradi­
ation used to treat the Wilms tumor
may lead to radiogenic lumbar sco­
liosis.
12.2. 1. Treatment options for
growth disturba nces
Conservative treatment is preferred if the
degree of shortening of the lower limb is less
than 3 centimeters and there is no axial devia­
tion. In such cases orthopedic appliances (an
increase of in thickness of the sole of the shoe)
are used to compensate the growth distur­
bance. As upper limbs are concerned - as they
are not weight-bearing - an upper limb short­
ening causes less functional problems. If the
difference in length of the lower limbs is too
considerable and even a series of surgical in­
terventions cannot compensate the difference
(primarily limb development deficiencies),
the use of orthoses is recommended in order to
ensure the ability to walk.
88 � 1 2 . N o r m a l g rowth, g ro w t h d i s t u r b a n ce s
Operative treatment is required if the
difference in length of the lower limbs ex­
ceeds 3 centimeters and / or an axis deviation
persists. The surgical strategy should always
be planned individually. Operative treatment
may be applied either before or after the final
closure of the growth cartilage. In case of seri­
ous growth disturbances step-by-step correc­
tive treatment is required.
From an operative technical aspect, the
possibilities include lengthening of the
shorter limb and/or shortening the longer
limb . Today, instead of maj or limb lengthen­
ing, gradual elongation techniques are consid­
ered more appropriate since the margins of
elongation of muscles, nerves and veins are
much more constrained when the former tech­
nique is used. Gradual diaphyseal or meta­
physeal elongation may be performed, de­
pending on whether the osteotomy was in the
diaphysis or the metaphysis area.
Altering the length of limbs requires ex­
treme caution. When limb shortening is per­
formed, the origin and attachment of the mus­
cles draw closer to each other which in turn
leads to a certain amount of muscle weaken­
ing, determined by the degree of shortening.
Limb lengthening poses a different prob­
lem: the degree of tension of the soft tissues of
the body (muscles, nerves, veins) . The
contracture of joints above and below the
elongation is avoided by continuous physio­
therapy, nevertheless, in some cases the
lengthening apparatus has to be augmented
with passive supplementary elements. Fur­
ther, the pace at which the bone should be
elongated is a difficult question. If lengthen­
ing progresses too rapidly, calcification can­
not proceed at the optimal pace, but if the pace
of lengthening is slower than the pace of bone
development, then the lengthening will cease
before the desired length is achieved.
12.3. Limb deformities
Limb shortenings are often encountered
together with axis deformities. The deformity
of the limb in question can be described by its
localization, the degree of deformity and the
difference in length between the limbs.
In the frontal plane there are valgus and
varus deformities, in the sagittal plane
ante-curvation (the distal segment of the limb
curves back) and retro-curvation (see Chapter
4.2.3 .), while deformities affecting the axis
are called outer and inner rotation.
Limb deformities may occur for various rea­
sons:
� I njury to growth plates (inflammation,
tu mor)
� Post-trau matic condition
� Bone dysplasia (Paget's disease, fibrous
dysplasia)
� Bone metabolism disorder (rickets,
osteomalacia)
� Weakness of connective tissue, loose
ligaments
� Muscle paralysis (Heine-Medin disease)
� Compensatory
It is important that a certain axis deviation
of the limbs frequently occurs in childhood,
but remains within the physiological level : in
the lower limbs this involves varus and valgus
deformation and torsion of the tibia, the tibia
usually curving inward. The child compen­
sates for such a tibia torsion by valgus posi­
tioning of the tarsus and abduction ofthe fore­
foot. These deformities are usually rectified
spontaneously during growth, and by the age
of 7 to 1 0 such axial deformities have disap­
peared. If not, correctional osteotomy is ad­
vised, since a prolonged axial deformity will
lead to uneven pressure on the joint and later
on to osteoarthritis (see Chapter 29).
Due to the many possible variations, com­
bined treatment is also an option, when the
length difference and the axial deformity are
corrected at the same time, either in one stage
or in several stages.
 Tibor Viz ke lety
13. Bone dysplasias and developmental
anomalies
� Bone dysplasias
Disturbances in skeletal development and
ossification demonstrate high variability in
the clinical picture depending on the localiza­
tion and age.
A good understanding of the bone
dysplasias is provided by the dynamic classi­
fication of Rubin. Fig. 1 3 . 1 . presents the dis­
eases caused by hypoplasia or hyperplasia
during skeletal development.
The earlier used classification and nomen­
clature of Paris was later further developed by
an international group of experts in 1 997. The
diseases are divided into three groups from a
practical and theoretical point of view:
dysostoses, i.e. developmental disturbances of
one or more bones, disruptions, i.e. secondary
developmental disturbances of the bones, and
skeletal dysplasia, i.e. diseases of the
chondro-osseal tissue. The osteochondro­
dysplasias are a group of diseases caused by
abnormal growth and abnormal development
of the bone and/or cartilage (Table 1 3 . 1 .) .
W e deal here only with the well-known
and frequently occurring forms of bone
dysplasias and developmental anomalies.
Table 13. 1.
Classification of osteochond rodysplasias
I. Lethal osteochond rodysplasias
11. Chond rodysplasia pu nctata group
Ill. Skeletal dysplasias with predominant
metaphyseal involvement
- Achondroplasia
IV. Skeletal dysplasias with predominant
epiphyseal involvement
- Multiple epiphyseal dysplasia
V. Skeletal dysplasias with major involvement
of the spine
- Spondyloepiphyseal dysplasia, congenital
form
- Spondyloepiphysea l dysplasia, tarda form,
X- linked
- Diastrophic dwarfism
VI. Skeletal dysplasias with multiple dislocations
VII. Dysostosis multiplex, complex carbohyd rate
storage diseases
- Mucopolysaccharidoses I-VI I
- Mucopolysaccharidosis I-H (Pfaundler-Hurler
disease)
- Homocystinu ria
VIII. Skeletal dysplasias with predominant
involvement of single sites or segments
IX. Dysplasias with prominent diaphyseal
involvement
X. Skeletal dysplasias with decreased bone
density
- Osteogenesis imperfecta
XI. Skeletal dysplasias with increased bone density
- Osteopoikilosis
- Paget's disease
- Osteopetrosis (Albers-Schonberg disease)
XII. Skeletal dysplasias due to disorganized
development of bone constituents
- Dysplasia epiphysealis hemimelica
- Multiple cartilaginous exostosis (exostosis
cartilaginea multiplex)
- Enchondromatosis (dyschondroplasia, Oilier
disease)
- Fibrous dysplasia
- Hyperchondroplasia (Marfan syndrome)
- Ehlers-Danlos syndrome
- Neurofibromatosis
XIII. Osteolyses
90 � 1 3 . B o n e d y s p l a s i a s a n d d eve l o p m e n t a l a n o m a l i es

1. Epiphyseal hemimelic dysplasia

1 11
2. Epiphysis
3. Hyperchondroplasia
2
4. Endchondromatosis
12

3
5. Physis
6. Metaphysis
4
7. Multiple cartilagineous exostoses
13
8. Progressive diaphyseal dysplasia
5
14
9. Diaphysis
10. Hyperphosphatasaemy
1 1. Spondyloepiphyseal dysplasia
15
6
12. Multiple epiphyseal dysplasia
7 16 13. Chondrodystrophy
14. Metaphyseal dysplasia
17
15. Hypophosphatasy
8 16. Osteoporosis
17. Craniometaphyseal dysplasia
18
18. Osteogenesis imperfecta
9
19. Osteopetrosis
10 19

Fig. 13.1.
Dynamic classification of bone dysplasias according to Rubin

13. 1. Frequently occurring Major clinical findings: Disproportionate

osteochondrod}'.splasias dwarfism with short limbs, wide and short
hands, and varus curved lower extremities.
The head is disproportionately large, with a
depressed nasal bridge (Figures 13.2. a, b).
13. 1 . 1 . Skeletal dysplasias with
predominant metaphyseal The endocrine and sexual functions and intel­
involvement ligence level are normal.

� Achondroplasia (chondrodystrophia The long tubular bones are short (often

fetalis, chondrodysplasia fetalis,
chondrodystrophic dwarfism)
Etiopathology: Achondroplasia is a de-
velopmental disturbance ofthe growth plate, a
development is caused by a G 1 1 3 8A mutation
of the FGFR3 gene on the chromosome 4
(4p 1 6. 3 ) . It is responsible for coding the
fibroblast growth factor receptor. The muta­
tions develop only on the paternal chromo­
somes. The incidence rate of the mutation in­
creases with increasing age of the father. The
altered receptor function results in inappropri­
ate differentiation of the growth plate (physis)
cartilage cells and ultimately deficient en­
chondral growth.
less than one-third of the normal length). On
radiographic pictures, the ends of the bones
are massive, and the acetabulum and the spine
show very special radiographic features (Fig.
13.3. a, b).
Incidence: This condition is recognizable
at birth. The incidence rate is one patient in
3 0 . 000 live births.
In the tarda form the clinical symptoms are
not as markedly expressed. These dwarfs are
usually intelligent with great sense of humor.
Once they were jesters of the kings.
Though examples present these patients
may accept their dwarfism, creating closed
communities, but there are nowadays increas-
 1 3 . B o n e d y s p l a s i a s a n d d e v e l o p m e n t a l a n o m a l i es � 91
a b

Fig. 13.2.
Achondroplasia
a} An eight-year-old normal boy and an eight-yea r-old achondroplasia patient.
b} The typical trunk and extremity length relations in achondroplasia dwarf patient (rhizomelia).

a b

Fig. 13.3.
The long tubular bones are short and thick, while the metaphysis is broadened in an achondroplasia dwarf:
Upper extremity bones (a) and lower extremity bones (b)
92 � 1 3 . B o n e d ys p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i es

ingly frequent requests for orthopedic length­
ening surgery to enable them to attain nearly
normal height. In special cases modem limb
lengthening techniques provide a possibility
to reach the lower border of normal body
height through bilateral elongation of the
thighs and lower legs (a 1 0 cm lengthening
each). These long surgical procedures we can
definitely help these patients to play a more
active in the society and in modem technical
environment.
13.1.2. Skeletal dysplasias with
predominant epiphyseal
involvement
� Multiple epiphyseal dysplasia (MED)
(Fairbank disease, dysostosis
epiphysealis multiplex, dysplasia
epiphysealis multiplex)
In this disease abnormal epiphyseal ossifi­
cation occurs in the ossific nuclei. Point-like
ossifications are observable on radiographic
pictures of the cartilaginous epiphyses. This is
dwarfism with shortening mainly of the upper
parts of upper and lower extremities. In the
tarda form the abnormality of the epiphyses
frequently lead to precocious and often pro­
gressive early degenerative osteoarthritis,
with a short stature and short fingers . It is hard
to influence the severe disturbance of joint
function.
13. 1.3. Skeletal dysplasias with ma­
jor involvement of the spine
� Spondyloepiphyseal dysplasia
congenita (SED) (Spranger- Wiedemann
disease, osteochondrodystrophia
deformans, Strudwick disease)
This is a hypoplasia of the epiphysis.
Major clinical findings: The extremities and
the trunk a re short and dwarfism is observ­
a ble at birth. Delayed ossification and defor­
m ities are visible in the radiographic pictu res.
Incidence : This is a rare developmental
disease which is observable at birth .
The development of the vertebral bodies is
disturbed with flattening, immaturity and an­
terior ossification defects of the vertebral bod-

a b

Fig. 13.4.
Spondyloepiphyseal dysplasia congenita. Dorsolumbar kyphosis with ventral narrowing of the vertebral bodies
(a) and disturbance of the ossification in the pelvic bones and femurs (b).
 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i e s
ies combined with a dorsally wedged defor­
mity (Fig. 13.4. a) and lumbodorsal kyphosis.
Retarded ossification of the epiphysis of the
tubular bones with irregularity is common
(Fig. 13.4. b) with protrusion of the aceta­
bulum. A disproportionately short stature is
typical, with brachydactyly. Later muscle
weakness and progressive myopia may de­
velop with slightly protruding eyes will be
presented.
The deformities and short stature can be
corrected to a certain extent.
� Spondyloepiphyseal dysplasia tarda.
Major clinical findings : A short stature
due to a short spine with relatively long ex­
tremities. The radiographic pictures is typical :
general ized flattening of the vertebral bodies,
named platyspondyly. This is an X-linked re­
cessive disease that occurs in males. The
symptoms are milder than those in the con­
genital form. In this disease, premature osteo­
arthrosis is common, most notably in the hips
and shoulders.
13. 1.4. Dysostosis multiplex,
complex carbohydrate storage
diseases
� Mucopolysaccharidoses
The group of mucopolysaccharidoses,
oligosaccharidoses and glycoproteinoses in­
cludes all of those diseases in which generally
disturbed ossification causing a short stature
or dwarfism with an increased level of
mucopolysaccharides in the urine. According
to the form of genetic transmission and ap­
pearance of corneal opacity and metachro­
matic granules in lymphocytes many types of
mucopolysaccharidoses can be differentiated.
(Pfaundler-Hurler disease, Hunter disease,
Sanfilippo disease, Scheie disease, Maro­
teaux-Lamy disease, Morquio- Ulrich disease
etc.). The best known form is the gargoylism.
� Gargoylism (Pfaundler-Hurler disease)
The genetic transmission is autosomal re­
cessive. The maj or clinical findings are the
short stature, a di stinctive facial appearance,
� 93
corneal opacity, mental retardation, hepato­
megaly and splenomegaly. The distinctive fa­
cial appearance is due to the wide head, the
prominent frontal bone, the massive mandible
and the widely spaced prominent eyes gives.
The spinal column and the hands are short,
and thoracolumbar kyphosis occurs . There is
a significant delay in the development of the
movements with mental retardation. The cor­
neal opacity is observed between the ages of
one and three years. The radiographic pictures
show a characteristic deformity of the verte­
bral bodies, as an ovoid-biconvex (immature)
shape with anterior narrowing caused by
antero-superior ossification defects . The ra­
diographic symptoms are usually more severe
in the upper extremities than in the lower
ones. The intramedullary space of tubular
bones is widened, the epiphyses are flattened
and multicentric. The radius and the ulna are
short.
There is an increased urinary excretion of
dermatan sui fate and heparin monosulfate.
The aim of orthopedic surgical interventions
is to correct the extremity deformities and fa­
cilitate nursing.
13. 1.5. Skeletal dysplasias with
decreased bone density
� Osteogenesis imperfecta
There is an increased generalized bone fragil­
ity.
Major clin ical symptoms: Bowing deformi­
ties of the tubular bones with blue sclera in
some cases (Fig. 13.5.).
Incidence: This varies with the type of the
disease. It can exist at birth, but can develop
later too .
The bones are narrow and fragile, with
poorly trabeculated spongiosa, and the cortex
of the tubular bones is thin because of the
quantitative and structural deficits of type I
collagen synthesis leading to a lack of peri­
osteal new bone formation. There are signs of
previously healed bone fractures and sub se-
94 � 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i es

microfractures (Fig. 13.6.). In addition the

skin is thin and translucent, and the sclera is
blue in some types. The development of the
teeth may be abnormal (dentinogenesis
imperfecta), point-like skin hemorrhage can
occur secondary to an increased capillary fra­
gility, and there may be mild ligamentous
hyperlaxity. Conductive or mixed hearing
loss or deafness can develop .
Four classes of osteogenesis imperfecta
may be distinguished, according to time ofthe
manifestation of the disease, the symptoms
Fig. 13.5.
associated with fragility, and the type of ge­
Typical blue sclera in a child with osteogenesis
netic transmission.
imperfecta.

Type 1 : Osteogenesis imperfecta tarda.

quent deformities. The vertebral bodies are
flattened, often with a biconcave shape. In the
tarda form there is a decreased bone density
with thinner cortex and signs of fractures and
Type 2: Lethal neonatal osteogenesis im­
Type 3: Progressive deforming osteogene­
Type 4: Osteogenesis imperfecta tarda. The
Fig. 13.6.
Curved, deformed bones with thin cortex i n the
lower extremities in an osteogenesis imperfecta pa­
tient.
Osteopsathyrosis. There is usually no
sign of abnormal bone fragility at birth.
Fractures occur later but are not too fre­
quent. The incidence of scoliosis is 20 per­
cent. The sclera is blue and ligamentous
hyperlaxity is observed. Deafness devel­
ops in 40 percent of the patient. The ge­
netic transmission is autosomal dominant.
perfecta. Many bone features can occur
already during intrauterine life (some­
times more than one hundred and healed
fractures can be detected). Fractures are
very frequent after birth too. Because of
the healing in malposition, the long tubu­
lar bones are curved, deformed and wid­
ened. The babies usually die early. The ge­
netic transmission is autosomal recessive.
sis imperfecta. This is also congenital.
Fractures already occur during the first
few weeks of extrauterine life or during
the first year. The long tubular bones and
spinal column develop deformities com­
bined with scoliosis. The sclera of the in­
fant is blue, but later the color of the sclera
normalizes. This disease with its serious
outcome has autosomal recessive genetic
transmission.
fragility of the bones ranges from moder­
ate to severe. The color of the sclera is blue
during the neonatal period, but later be-
 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i e s
comes normal. The long tubular bones and
spinal column are deformed. This type can
occur with or without dentinogenesis
imperfecta. There is an X chromo­
some-linked version with autosomal dom­
inant genetic transmission.
Therapy: Good results have recently been
published following cyclic bisphosphonate
treatment which provides stronger bones and
decreased bone fragility. Fractures heal well
by the standard treatment with the aims of
good alignment and the prevention of defor­
mities. The deformities can surgically be cor­
rected by osteotomies, fixed by different types
of intramedullary nails or wide Kirschner
wires. Plaster fixation and skeletal or non­
skeletal traction are applicable to treat frac-
Fig. 13.7.
Correction of the curved femur in osteogenesis
imperfecta, with rotation of the segments and
intramedullary fixation.
� 95
tures in osteogenesis imperfecta. Seriously
curved long tubular bones need multilevel
osteotomies (salami osteotomy) with the cor­
rection of rotation deformity in different seg­
ments fixed by intramedullary nails (Fig.
13.7.).
13. 1.6. Skeletal dysplasias with
increased bone density
� Paget's disease (osteitis deformans)
The aetiology of this disease is not known. It
involves chronic disturbance of the bone
transformation with abnormally increased
osteoblast and osteoclast activity.
Etiopathology : Viral infection, a genetic
predisposition, an endocrine malfunction or
disturbance of the phosphorus metabolism are
supposed as etiologic factors. The essence of
the disease is osseous tissue transformation
with simultaneously increased bone forma­
tion and bone resorption. The intramedullary
space is usually filled up with fibrous tissue. It
may occur in only one bone, rarely turns into a
generalized form. It develops more frequently
in males in the fourth or fifth decade of life .
Major clinical findings : At the beginning
the complaints are usually mild with pain in
the extremities with or without weight­
bearing. The patient often observes the pro­
gressive curving deformity of the femur or
tibia. The volume of the skull can increase and
the hat may be noted to tightly fitting, if the
disease is localized in the head too. In these
cases, neurological symptoms can develop,
with a mixed hearing and vision loss.
Major radiologic features : The struc­
tural changes of the involved bone are very
typical. The normal bone structure is lost and
interspersed trabecular areas of increased or
decreased density substitute the normal
trabecular osseous structure of spongiosa
(Fig. 1 3.8.), followed by dissolution of the
cortical architecture and replacement by
strands of longitudinally oriented trabeculae.
The bones become thicker and curved.
96 � 1 3 . B o n e d y s p l a s i a s a n d d eve l o p m e nta l a n o m a l i e s
a b

Fig. 13.8.
Paget's disease: loss of normal bone structure and interspersed areas of increased and decreased density of
the trabecu lae in the right pelvic half (a), and curved scabbard- like tibia (b).

Therapy: The unknown cause of this dis­

ease can clearly not be treated. Clinical, radio­
graphic and laboratory improvements can be
achieved with intravenous bisphosphonate
therapy. Osteosynthesis is performed in cases
ofpathologic fractures of the seriously curved
bones. Severe osteoarthrosis of the large
j oints demands hip or knee arthroplasty.

Fibrosarcoma, osteosarcoma or malig­

nant fibrous histiocytoma develop in the
bones in 5-20% of patients with Paget' s dis­
ease. In cases of malignant transformation,
the clinical and radiographic symptoms show
rapid progression. After the definitive diagno­
sis, amputation or exarticulation is necessary.

� Osteopetrosis (Albers-Schonberg
disease, marble disease, j uvenile
osteopetrosis)
The term osteopetrosis is applied to a group
of disorders caused by defective bone resorp­
tion due to a n impaired osteoclast function.
The entire skeleton is sclerotic, and the corti­
cal bone and the spongiosa do not separate
(Fig. 13.9.).
Fig. 13.9.
Typical radiographic picture of osteopetrosis: the
pelvis shows cortical-like radiodense bone every­
where, the intramedullary spongiosa does not sepa­
rate from the cortical bone.
The vertebral bodies show a frame-like ap­
pearance in the radiographs, the bones are
brittle, and there is myelosclerosis. Defects of
 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i e s � 97

vision or hearing, facial paresis, hydrocepha­

lus can develop leading to early death. In the
tarda form sclerotic changes are observed
only in some regions of the skeleton, with
mild clinical process.

13. 1.7. Skeletal dysplasias due to

the disorganized development of
bone constituens
� Dysplasia epiphysealis hemimelica
( Trevor's disease, tarsomegaly, tarsal
epiphyseal aclasis)
This is a consequence of epiphyseal over­
growth. Independent ossific nuclei exist adj a­
cent to the original epiphyses in the medial or
lateral part of the extremity, because of the ab­
normally increased growth of articular carti­
lage. They are separated from the original
epiphysis by a narrow gap, which persists dur­
ing bony maturation. This can be regarded as Fig. 13.10.
an epiphyseal exostosis. Resection IS sug­ Typical radiographic picture of osteochondromas on
the distal metaphysis of the femur and on the proxi­
gested in case of complaints.
mal metaphysis of the tibia in multiple cartilaginous
exostosis.
� Multiple cartilaginous exostosis
(exostosis cartilaginea multiplex,
multiplex osteochondromatosis,
� Enchondromatosis (dyschondroplasia,
hereditary multiple exostosis)
Exostoses occur on the metaphysis of tu­ Oilier's disease)
bular bones covered by cartilage. They are This is caused by an abnormally increased
usually detected after two years of age. The cell division rate in the hypertrophic zone of
cartilage covering the exostosis is similar to the growth plate. The metaphyses of the long
the cartilage of the growth plate, and the plane tubular bones are widened and islands of car­
of this cartilage is perpendicular to the growth tilage are observed inside situated parallel to
plate. It can occur rarely in cubic and flat the longitudinal alignment of the bone (Fig.
bones too. The bones can be deformed short­ 1 3 . 1 1 .) .
ened and luxation of the radial head and a Limb length discrepancy and deformity
short stature may be observed. The tendons may develop, which can be treated by surgical
and muscles sometimes jump over the intervention. Oilier disease is enchondro­
exostosis next to the joints during movement, matosis which occurs only unilaterally. In the
causing complains. An exostosis can fracture MafJucci syndrome the enchondromatosis is
resulting in inflammation in the surrounding associated with subcutaneous hemangiomata.
bursa. Surgical resection is required for large,
� Fibrous dysplasia (JafJe-Lichtenstein
disturbing and painful exostoses. It is also
suggested in cases of an exostosis that under­ disease)
goes further growth after bony maturation, as
the possibility of malignant transformation is This is a developmental disturbance of the
3-6 percent (Fig. 13.1 0.). bones. The osseous tissue is replaced by fi
98 � 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i es
Fig. 13.11.
Dyschondroplasia (enchondromatosis): Large carti­
lage islands in the proximal and distal metaphyses in
the bones of the lower leg
brous tissue and premature bone trabeculae.
Monoostotic and polyostotic forms are
known.
Etiopathology : The cause of fibrous
dysplasia is a somatic gene mutation that re­
sults in the enhanced activity of a cell mem­
brane-linked protein (G-protein) and in­
creases the intracellular concentrations of a
few proteins responsible for gene regulation.
It leads to abnormal differentiation of the os­
teoblasts and the development of fibrous tis­
sue which can not be mineralized. Moreover
the activity of the osteoclasts is increased re­
sulting in intensive bone re sorption.
Major radiographic features : The long
tubular bones show eccentric but not cortical
lesions, with endosteal scalloping, occasion­
ally surrounded by a sclerotic margin. The
lytic lesions are often multiple with milk
glass-like radiographic appearance (Fig.
1 3 . 1 2.).
Maj or clinical findings : In severe in­
volvement the softened bone often curves be­
cause of the weight bearing. The "shepherd' s
crook" femur deformity i s typical i n fibrous
Fig. 13.12.
Fibrous dysplasia: "shepherd crook" deformity of the
femur with milk glass-like lytic defects.
dysplasia. In the McCune-A lbright syndrome,
the fibrous dysplasia is combined with unilat­
eral cafe-au-Iait spots with irregular margins
and other disturbances of the endocrine func­
tions. Very rarely, intramuscular myxomas
are coupled with fibrous dysplasia (Maza­
braud syndrome) .
Therapy: Earlier the only method for the
treatment of fibrous dysplasia with a severe
involvement was surgical excochleation of
the lesion which was than filled with bone
chips. Spontaneous pathologic fractures can
occur in case of seriously curved deformities,
and stabilization with osteosynthesis is very
difficult because the screws can not be fixed
in the soft bone. Surprisingly good results
have recently been published after long
bisphosphonates treatment.
� Hyperchondroplasia (Marfan
syndrome, arachnodactyly)
The Marfan syndrome is a well-known
autosomal dominant disorder with variable
 1 3 . B o n e d y s p l a s i a s a n d d e ve l o p m e n t a l a n o m a l i e s
expresslVlty. Its cause i s a mutation o f the
fibrillin gene which results in a disturbance of
elastic fib er production. The maj or clinical
findings are concentrated in three regions of
the body: the eyes, the cardiovascular system,
and the skeletal system.
Major clinical findings : Bilateral lens
displacement (ectopia lentis) is typical be­
cause of the insufficient function of the sus­
pensory ligaments. Myopia is frequent and
blue sclera, ptosis and cataract sometimes oc­
cur. The most serious cardiovascular manifes­
tations are aortic aneurysm, aortic dissection
and insufficiency of the mitral and tricuspidal
valves because of the fragmentation of elastic
fibers in the aorta and valves. The most com­
mon alterations involve the skeletal system.
There is abnormal activity in the proliferative
layer of the growth plate. Children with Mar­
fan syndrome are tall with excessive limb and
digital length, resembling spider legs (arach­
nodactyly), combined with symptoms of gen­
eralized joint laxity. Hypermobility of the ar­
ticulations, dislocations, flatfoot and a gothic
high-arched palate are frequent clinical find­
ings. The pelvis is wider, the overgrowth of
the ribs results in a chest deformity (pectus
excavatum and carinatum) . Progressive sco­
liosis frequently occurs (Chapter 24.5 .2.2.).
The major clinical symptoms are very sim­
ilar in homocystinuria.
• Ehlers-Danlos syndrome
The Ehler-Danlos syndrome is a group of heri­
table connective tissue disorders that share
the common features of skin hyper­
extensibility, articular hyper mobility and tis­
sue fragility. There is genetic heterogeneity
mainly with autosomal dominant inheritance.
Major clinical findings : The velvety, ex­
tendable, white and vulnerable skin is typical,
with calcification after injections. Hyper­
extensibility of the articulations (rubber man),
luxations, foot deformities, kyphoscoliosis,
blue sclera and other developmental anoma­
lies of the eyes can occur in this syndrome. It
is supposed that the causes of the disease are a
� 99
decrease of elastic fibers of the corium and the
development of abnormal collagen fibers.
Therapy: The standard orthopaedic con­
servative and surgical methods are applied to
treat foot deformities, recurvation of the knee
and the frequent congenital hip dislocation.
• Neurofibromatosis
(von Recklinghausen disease)
Neurofibromatosis is a frequent disease
with autosomal dominant inheritance. Its inci­
dence is 1 : 3 000. It occurs with identical inci­
dence and severity of clinical appearance in
females and males. Type I neurofibromatosis
(known as von Recklinghausen disease)
shows mainly peripheral involvement: subcu­
taneous nodules (neurofibromas), pigmented
skin lesions (cafe-au-lait spots), pigmented
iris hamartomas (Lisch nodules), scoliosis,
narrow, long tubular bones. Type 11 is a cen­
tral form characterized by fewer peripheral,
but more intracranial lesions than in type I, in­
cluding acoustic neuromata, spinal involve­
ment, cataract.
Major clinical findings : The type I form
develops in infancy. A varus tibia deformity
(crus varum congenitum) and juvenile pro­
gressive scoliosis are typical. Spontaneous
fractures occur because of the varus deformity
of the lower leg with a reduced healing ability,
leading to pseudo-arthrosis of the tibia. Com­
pression fractures of the vertebrae develop be­
cause of the destruction of the vertebral bod­
ies caused by the neurofibromas leading to
very progressive scoliosis with short curves
and often neurological deficits (Chapter
24. 5 .2 . ) .
Therapy: In cases of scoliosis early surgi­
cal intervention is suggested (spondylodesis
with internal fixation). Treatment of the tibia
pseudo-arthrosis is extremely difficult be­
cause of the poor healing ability. The pse­
udo-arthrosis can be resected and vascu­
larized bone graft can be used, fixed by
llizarov device to compress the site of
pseudo-arthrosis and to attain lengthening
proximally. Unfortunately, amputation is nec­
essary in unsuccessful cases.
 La sz l 6 B u cs i

14. Metabolic diseases of the bone

Metabolic diseases of the bone develop as classifications, they are grouped collectively
a consequence of various disorders related to under the term of involution osteoporosis.
bone formation, mineralization and remodel­
ing. These diseases are generally classified Etiopathology. As a result of the signifi­
into two major categories : one category com­ cant developments in medicine in the past
prises osteopenic conditions, like osteoporo­ 1 00- 1 50 years, life expectancy at birth has in­
sis, osteomalacia, hyperparathyroidism and creased considerably, and the number of de­
osteopenia associated with other illnesses. In cades spent with a deficiency of estrogen and
these cases a decrease in bone substance and gestagenic hormones entail serious risk fac­
inadequate mineralization are observed. tors for the skeletal system. The accelerated
The other category comprises osteo­ pace of everyday life, an unhealthy lifestyle
sclerotic conditions such as Paget ' s disease, (the lack of exercise, phosphate-rich nutrition,
osteopetrosis, which are accompanied by an
increase in bone mass as a consequence of a
bone remodeling disorder.
Osteopenia may develop as a result of a
proportional reduction (30% organic, 70% in­
organic) in the bone components, e.g. in os­
teoporosis, or as a result of shift in this ratio,
as in osteomalacia, which indicates predomi­
nantly a decrease in the level of mineral sub­
stances in the bone. In certain cases, osteo­
penia or a reduction in bone mass occurs lo­
cally (immobilization, inflammation, neo­
plasm), while in other cases it may exhibit a a

b
combined character, and develop in both a
widespread manner and localized (hyper­
parathyroidism) (Table 1 4 . 1 .) .

14. 1. Osteoporosis

Osteoporosis is a generalized, progressive dis­

ease of the skeletal system which involves a
reduction in bone mass, impairment of the
microarchitecture and a decrease in the bone
density while the bone proportions are main­
tained, leading to increased fragility of the
bone.
Fig. 14.1.
Postmenopausal and senile osteoporosis Reconstructed image of normal (a) and osteoporotic
are generally distinguished, although in some (b) cancellous bone tissue 3D-G.
102 � 1 4 . Meta bo l i c d i s e a s e s of t h e b o n e

and a n extremely low calcium intake among
the Hungarian population) and the ever
greater use of medication harmful to the skel­
etal system (steroids, certain antiepileptic and
anticoagulant drugs, thyroid medicines) fur­
ther increase the risk and account for the fact
that in Hungary approximately 3 00000 men
and 600 000 women are currently affected by
osteoporosis.
Osteoporosis is the manifestation of de­
crease in the cancellous and cortical bone sub­
stance. The bone trabeculae decompose, and
microfractures develop on them (Fig. 1 4 . 1 . a,
b). The cancellous bone trabeculae do not dis­
integrate in the same manner throughout the
organism; in the femur neck and the vertebral
bodies, the push-pull traj ection lines persist
for a long time.
Osteoporosis instigates bone fractures. In
a large number of cases, the fracture occurs
following a trivial trauma or a fall, but it may
even result from coughing, sneezing or occurs
spontaneously. Prior to the fracture, osteopo­
rosis does not induce any complaints; it at­
tacks its victims as a silent epidemic.
The fractures habitually situated in typical
locations. Following menopause, as a conse­
quence of the rapid bone density reduction,
predominantly fractures of the cancellous
bone substances (the vertebral body, distal
end of the radius) occur, while as time passes,
the cortical bone mass also decreases.
In old age, coordination disorders, the de­
terioration of eyesight, and dizziness addi­
tionally contribute to elderly patients falling
without protection, and further types of bone
fractures occur; in the hip area, at the proxi­
mal end of the humerus. It has been estimated
that these conditions are responsible for 25-
28 000 fractures at the distal end of the radius,
3 0-40 000 vertebral body fractures, 1 5 000
fractures in the hip area, and 8- 1 0 proximal
humerus fractures each year in Hungary.
Clinical symptoms. The first step in the
recognition process is conventional history
recording, which discloses the risk factors,
and allows identification of the affected popu­
lation with higher efficiency. The most impor­
tant risk factors are the age and a reduced min­
eral bone mass. The proven risk factors of
bone fracture include a low body mass, previ­
ous bone fractures, accelerated remodeling,
the regular intake of glucocorticoids, the exis­
tence of known osteoporosis-causing ill­
nesses and conditions that increase the risk of

Table 14.1.
Differential diagnostics of bone metabolism diseases based on laboratory and X-ray examinations
Diagnosis Serum-Ca Phosphorus Alkaline X-ray Isotope
phosphatase

Osteoporosis N N N Frame, wedge, fish + (in fractu res)

vertebra
Osteomalacia '" '" t Looser-zones, wedge, fish ++
vertebra
Hyperparathyroidism t '" .l' Subperiosteal usuration, +
J, t t
cysts
Secondary renal +
hyperparathyroidism
Paget's disease N N tt Plaited bone structure ++
I nfection N N N Localized lytic-sclerotic +
Tumor metastasis N-.l' N .l' Localized lytic -sclerotic +

(N = normal t = i ncreased J, = decreased .l' = moderately increased '" = moderately decreased

 1 4 . Meta b o l i c d i s e a s e s of t h e bo n e
Fig. 14.2.
Female patient suffering from osteoporosis: increased spinal kyphosis,
protruding abdomen, typical transversal plica on the torso.
accidents. Genetic examinations have fur­
nished an ever increasing number of data con­
firming the role of mutations of the colla­
gen- I A I gene. Among the physical parame­
ters, emphasis should be placed on decreases
in body height and weight, an intensification
of kyphosis, the appearance of transversal
plicas on the trunk, a decrease of the distance
between the lower costal arch and the iliac
crest, and an increase in the ratio in the dis­
tance between the widespread arms and body
height should be emphasized (Fig. 1 4.2.).
X-ray signs : A traditional X-ray examina­
tion facilitates inspection of the shape of the
bone and indicates whether the osteopathy is
of a diffuse or a local nature. The classical ra­
diological sign of osteoporosis is a semi­
transparent vertebral body, with fine vertical
streaks and sclerotic end-plates. The most im­
portant change is in the shape of the vertebra
(end-plate implosion, development of wedge
� 103
Fig. 14.3.
Typical X-ray image of an
osteoporotic spine: "frame verte­
brae" lumbar 3 "fish vertebra ", lum­
bar 5 flat vertebra
vertebrae, biconcavity, fish shaped vertebrae,
total compression of vertebrae, flat vertebrae)
(Fig. 1 4.3.), which is most common along the
thoracic and lumbar section, especially by the
dorsolumbar transition. Multiple compres­
sions often occur. In tubular bones, osteopo­
rosis is manifested in a thinning of the cortical
layer.
An accurate assessment of bone density is
not possible from a traditional X-ray exami­
nation, since it is not sensitive enough. Osteo­
porosis can only be diagnosed in case of a
3 0% decrease in the mineral components. The
appearance of the bones also depends on the
duration of the X-ray exposure.
Osteodensitometric examinations, in
which the patients ' bone mineral component
contents in various positions are compared
with those of a control group of the same age
range, and by means of various measurement
techniques (SXA, pDEXA, DEXA) , are of
fundamental importance in diagnostics. In ev-
104 � 1 4 . Meta b o l i c d i s e a s e s of t h e b o n e

:: Prod;gy I K - [wt!meuhchc_dfx] t,J�,�

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Referencia: ll-U
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bai egOIgomb
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�� Be6rblett uzeoe,,, ,.� �� . •

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T:Starr -. ortClp-tDnyv = PrOOQY H · [w�. . EN I(�
Fig. 14.4.
Bone densitometry evaluation sheet of lu mbal vertebrae performed with a DEXA device.

eryday practice, both peripheral measure­ With the extent of deviation from the juvenile
ments (the distal end of the radius, the peak body mass expressed in standard devia­
corticalis of the central third of the radius, tion (SO) taken as the T-score, the WHO has
phalanges, the heel bone), and central mea­ defined osteopenia (a moderately reduced
surements (the vertebral bodies, the hip area) body mass) as a T-score between -, and -2.5
are also of maj or significance (Fig. 1 4.4.). SO and osteoporosis (a significantly reduced
bone mineral content) T-score less than -2.5.

Table 14.2.
Treatment of different forms of osteomalacia
Disease Vitamin 02 2S-hydroxy Vitamin 03 7,2S-dihydroxy Vitamin 03

Vitamin 0 deficient 50 000 Ulweek

nutrition
Malabsorption 50 000 U/day 20-200 flg/day
Osteomalacia due to 50 000 U/day 20-200 flg/day
anti-convulsion d rugs
Renal osteomalacia 1-2 flg/day
Metabolic acidosis 50 000 U/day 1-2 flg/day
X-chromosome linked 2-3 flg/day
hypophosphatemia
 1 4 . Meta b o l i c d i s e a s e s of t h e bo n e
Laboratory examinations. Laboratory ex­
aminations play an important role in deter­
mining the dynamics of osteoporosis, and in
distinguishing the condition from other osteo­
penic illnesses (Table 14.2.).
Treatment. The following drugs may be
utilized in the treatment of osteoporosis:
� calcium
� Vitamin D derivatives
� Hormone products
� Bisphosphonates
� Tibolone
� Selective estrogen receptor modulators
� Calcitonin
� Anabolic hormones
Orthopedic aspects of osteoporosis. It is
generally the traumatology department that
handles osteoporosis-related fractures of the
hip area and the wrist, in the framework of
emergency care.
Regarding the orthopedic aspect, the treat­
ment of vertebral fractures occurring as a re­
sult of osteoporosis is of significance. The
first step is to establish how many vertebrae
are fractured, and whether the fracture is sta­
ble or not. In most cases, the situation is a sta­
ble fracture with no neurological symptoms.
Conservative treatment is advocated: bed rest
in the first few days, alleviation of the severe
pain: a calcitonin nose spray, muscle relax­
ants, non-steroid anti-inflammatory drugs (in­
creased risk of gastrointestinal ulcer ! ) . Cor­
sets should be prescribed as required by indi­
vidual conditions, since many patients can not
tolerate corsets. In cases of unstable fractures,
semi-flexible, elastic corsets may be pre­
scribed. Early mobilization and careful phys­
iotherapy are important.
Rarely, in case of persistent complaints,
severe pain or neurological symptoms, sur­
gery may be suggested, removal of the verte­
bral body fragments forced into the vertebral
canal, and stabilization of the spine (flexible
thoracolumbar transpedicular fixation). Per­
cutaneous vertebroplasty is a modem method,
in which liquid bone cement is injected into
� 105
the vertebral body i n question under image in­
tensifier control; as is kyphoplasty, which en­
tails inflating a balloon in the affected verte­
bral body, and then inj ecting liquid bone ce­
ment into it.
It is important for the orthopedic surgeon
to be conscious of the fact that screws and
wires loosen more easily from weakened
osteoporotic bones. Hence more stable fixing
techniques should be utilized: in spine, a lon­
ger section has to be secured than for normal
patients, and in hip arthroplasty, a cemented
implant is advisable.
14.2. Osteomalacia
(mollities ossium)
Osteomalacia i s a metabolic disease reflecting
a disorder in the mineralization of the newly
formed osteoid, which results in the develop­
ment of an osteopenic condition, with all the
relevant symptoms.
Pathology. Many factors may influence
the development of osteomalacia. A vitamin
D deficiency, a disorder in the its synthesis, an
absorption disorder caused by bowel illness,
acquired or hereditary liver and kidney dis­
eases, and intoxication caused by various
heavy metals can all be root causes. Osteo­
malacia often arises in the elderly, besides the
lack of sunshine, this reflects the absorption
disorders developing in such patients, result­
ing in metabolic diseases of the bone. Com­
monly prescribed anti-convulsion drugs cause
osteomalacia through the enzyme induction
taking place in the liver.
Osteomalacia can often be observed in pa­
tients undergoing dialysis, the main reason for
this condition is aluminum intoxication. In a
significant proportion of the cases, patients
are diagnosed with a combined condition des­
ignated osteoporo-malacia.
Clinical symptoms. The clinical diagno­
sis of osteomalacia is often problematic, since
the patients ' complaints are not specific; the
symptoms include muscle weakness in the
106 � 1 4 . M eta b o l i c d i s e a s e s of t h e b o n e
" treatment with 1 ,5 g Ca/day.
" Looser zones on the meta- diaphysis o f both tibia
(marked by arrows, biplanar X-ray of tibia).
proximal limbs and diffuse pain. The patients
are generally gracile in build and suffer from a
hypotonic condition.
X-ray signs: From a radiological aspect,
the disease often imitates osteoporosis, but the
appearance of pseudo fractures or Looser
zones implies a certain level of osteomalacia.
"Looser" zones are radiolucent areas that are
the healing zones of fractures resulting from a
series of micro stress impacts, where the
newly generated bone did not mineralize, it
contains osteomalacic bone (Fig. 1 4.5.). Such
zones appear most commonly in the femur,
the tibia, the radius, the ulna, the rami of the os
pubis and the ribs. In a significant number of
cases, the Looser zone completed and a frac­
ture occurs. Because of the softness of the
bones, the varus position of the femur neck,
protrusio acetabuli, and intrusion of the sa­
crum into the small pelvis ( heart shaped"­
"
pelvis) is common. The disorders ofthe verte­
brae are similar to those observed in osteopo­
rosis (wedge, fish-shaped vertebrae) .
Laboratory signs. Diverse forms of
osteomalacia may demonstrate different
signs. Both increased alkaline phosphatase
levels and low Ca and P levels are often char­
acteristic of osteomalacia (see Table 1 4. l .).
The result of assessment of the patient' s vita­
min D metabolites may also be decisive (see
Chapter 1 4 . 1 ) .
I n many cases, a bone biopsy i s necessary
in order to establish an exact diagnosis. The
result of the histology will unquestionably re­
veal the extreme rises in the breadth and ex­
tent of the osteoid edge, and, tetracycline trac­
ing can help in the confirmation of mineral­
ization disorders .
Treatment. The modes of treatment of
different forms of osteomalacia are summa­
rized in Table 14.2.
All patients should receive supplementary
In renal osteomalacia caused by aluminum
intoxication, the prescription of deferro­
xamine is advised.
In case of hypophosphatemia, 1 -2 g phos­
phorus /day is also reasonable.
14.2. 1. Rachitis
(rickets, avitaminosis D)
Rickets is a childhood bone metabolism disor­
der (osteomalacia), in which the mineralization
of the newly formed osteoid is insufficient on
accou nt of vitamin D deficiency, reSUlting in
the development of an osteopenic condition
bearing all the applicable symptoms.
Nowadays, vitamin D deficiency - related
rickets scarcely occurs in Europe, which can
be explained with the revolutionary transfor­
mation of nutritional factors and the adequate
vitamin D supplements given to infants. More
often, however, so called vitamin D resistant
rickets, the Fanconi syndrome or phosphate
diabetes is encountered, when characteristic
symptoms may develop in spite of vitamin D
treatment.
 1 4 . Meta bo l i c d i s e a s e s of t h e b o n e � 107

a b c

Fig. 14.6.
a. Rachitic child of abnormally low heig ht, with quadrate skull, varus deformity of limbs.
b. On this a-p x-ray of the limbs, the varus deformity of the femur-neck and the knees and the cup shaped
expansion of the metaphysis is clearly observable.
c. The former contour of the vertebral body in the new, remodeling vertebrae is observable for a consider­
able while even after treatment (bone in bone).

Clinical symptoms. Cranium deformity, X-ray signs. The expansion of the

square head (caput quadratum) is typical,
characterized by thin, fragile skull bones
(craniotabes). Soft ribs and sternum can cause
pectus carinatum, rickets rosary in the bone -
cartilage border, and Harrison ' s groove may
be diagnosed due to the adherence of the dia­
phragm. The stomach protrudes forward, the
pelvis tightens, and the epiphyses widen, es­
pecially on the knees and the wrists. The long
tubular bones of the limbs deform, varus or
valgus deformity may be observed in the
knees, furthermore, a "duckling-like" walk
may develop owing to the coxa vara (Fig.
14.6, a, b, c) . Bones are fragile, incomplete,
so called greenstick fractures may occur. If
the patient remains without treatment for a
long while, a significant decrease in body
height may be observed.
epiphysical fugues, cup shaped metaphyses
and the curving of the long tubular bones are
characteristic radiological symptoms (Fig.
1 4.6, a, b, c).
Laboratory signs. Hypophosphatemia
and elevated alkaline phosphatase enzyme
levels.
Treatment. Already after two weeks, the
effect of 5 00- 1 000 U/day of vitamin D can be
radiologically detected. The curving of the
limb bones generally remodel spontaneously,
even in case of a significant deformity (20 de­
grees), there is seldom need for a correctional
osteotomy. The absence of spontaneous cor­
rection is often the consequence of insuffi­
cient vitamin D treatment.
108 � 1 4 . Meta b o l i c d i s e a s e s of t h e b o n e

14.2.2. Vitamin D resistant rachitis may develop as a result of the adenoma ofhor­
mone-producing cells (primary hyperpara­
(Fanconi syndrome, phosphate
thyroidism), renal hypo- or hyperphos­
diabetes) phatemia, Calcium or vitamin D deficiency
(secondary hyperparathyroidism), or as a re­
Earlier, this was considered to be a rare
sult of parathyroid gland hyperplasia induced
disease, but nowadays, it is a frequently diag­
by secondary hyperparathyroidism (tertiary
nosed illness, regarded as the most common
hyperparathyroidism).
reason for dwarfism. The foremost character­
istic of vitamin D resistant rachitis is that it
Etiopathology. Elevated secretion of the
does not react to normal dosages of Vitamin
parathormone augments bone re sorption by
D.
increasing the osteoclast activity. The
corticalis of the tubular bones softens, cortical
Etiopathology. Nowadays, renal tubular
cysts develop, the bone marrow undergoes a
defects are considered to be the most frequent
fibrotic transformation. Because of these typi­
pathophysiologic conditions, such as Fanconi
cal disorders is the illness also labeled
syndrome and phosphate diabetes. Compared
osteodystrophia fibrosa cystica generalisata.
to rachitis caused by vitamin D deficiency, the
(van Recklinghausen 's disease). The dimin­
foremost difference is that the development of
ished carrying capacity of the skeleton may
the disease depends on hereditary factors,
lead to fractures involving intraosseous hem­
muscle weakness and hypocalcemic tetany
orrhage. After healing, the residue of the
are not characteristic, the serum phosphate
bleeding, a resorptional giant cell granuloma,
level is low and even following treatment,
also known as the "brown tumor" is a typical
does not reach the normal level. Dwarfism is
finding.
characteristic, despite treatment (renal dwarf­
ism) .
Clinical symptoms. Early clinical symp­
Symptoms generally match those charac­
toms are renal calculi, rheumatic pain in the
teristic of rachitis caused by avitaminosis D .
members caused by chondrocalcinosis and
stomach pain on account of a peptic ulcer. All
Treatment. Prescription of 1 ,25-dihydr­
symptoms are consequences of hyper­
oxi D3 vitamin is suggested (see Table 1 4.2.),
calcemia, which leads to the crystallization
the curving of the long tubular bones often re­
and deposition of calcium pyrophosphate
quires surgical correction, sometimes includ­
ing limb - lengthening.
X-ray signs. Usuration on the mandible
and on the phalanxes and subperiosteal re­
sorption are early symptoms. The radiological
14.3. Hormonal metabolic bone image of the "brown tumor" may imitate that
diseases of an osteolytic bone turnor (Fig. 14.7. a, b.).
Generally, multiple lytic, sharp-edged de­
structions are observed in the meta-diaphysis
14.3. 1. Hyperparathyroidism of long tubular bones, extending to the
(Osteodystrophia fibrosa cystica corticalis, with no periosteal reaction.
generalisata, von Recklinghausen's In childhood, proximal epiphysiolysis of
disease) the femur is also experienced.

Hyperparathyroidism is an osteopenia Laboratory signs. Hypercalcemia is deci­

causing metabolic disease of the bone of an sive in declaration of the diagnosis, elevated
endocrinological origin that develops as a re­ phosphorus and alkaline phosphatase enzyme
sult of elevated parathormone production. It levels are also typical.
 1 4 . Meta b o l i c d i s e a s e s of t h e b o n e
a b
Fig. 14.7.
Multiple lytic lesions in the ulna (a), femur and tibia
(b) caused by osteodystrophy fibrosa cystica
generalisata (van Recklinghausen's disease).
Treatment. Removal of the parathyroid
gland adenoma, with the obj ective of
re-establishing the Ca balance. In most cases,
the bone-defect fills up spontaneously, but in
case of a bone-fracture hazard, the scraping
out of the giant cell granuloma, filling the
bone defect, spongiosa plasty may come into
question, along with the stabilization of the
bone if necessary.
� 109
14.3.2. Hypogonadism
(Adiposogenital dystrophy,
Frohlich's disease)
A bone metabolism disorder explained by
deficiency of gonadotrope hormones that is
accompanied by distinctive obesity taking
place during puberty.
Genitals and the secondary sexual charac­
ter are underdeveloped; regarding the ortho­
pedic aspect, the often observed valgus defor­
mity of the knees, the femur epiphyseolysis
(see chapter 2 8 . 1 .) and kyphosis of the spine
are to be emphasized.
14.3.3. Cushing's disease
A syndrome based on abnormally high
production of glucocorticoid hormones by the
adrenal cortex, accompanied by a bone me­
tabolism disorder. A prolonged, large dose
glucocorticoid therapy may lead to similar
disorders.
Clinical symptoms. Upper body obesity,
rounded face and stretch marks appearing
mainly on the abdomen and the hips are all
very characteristic symptoms. On the skeletal
system and primarily localizing on the verte­
bra, extensive osteoporosis causing fish
shaped vertebrae, kyphosis and scoliosis ap­
pear.
This condition is treated with steroids.
Osteonecrosis may often occur during this
kind of treatment, especially on the head of
the femur, entailing a truly severe prognosis.
Treatment. Besides treatment of the basic
illness, namely the normalization of the hor­
mone system, the prosthetic replacement of
the hip j oint may be indicated in case of hip
joint damage (see chapter 2 8 . 2 . ) .
 T i b o r Vfz k e l e t y

1 5. Developmental anomalies of the

extremities

These diseases can be hereditary or caused • the lack of differentiation or separation of

by noxious factors during intrauterine life.
The origin of transverse extremity absences is
exogen one, while hereditary factors cause
mainly longitudinal anomalies such as
polydactyly, syndactyly, cleft hand and cleft
foot.
The classification of the developmental
anomalies:
• the development of certain parts of the
extremity is deficient,
certain parts,
• duplication,
• overgrowth (gigantism),
• u ndergrowth (hypoplasia),
• congenital constriction bands syndrome,
• generalized deformities of the skeletal system.
The most important group from this classi­
fication is the first. However there are no
clear-cut borders between the different
groups.

o
pOOO
0 000
0 ° 0 0
0 0 0

Fig. 15.1.
Combined longitudinal defects o f lower extremity. Femur and fibula hypoplasia (a), partial absence o f femur­
total absence of fibula, metatarsi IV-V, and toes (b), absence of the proximal half of the femur and absence of
the fibula, metatarsi IV-V. and the toes (c), total absence of the femur, fibula, metatarsus V. and toes with talo­
calcaneal coalition (d), absence of the femur, tibia, fibula, tarsal bones and metatarsi I I-V. and toes (e), total
lack of the lower extremity (f).
1 12 � 1 5 . Deve l o p m e n t a l a n o m a l i e s of t h e ext r e m i t i e s
The lack of some part is described with the
word defect. The anomaly is transverse if the
total cross-section of the extremity is absent.
The anomaly is said to be longitudinal if the
absence of the extremity is only partial.
Hypoplasia means that the whole bone is
present, but is underdeveloped. In longitudi­
nal developmental anomalies, the name of the
affected bone is used: radial, ulnar, metacar­
pal, phalangeal, tibial, fibular) (Fig. 1 5 . 1 .) .
In longitudinal developmental anomalies
the proximal bone of the extremity or one
a but it led to serious consequences. Between
Fig. 15.2.
Bilateral total a bsence of the femur (a) and an ante­
roposterior radiographic picture of this anomaly (b).
bone of the distal parts in a medial, central or
lateral position may be absent (Fig. 15.2. a, b).
In an upper extremity there may be a prox­
imal (humerus) defect previously named
phocomelia (Fig. 1 5.3.), or a distal radial de­
fect combined with hypoplasia of the radial
fingers or with a partial lack of the metacarpal
and carpal bones, or even with a total lack of
the radial ray. This type of developmental
anomaly can also occur on the ulnar side.
The longitudinal combined defect can
comprise for example the joint lack of the hu­
merus and radius or the humerus and ulna. In
case of a partial or total lack of the humerus
and the bones of the forearm (complete de­
fects of the humerus, radius and ulna) the
hand develops directly from the shoulder, re­
sembling the swimming leg of a seal
(phocomel ia).
Cleft hand and foot is a peripheral longitu­
dinal defect (manus fissa, pes fissus).
A very high incidence of developmental
anomalies, mainly of the extremities was de­
tected in the newborns after women took
Contergan pills during pregnancy. Luckily
this tragedy was restricted in time and place
1 95 8 and 1 963 , pregnant women took these
tablets for sedation Contergan pill which con­
tained thalidomide in West-Germany and
other European countries. During that period
more than 1 0.000 children were born with se-
Fig. 15.3.
Phocomelia: the left forearm is joined directly to the
shoulder.
 1 5 . Deve l o p m e n t a l a n o m a l i es of t h e ext r e m it i e s � 1 13

vere developmental anomalies, such as lack of

an extremity. This tragic series was termi­
nated after these tablets were withdrawn from
the market, but the sad consequences remain.
Therapy. The first decision to be made is
to improve the function of the affected ex­
tremity. The aim must always be to provide
the patient with better function.
In case of total absence of upper extremity
the lost hand function can be substituted with
a prosthesis, but it is sometimes better to prac­
tise good grasping movement of the foot in or­
der to supplement the hand function. These
a
patients learn to eat, drink and draw with their
b foot. It is sometimes necessary to elongate a
short extremity stump so as to improve useful­
ness of the prosthesis.
Careful consideration must be given to
what is advantageous for a better function in
case of a contracture of the joint (flexion
contracture of the elbow, clubhand) taking
into account the length of the extremity. Is it
better with or without the correction achieved,
by elongation of the short bones?
In certain cases the function of the extrem­
ity can be improved by surgical intervention.
For example in clubhand caused by the ab­
sence of the radius, this can be corrected by
soft tissue release and later by arthrodesis of
the wrist (Fig. 1 5.4. a-c). In cases of cleft hand
and foot the function of the hand can be im­
proved by bringing the diverted rays closer to­
gether and giving the possibility to wear a nor­
mal shoe. A missing tibia can be substituted
by transplantation of a contralateral or
ipsilateral fibula (Fig. 1 5.5. a-e).
An effort must be made to clarify the
cause. So that teratogenic harm can be
avoided. With inherited defects, intrauterine
diagnostic methods (for example ultra­
sonography) may be used to distinguish be­
tween the healthy and unhealthy fetus.
Function can not be subordinated to esthe­
tic considerations. The very early use of vari­
ous prostheses is suggested in cases of serious
absences in the upper and lower extremities.
Fig. 15.4. Modem surgical techniques can prove of
Clubhand (partial absence of the radius) (a- b) and ra­ great help to children born with developmen­
diographic picture (c) of the anomaly. tal extremity anomalies.
1 14 � 1 5 . Deve l o p m e n t a l a n o m a l i e s of t h e ext r e m i t i e s

a c

b d

Fig. 15.5.
Lack of the distal two-third of the tibia in an 18 month old boy. The fibula was transplanted to the calcaneus
and proximally into the tibia. A good bony consolidation was achieved. Clinical picture of the extremity be­
fore the operation (a) and a radiographic picture (b). Clinical pictu re after the operation (c) and a radiographic
picture (d). Outline of the surgical procedu re (e).
 T i b o r Vfz k e l e t y

16. Neuromuscular diseases

16. 1. Spinal dysraphisms, case of a disturbance of the development the

myelodysplasia
Myelodysplasia is part of a spectrum of
deformities resulting from failure of the neu­
ral tube to close, accompanied by paresis.
16. 1.1. Meningocele,
myelomeningocele, myelodysplasia
Meningocele and myelomeningocele is a pro­
trusion of the membranes of the spinal chord
through a defect of the vertebral column,
usually in the lumbar region. Myelodysplasia is
a developmental abnormality of the spinal
chord.
closure is incomplete and certain parts of the
neural tubes remain open. The severity of the
disease depends on the extent of the closure
disturbance.
In the mildest form of the disease only the
closure of the bony elements are disturbed,
one or a few vertebral arches remain open in
the radiographic picture (spina bifida occulta)
(Fig. 1 6 . 1 .) . This occurs most frequently on
the sacral vertebra I. and lumbar vertebra V .
and rarely causes complaints. Lumbar hyper­
trichosis and a pes exavatus deformity ob­
served around the age of l O- 1 2 years, together

Spina bifida usually presents no external

sign apart from hypertrichosis, but the radio­
graphic picture indicates widening of the ca­
nal and the loss of posterior elements are ob­
servable in spina bifida.

Incidence. Meningo- and myelomeningo­

cele : 0, 1 -0,2 %, spina bifida : 1 5 %.
The reason why the spine and the neural
tube fail to close is not known. The probabil­
ity of the occurrence of this condition in the
next newborn in the family is 5% if it is pres­
ent in one sibling, and 1 2 % if it is present in
two of the siblings.
In cases of spinal dysraphism the increase
in the level of alpha-fetoprotein is so signifi­
cant after the sixteenth week of pregnancy
that termination of pregnancy is the likely out­
come. This laboratory test is suggested if the
mother has previously had pregnancy involv­
ing a child with myelodysplasia.
During development, the spinal cord
forms from the neural plate as a neural tube. In Spina bifida occu lta, sacral arch I. is opened.
1 16 � 16. Neuromuscu lar d iseases

with persisting enuresis nocturna, focuses the
attention on this developmental disease. Spina
bifida can cause lumbar pain during adoles­
cence and adulthood.
When the radiographic picture shows a
widening of the spinal canal (spina bifida), ac­
companied by a disturbance of the spinal cord
development, (myelodysplasia), in all proba­
bility there will be a decreased ability of the
spinal cord to move upward (tethered cord) .
Spondylolysis often presents in spina bifida.
� Meningocele
Large, wide spinal cord layers filled with
cerebrospinal fluid p rolapse through the usu­
a lly relatively long gap in the posterior verte­
bral arch, the neural elements are sometimes
intact. The spinal cord layers are covered by
vulnerable skin.
ric surgeons, urologists and orthopedic sur­
geons. Surgical care immediately after the
birth can prevent spinal cord septic inflamma­
tion. It is necessary to guarantee the free flow
of the cerebrospinal fluid because of the hy­
drocephalus itself or the increase in the hydro­
cephalus. The treatment of incontinence and
the prevention of urinary tract infections are
very difficult. Because of the loss of sensa­
tion, close attention must be paid to the care
and nursing because the patient can easily suf­
fer burning and scalding.
The severity of the disease can vary
widely, depending on the extent of the neural
defects, ranging from a mild form involving
only a few muscles up to a very serious condi­
tion or even a fatal outcome. Fortunately in
most myelomeningocele patients with lower
extremity paralysis the ability to stand and

� Myelomeningocele

The neural tube is widened, and the neural

tube, the spinal cord itself participates in the
formation of the wal l of the prolapsed neural
sack (Fig. 16.2.).

In case of open myelomeningocele, the

spinal cord is not covered by either skin or a
neural layer and therefore it is freely open to
the outside world. Additionally the neural
tube may be open.
All forms of this closure disturbance occur
most often in the region the of lumbar and sa­
cral spine, with different degrees of extension
cranially. Only cervical or only thoracic spi­
nal dysraphism is rare.
An open neural tube leads to neurological
symptoms e.g. typically the paralysis with dif­
ferent degrees of involvement, mainly affect­
ing the lower extremities.
The height of the spinal lesion determines
the distribution and extent of muscle paralysis
and the subsequent abnormal j oint conditions
and deformities.
Fig. 16.2.
Therapy. Successful treatment demands Clinical picture of a child with myelomeningocele af­
well-organized co-operation between pediat- ter primary su rgical closure.

walk can be ensured by the complex orthope­
dic treatment and surgical interventions and
the use of orthosis. As these patients are usu­
alJy mentalJy normal and live to an average
age, every effort must be made by the ortho­
pedist to help them to be self-supporting and
to be able to work independently. A seriously
paralyzed patient should, ifpossible be able to
walk a few steps with a lower extremity
orthosis, walking sticks, or crutches and to be
able to use a wheelchair or drive a car. The
ability to perform independent movement is
very important because this is the way to be
realJy a part of society.
In consequence of the disturbance of the
muscle balance special deformities develop in
the paralyzed lower extremities. These defor­
mities cannot be prevented by plaster re­
dression or the use of different orthoses. Only
reconstruction ofthe muscle balance can lead
Fig. 16.3.
Radiographic picture of a myelomeningocele pa­
tient. The open vertebral canal appears with a
wedge-shaped vertebra and subluxation of the
right hip.
16. Neuromuscular d iseases � 1 17
to a good result. During the surgical correc­
tion of these deformities, the aim is a total and
final correction because postoperative plaster
redressement will not give further improve­
ment. Different contractures and deformities
develop, depending on the localization and
extent of the paralysis. The most frequent de­
formities :
Deformities of the spinal column. Lack
of the posterior vertebral arch, developmental
anomalies of the vertebral body, half­
vertebra, wedge-shape vertebra, kyphosis,
lordosis or scoliosis because of the paralysis
of the spinal muscles. Deformities can appear
at birth or can develop later (Fig. 1 6.3.).
Hip j oint deformities. The hip j oint is
usually in flexion and in external rotation
contracture. During a rest situation the hips
are in a flexed, abducted and externally ro­
tated position. An adduction contracture pres­
ents when the paralysis occurs under nerve
roots L3 -L4.
A valgus deformity of the femoral neck
and increased antetorsion can be seen in the
radiographic pictures. Hip subluxation or
luxation often develops.
Hip dislocation develops at birth or during
the first three years of life in more than half of
the patients with myelomeningocele (Fig.
1 6.3.).
Knee j oint deformities. A recurvatum de­
formity, flexion contracture or valgus
mal alignment of the knee presents in conse­
quence of muscle paralysis. Genu recurvatum
is often combined with anterior subluxation of
the knee.
Deformities of the foot. Foot deformities
are observed in more than half of the patients
with myelomeningocele: equinus deformity,
equinovarus deformity, calcaneus deformity,
calcaneovarus deformity, vertical talus, pes
excavatus.
Foot deformities must be corrected surgi­
cally before the beginning of walking. In
myelomeningocele, conservative treatment
1 18 � 16. Neuromuscu lar d iseases

lesion. The incidence is 2-3 %0 in every live

births.

Major clinical findings. Retardation of

mental functions and movement development
is observed, with spasticity, involuntary
movements, ataxia.
The abnormal cerebral development can
lead to different groups of symptoms, which
are determined not by the character of the
damage but by the localization and the dura­
tion. The primary brain damage itself does not
progress; as a special feature of the disease,
the cerebral development can proceed in an
abnormal direction.
The cerebral palsy disease group can be
classified on the basis of the predominant
clinical symptoms :

� Spasticity 75 %
� Dyskynesis 10 %
� Ataxia 15 %
Fig. 16.4.
Tremor, dystonia and atonia are relatively
A myelomeningocele patient with left-side lower ex­
tremity paralysis, supported by a fou r level fixed leg
rare, and mixed clinical forms can develop.
walking orthosis. From an orthopedic point of view the most
important group of cerebral palsy is the spas­
tic type, which can be greatly helped by ortho­
with plaster redressement and orthoses is not pedic surgery. Spasticity can be subgrouped
as successful as in other congenital anomalies. according to the localization and extent (Ta­
The ability of children with myelo­ ble 1 6 . 1 .).
meningocele to walk. Nearly all patients with
myelomenigocele, who have good mental and Anamnesis. The circumstances of the de­
renal functions will be able to walk independ­ livery are very important. Abnormalities dur­
ently supported by braces and a walking ing pregnancy can impair the development of
orthosis after successful early surgical correc­
tion of the deformities; ten or fifteen meters
Table 16. 1.
with walking orthosis or stick or crutch, in
Classification of spasticity
milder cases even kilometers (Fig. 1 6.4.).
Larger distances can be covered by wheel­ Localization Cause
chair or special car. Hemiplegia lateral half of the perinatal
body hemorrhage
Diplegia lower extremities cerebral anoxia
16.2. Cerebral palsy (infantile (premature)
cerebral paresis, Little disease) Quadriplegia all four cerebral
extremities, and developmental
Cerebral palsy is a psycho-senso-motor distur­ the whole body anomalies
bance caused by a pre- or perinatal cerebral Monoplegia one extremity
 1 6 . N e u ro m u s c u l a r d i s e a s e s � 1 19

the central nervous system, the fetus sustain­

ing a loss of oxygenisation. Detailed
informations must be acquired on the delivery
process. A long or a rapid delivery, involving
the use of forceps, can lead to intracranial
hemorrhage. An abnormal uterus contraction
rhythm, for example with too long womb con­
tractions, can cause fetal cerebral anoxia.
A premature neonate is much more vul­
nerable and can suffer damages more easily
even during otherwise normal delivery. Im­
portant information concerning the health sta­
tus of a newborn can relate to neonatal as­
phyxia, the need for reanimation, treatment in
incubator, the level of neonatal icterus, trans­
fusion.
Little disease must be considered, when
the newborn is retarded in movement devel­
opment (does not turn, sit and stand up at the
expected time) .
The most frequent complaints in a child
who can walk are crossing of the lower ex­
a
tremities, flexed knees, and walking on tiptoe
b
because of the equinus deformity of the an­
kles .

Examination. The disease can show dif­

ferent levels of severity from the very mild
form of cerebral palsy that is very difficult to
diagnose in an elder child to the very serious
type presenting immediately after the birth. At
the start of the examination the child is in a su­
pine position. During the observation the baby
may display involuntary movements and a
typical position of the j oints. The mobility of
the articulations and tension of the muscles
are examined first, followed by the ability to
hold the neck-head in infants and the ability to
sit, stand and walk in early childhood.

Major clinical findings. After the age of

Fig. 16.5.
The typical body posture of a cerebral palsy patient;
lateral view (a) and anterior view (b).
two years the Babinsky reflex and the
Oppenheim reflex are pathologic symptoms.
The tendon reflexes are enhanced, the
reflexogenic zones are wider, clonus presents.
The upper extremities exhibit pronation
spasm, the supination of the forearms is lim­
ited, the patient can not turn the hands up­
ward. The first symptoms of athetosis present
120 � 1 6 . N e u ro m u s c u l a r d i s e a s e s

only at the age of two-three years as special
movements of the fingers, hands and feet.
In early childhood, the previous symp­
toms become more expressed, without any
progression of the disease (Fig. 1 6.5. a-b) . In
mild cerebral palsy the following symptoms
may be seen:
� the hands of the ch�d a re clumsy,
� the walking is not harmonious,
� the child close the knees each other and
flex,
� the child does not put down the heels.
In severe cases, mental and motor retarda­
tion appears .
It is important to determine the mental
state and motor condition of the patient be­
cause these greatly influence the therapy plan.
Spastic patients pose not only medical
problems, but also social and pedagogic prob­
lems. The parents must be informed about the
possible cerebral palsy disease in time after
the presumed medical diagnosis and a therapy
plan must be developed in co-operation with
them. Besides the movement pedagogy and
orthopedic surgical and conservative therapy
the question ofeducation too has to be solved.
The intramuscular inj ection of botulinum
toxin can temporarily decrease the spasm of a
muscle or a group of muscles. Repeated injec­
tions provide a longer effect. It must be re­
membered that the children with infantile ce­
rebral palsy will become adult cerebral palsy
patients. Hopefully the conservative treat­
ment methods and interventions enable them
to live an independent and self-supporting
life. The goal is to provide them with the fol­
lowing abilities, in order of importance:
� the ability to communicate
� the activity necessary for a normal daily life
� mobility, social integration and if possible,
employment
� ability to walk
Orthopedic operations are very important
in the treatment of cerebral palsy. It is essen­
tial to choose the best type of operation, with a
good indication and also to optimize the tim-

Therapy. Before treatment the level of

damage of central nerve system must be mea­
sured accurately, and the vision, hearing and
speech ability must be assessed. In about
two-third of children with cerebral palsy the
intelligence quotient is under 70. The therapy
plan must take into consideration the fact that
more serious the level of mental retardation,
the less effective the therapy.
b
Therapy can start at one-two months of
a
age . Adequately selected special exercises
can prevent or decrease the development of
the typical contractures and deformities. The
aim is to provide to the patient with the chance
of an independent life. The conductive peda­
gogic method developed by Andrew Peto (In­
ternational Peto Institute, Budapest) can lead Fig. 16.6.
to very good results in the treatment of cere­
Typical postu re and contracture of the lower ex­
bral palsy. tremity and lu mbar spine in frontal plane (a) and in
the sagittal plane (b).

ing of the surgical intervention. The need for
further more serious surgeries to correct ma­
jor deformities can be avoided if one opera­
tion is performed at the appropriate time. The
most typical positions and contractures of
joints in cerebral palsy patients are as follows :
In the sagittal plane: increased lordosis,
flexed hips, flexed knees, equinus in the an­
kles. The muscles of the lower extremity cre­
ate a closed kinetic train. In order to place the
center of gravity just above the lower support­
ing point on the ground, one of the j oints must
adopt an abnormal position, which will be fol­
lowed by the malposition of the other two ar­
ticulations. For this reason the deformities and
contractures in all of three j oints must be
solved at the same time (Fig. 1 6.6.).
Fig. 16.7.
Release of the muscles around the hip. Tenotomy of
the muscles on the spina iliaca anterior superior and
inferior and tenotomy of the adductor muscles. a:
muscles on spina iliaca anterior su perior and inferior,
b: adductor muscles
16. Neurom uscu lar diseases � 121
� In the frontal plane: the hip adductors are
in tension, creating contractures and the
calcaneus is in valgus or varus to com­
pensate the equinus contracture.
� In the horizontal plane: the hips are in an
internally rotated position, which, with the
adduction spasm makes walking difficult.
The most common orthopedic operations
in the surgical treatment of cerebral palsy :
open adductor tenotomy, tenotomy of the
iliopsoas (Fig. 1 6.7.), varization-derotation
osteotomy of the femur (Fig. 1 6.8.), open re­
position of the hip joint, innominate
osteotomy, posterior transposition of the
adductor muscles, knee flexor tenotomy,
Achilles tendon lengthening (Fig. 1 6.9. a-c),
Fig. 16.8.
Luxation of the left hip and subluxation of the right
hip in a cerebral palsy patient (top). After surgical re­
positioning and femur varization-derotation
osteotomy (bottom).
122 � 16. Neuromuscular d iseases
Fig. 16.9.
"Z" shaped Achilles tendon lengthening to treat
equinus contracture (a), recession of the gastrocne­
mius muscles (b) and Achilles tendon lengthening
according to Hoke without sutu res (c).
recession of the gastrocnemius, Grice opera­
tion.
Discussion with the parents should stress
that the operations merely offer a possibility
for improvement in the movements and am­
bulation of the patient, but further long exer­
cises are necessary to utilize the effects of the
intervention.
The operations are rarer on the upper ex­
tremities.
Orthopedic surgeons often order special
corrective orthopedic shoes to improve the
function of deformed feet, and in special cases
a walking orthosis to stabilize the knee or an­
kle joints and exclude abnormal movements .
Medications can be administered to de­
crease the spasticity in any phase of the treat­
ment.
A proportion of the cerebral palsy patients
will gain independence after the course of
treatment, and will be able to take up employ­
ment.
16.3. Poliomyelitis
(poliomyelitis anterior acuta,
Heine-Medin disease)
Epidemic poliomyelitis is a disease that
earlier caused serious problems. After such
epidemics, crowds of partially paralytic pa­
tients were in need of treatment in hospitals,
and sometimes they had to live their life in an
"iron lung " . After long courses of physiother­
apy, exercises, and series of corrective opera­
tions or the use of a walking orthosis, others
regained their abilities to be self-supporting
and to work.
Acute poliomyelitis is a viral infection that
damages the anterior horn cells and brain
stem motor nuclei, causing flaccid paralysis.
One of the majors result in medicine was
the development of an effective vaccine
against acute poliomyelitis. Salk made a vac­
cine from the killed virus, but the vaccine pro­
duced by Sabin from the living, but weakened
poliomyelitis virus was much more effective
and its use was easier in the form of oral
drops. Where the Sabin-drops are used as pro­
phylaxis, epidemic poliomyelitis practically
no longer exists . Because of the well orga­
nized prophylaxis acute poliomyelitis has not
occurred in Hungary since 1 960.
Major clinical findings. In the acute stage
the symptoms of acute poliomyelitis appear
after an incubation period of one-two weeks.
Following the sudden onset, fever, headache,
throat ache, resembling systemic influenza
are often followed by a fever-free period and
subsequent meningeal symptoms. Paralysis of
the muscles suddenly appear after a two-three
days of fever, the patients often waking up
with paralyzed muscles. The paralysis varies
in extent, the lower extremities are involved
more frequently but it can extend to the trunk
and the upper extremities too. If the brain
stem is affected, cerebral nerves, the nervus
phrenicus and intercostal nerves can also be

paralyzed leading to the possibility of as­
phyxia.
After an acute stage lasting five-seven
days further paralysis does not usually occur,
and a gradual improvement may begin. In the
convalescent stage, the recovery phase lasts
for a period of about two years. Most recovery
takes place during the first few months.
Therapy. The most important goals are to
strengthen the weakened muscles by active
exercises, to replace the function of the para­
lyzed muscles, and to prevent the develop­
ment of contractures. In a definitive condition
the ability of the patient to move can be im­
proved by means of different operations and
the use of special orthoses. In suitable cases
the function of paralyzed muscles can be sub­
stituted by transposition of an other one .
The limitation of the range of movement
of articulation with a surgically created bony
bumper (arthrorisis) can promote the useful­
ness of the extremity. The arthrodesis of the
joints stabilizes the extremity and the patient
will be able to put more body weight on the
knee for example. Resections of articulations
give a possibility to correct deformities that
develop due to paralysis.
The different special orthoses are intended
to stabilize the j oints.
Severe scoliosis can develop after paraly­
sis of the trunk muscles. An attempt should be
made to stop the progression of secondary
scoliosis by wearing corrective braces, but
surgery is sometimes necessary to correct
contracture of the spine and to create spondy­
lodeses in the deformed spinal region.
16.4. Developmental anomalies and
diseases of the muscle system
The developmental anomalies and diseases of
the muscles are most often observed in as
muscle absences, systemic disorders and
contractures.
It is important to clarify the beginning of
the disease, the initial localization and the de-
16. Neurom uscu lar d iseases � 1 23
tails of progression according to the present­
ing complaints . To assess the function of the
muscles, it is often enough to observe the
daily movement activity of the patient. The
absence of muscles, weakened muscles or
paralytic muscles can be detected by careful
inspection of the movements of the patient. In
further examinations strength of different
muscles, the direction and extent of limitation
of the joint range of motion can be deter­
mined. It is hard to examine the active mo­
tions in an infant or a small child because of
the lack of cooperation. Serum enzyme tests,
electromyography and muscle biopsy can fa­
cilitate the diagnosis in uncertain cases.
Electromyography can help in the differentia­
tion of diseases of myogenic or neurogenic or­
igin. In newborns, measurement of the nerve
conduction speed can help with the diagnosis
if peripheral nerves are involved.
16.4. 1. Congenital m uscle absences
The most frequent such condition is the to­
tal or partial absence of musculus pectoralis
major. In this case the sternocostal part of the
muscle is missing. The musculus trapezius
sometimes totally or partially absent. The ab­
sence can affect a group of muscles, for exam­
ple unilateral partial absence of musculus pec­
toralis, trapezius and sternocleidomasto­
ideus. Other muscles can substitute absent
muscles, but surgical replacement is some­
times necessary for example when the mus­
culus quadriceps is absent. The absence of ab­
dominal muscles can cause a disturbance of
the respiration and spinal deformities.
16.4.2. Muscular dystrophy
The muscu lar dystrophies are a group of un­
common, genetical ly determined primary
myopathies characterized by progressive
muscle wasting and weakening, and by
pathologic changes in the muscles.
Different types of muscular dystrophies
are distinguished on the clinical picture, the
1 24 � 16. Neuromuscular d iseases
progression of the disorder, and the nature o f
inheritance. The most important type is the
Duchenne muscular dystrophy (dystrophia
musculorum progressiva, Duchenne disease).
Progressive muscular atrophy usually
starts before the age of 3 years. It appears in
boys, with sex-linked recessive inheritance.
The inheritance is autosomal dominant in
1 0% of the cases. Genetic research into this
disorder has yielded impressive results, but
even these can barely be utilized in the treat­
ment of muscular dystrophies.
Major clinical findings. The patient
walks with the lower extremities widely sepa­
rated with lumbar hyperiordosis, on tiptoe;
climbing stairs is difficult. The patient has to
lean on the knees to straighten up from a for­
ward bending position. The disease starts
slowly, often at the age of two, and shows
continuous progression. The progression can
appear to stop for half or one year, but unfor­
tunately this is followed by a sudden worsen-
Fig. 16.10.
Typical standing upright from forward bending posi­
tion in a muscu lar dystrophy patient. The pseudo­
hypertrophy of the calf muscles is clearly observable.
ing. The first symptom appears in the posture
of the child. The abdomen becomes promi­
nent, and the lumbar lordosis increases. Later
an equinus contracture develops resulting in
the child walking in tiptoe and weakness of
the shoulder muscles also appears (Fig.
1 6 . 1 0.).
Mainly the proximal extremity muscles
and the lower extremities tend to be involved
during the initial phase of the disease. Weak­
ness of the musculus gluteus maxim us and
musculus quadriceps femoris usually appears
first, followed by the extensor muscles of the
foot.
In the upper extremities, the musculus
serratus anterior, musculus pectoralis major
and musculus latissimus dorsi shows weak­
ness first, followed by the elbow flexors.
Frequent symptom is pseudohypertrophy
of the calf muscles. The quantity of intramus­
cular fat and fibrotic tissue in the musculus tri­
ceps surae increases and the mass of the calf
looks larger, but the active muscle mass in
fact becomes smaller. With the progress of fi­
brosis an equinus contracture develops.
Rarely a same phenomenon can occur in the
musculus quadriceps femoris and musculus
gluteus maximus, with the development of ex­
tension knee and hip contracture.
Between the ages of ten and fifteen years,
the patients usually lose the ability to walk
and have to use a wheelchair. Flexion elbow,
knee and hip contractures develop because of
the long-lasting fixed position in the wheel­
chair. When the sole is not supported by cor­
rective orthosis, an equinovarus foot defor­
mity develops. Scoliosis can appear because
of the weakness ofthe spinal muscles. The pa­
tient is later confined to bed because of the
further deterioration in the general health sta­
tus. No muscle activity is observed at all, ex­
cept for the muscle function of the face, swal­
lowing and breathing. In the final stage, the
disease extends to the intercostal, facial and
heart muscles.
In certain patients level of intelligence de­
teriorates, before the serious physical status
develops. It is easy to diagnose the disease
from the typical clinical picture.

The patients usually die from cardiac
decompensation and lung infection around
the age of 20 years .
During the very early stage of the disease
the measurement of serum creatine kinase
(CK) level can help in the diagnosis. The level
of CK is greatly elevated, even before the
clinical symptoms appear.
Histological examination of the muscle
shows a typical picture. The histological and
enzyme examinations together give the possi­
bility of an exact differential diagnosis.
16.4.3. Myopathies due to
inflammation, polymyositis
This disease rarely occurs before the sec­
ond or third year of age; it is seen more often
in girls. Skin symptoms, fever and other signs
typical of collagen diseases appear. Two
forms are differentiated :
� In the subacute or chronic form, gradual
weakness develops around the shoulders,
the pelvic region and the trunk without
skin symptoms. Walking, particularly on
stairs becomes difficult similarly as in
dystrophia musculorum progressiva.
� The acute form is characterized by skin
and mucous membrane symptoms . Pain,
rigidity, sensitivity can occur, contractures
develop after a lengthy period. Exa­
cerbations and remissions alternate. The
final diagnosis is based on electro­
myography and histological pictures and
the elevated serum enzyme level.
The administration of corticosteroids
the treatment may lead to success.
16.4.4. Myositis ossificans
progressiva (fibrodysplasia
ossificans progressiva)
Myositis ossificans is a rare prog ressive dis­
ease in which the connective tissue of the
16. Neuromuscu l a r d iseases � 125
muscle, e.g. the fascias, tendons and
ligaments show edema and tend to u ndergo
calcification and ossification.
Other developmental anomalies often co­
exist. The disorder most often starts before the
age of six years without any apparent cause or
after a blunt trauma. Very sensitive lumps ap­
pear on the dorsal region of the hand, the cer­
vical area or the shoulder. The swelling di­
minishes and becomes compact during some
weeks, the pain decreases and calcification
and ossification develops in the lumps . The
disease is often localized in the paravertebral
muscles sometimes causing total rigidity of
the spine. Later, ossified bundles develop in
the abdominal and extremity muscles.
.T herapy. The very prominent bumps,
WhICh protrude the skin, sometimes develop­
ing skin necrosis, can be surgically resected.
Bisphosphonate (disodium etidronate) ad­
ministration is suggested which can decrease
the development of calcium phosphate crys­
tals in certain cases effectively.
16.4.5 Arthrogryposis
(arthrogryposis m u ltiplex
congenital
Arthrogryposis m U ltiplex congenita comprises
a heterogeneous group of d isorders charac­
terized by m u ltiple congenital joint
contractures without developmental anoma­
lies of the skeletal system. The d isorder occurs
in about one in 3000 births. The contou r of
the joints is indistinct because of the underde­
III
velopment or absence of muscles (Fig.
16. 1 1.).
More than 1 50 forms of arthrogryposis are
differentiated depending on the various symp­
toms . The disease is caused mainly by
neurogenic factors, but myogenic, skeletal or
environmental effects can also lead to
arthrogryposis. Autosomal recessive inheri­
tance is observed in 1 0% of the cases.
126 � 1 6 . N e u r o m u scu lar d iseases

laxity are already present in infancy. The typi­

cal symptoms are usually symmetrical in
many of the joints. The increased laxity of the
joints is mainly manifested in the meta­
carpophalangeal joints of the fingers, com­
bined with increased extension of the elbow,
wrist, knee and ankle articulations.
The diagnosis of joint laxity can be veri­
fied if three of the five most typical symptoms
are observed, (mentioned in Fig. 1 6.1 2.).
Luxations and elongation of the ligaments
(mainly of the ankle joint) are often present.
The inheritance of this disease is dominant.

Fig. 16.11.
A child with arthrogryposis. The contour o f the
joints is indistinct, muscles are atrophic, extension
contractu re of elbow, knee and flexion contracture
of wrist and fingers.

The contractures existing at birth result in

a special clinical appearance. Extension el­
bow, flexion wrist, flexion finger and ad­
duction thumb contractures are usually pres­
-
ent.
The hips are in flexion, abduction and ex­
ternal rotation. Extension contracture occurs
in the knees or there is some degree of move­
ment in the 20-30 degrees flexion position. A
clubfoot deformity is often combined with
arthrogryposis, but a caJcaneovalgus defor­
mity can likewise be present.

Therapy. The deformities and cont­

ractures can be corrected surgically; the use­
fulness of the hand has to be improved.
The ability to walk can be restored surgi­
cally by surgeries and the use of walking
orthoses. The arthrogryposis patients other­
wise use their hands surprisingly well.
16.4.6. Congenital laxity
of the joints
This may be observed at any age, but the
increased range of motion of the joints and
Fig. 16.12.
Symptoms of generalized joint laxity according to
Carter and Wilkinson. The thumb touches the sur­
face of the forearm in volar flexion, during
dorsiflexion of the wrist and fingers, the fingers are
parallel to the forearm, the extension of the elbow
and knee is larger than 10 degrees, the dorsiflexion
of the ankle is increased. The diagnosis of joint laxity
is verified when at least three of these signs exist.
 Zo lta n Cse r natony
1 7. Tunnel syndromes
In tunnel syndromes, peripheral nerves or
accompanying blood vessels come under ten­
sion in compartments or tunnels preformed by
bones, muscles or tendons .
Etiopathology. A tunnel syndrome can b e
caused b y the narrowing of a tunnel, chronic
inflammation, trauma or an anatomical abnor­
mality. Cases in which no anatomical alter­
ation can be demonstrated are called sponta­
neous tunnel syndromes.
These syndromes generally involve only
one nerve, i.e. they are mononeuropathies.
Clinical symptoms. The most usual
symptom is intermittent pain, in many cases
with monthly periodicity without other symp­
toms. If a sensory nerve is affected, the pain is
sharp and burning, and is limited to the
innervation area.
Paresthesia, hypesthesia or hyperalgesia
and also vibratory hyposensitization or an al­
tered two-point discrimination may also be
observed.
If a motor nerve is affected, the pain is dull
and strong, and is often located at a joint or
muscle group, with somewhat indeterminate
borders.
Tenderness may develop in the muscles,
with a function loss and subsequently even at­
rophy.
In the event of a mixed nerve injury, the
symptoms can be combined. The reflex arch
may also be impaired.
A detailed case history can be of particular
help.
Additionally, pathological lumps, local
hyperthermia and areas of tenderness must be
searched for by manual palpation. If the in­
jured nerve part is struck, the resulting sharp,
electric shock-like pain that generally radiates
in the distal direction is called the Tinel sign.
The motor function can be examined as
described in connection with the muscular
strength (see Chapter 4), and is assessed on
the conventional five-grade scale.
Of the instrumental examinations, EMG
and ENG are important for demonstration of
the location of the compression. A t this point,
the conduction slows down.
Apart from the distinction from other
types of nervous diseases, the differential di­
agnosis is made difficult by the considerable
number of individual variations.
Treatment. Because of the great variety
of locations and symptoms, the treatment can
only be discussed in principle here.
In the early stages and in mild cases, rest
and the administration of nonsteroidal
anti-inflammatory drugs and vitamin B I are
recommended.
Physiotherapy (massage to relax spastic
muscles, diadynamics to ease pain, and
short-wave therapy to stimulate nerve regen­
eration) can also be performed.
Local steroid infiltration can terminate
stubborn symptoms. If the effect is only tem­
porary, this can serve as a diagnostic test to
guide the operative indication. If the anatomi­
cal alteration is unambiguous, but conserva­
tive treatment is ineffective and the com­
plaints are severe, surgery may be necessary.
The aim of the operation is to pinpoint the
affected area and carry out decompression.
Tunnel syndromes that often occur in or­
thopedics are presented in Table 1 7 . 1 . , and
those most frequently observed in everyday
practice are depicted in Figs 1 7 . 1 .-17.7.
 N
--"

00
17. 1.
Tunnel syndromes
.....
Location Name Tunnel Involved structures Symptoms Differential diagnosis Treatment

a megaapophysis
--"
:--..t
-i
Cervical Cervical rib a. +/- v. subclavia +/- thenar, hypothenar , - Pancoast tumor conservative -+
c
spine surgical (resection of
:::J
or real accessory rib truncus inf. (plexus interosseus paresis - cervical disk prolapse
:::J
brachia lis +/- muscle atrophy, cervical or I. rib,
(l)
Thoracic - spinal chord tumor
scalenotomy)
'"
Outlet Raynaud-syndrome,
Scalenus gap between m. plexus brachialis + - plexus brachia lis
'<
Syndrome pulse reducing
:::J
anterior scalenus anterior - a. subclavia neu ropathy
c..
(TOS) provocation tests
syndrome medius - post trauma conditions
o
(Addson, signe du
3
Costoclavicular gap between clavicle plexus brachial is + plateau) - other upper limb tunnel
(l)
syndrome - I. rib a. + v. subclavia syndromes
dysesthesia '"

Shoulder Hyper - space between the plexus brachialis + paresthesia of costoclavicular syndrome conservative-+
abduction origin of m. pecto- a. + v. subclavia fingers, hand, arm, surgical (pectoralis
syndrome ralis minor - p ro- Raynaud-syndrome minor tenotomy)
cessus coracoideus
Incisura incisura scapulae - n. suprascapu laris painful elevation and - rotator cuff i nj u ry conservative -+
scapulae ligamentum rotation, impaired - tendovaginitis bicipitalis surgical (neurolysis)
syndrome transversum abduction and - polyradiculitis
(K6mar) scapulae external rotation in
shoulder
Arm - Coracobrachial between the m. n. muscu locutaneus weak elbow flexion CS-6 root damage conservative -+
Elbow - syndrome biceps brachii - in supination, lateral surgical
forearm m. brachial is forearm paresthesia
(m. coracobrachialis
is perforated)
Processus atavistic n. medianus elbow - hand pain, elbow trauma surgical (chiseling)
supracondyleus supracondylar paresthesia,
syndrome process opposition of thumb
and flexion of I-Ill
impaired
17. 1.
Tunnel syndromes
Location Name Tunnel Involved structures Symptoms Differential diagnosis Treatment

Cubital tunnel epicondyle med. n. u l naris pain, paresthesia, - accessory m. anconeus conservative �

syndrome humeri - u l na med. hand weakness - nerve damage su rgical

border (two origins without night
of m. flexor carpi increase
ulnaris )
Luxation nervi lack of lig. n. ulnaris painful elbow - epicondylitis med. humeri surgical (n. u l naris
ulnaris epicondylo - flex ion, , u lnar - C spine conditions a nteposition to
olecranicum paresis, atrophy, - cu bital tunnel syndrome cubital fossa)
contracture
Supinator canalis supinatorius ramus prof. n. n. radialis paresis, - n. radialis damage conservative �
tunnel (m. supinator) radialis no sensory changes, surgical (neurolysis)
syndrome full wrist and elbow
extension
Wrist, Carpal tunnel canalis carpi - n. medianus - acute: pain, - polyneuropathy conservative �
hand syndrome ligamentum carpi swelling, - C spine spondylarthrosis surgical (iig. carpi
transversum - chronic: - pronator teres syndrome transversum incision)
paresthesia, thenar
atrophy, Tinel +
-'
:'..I
Guyon tunnel lig. carpi volare - ramus spfc. n. ulnaris IV-V. finger - cubital tunnel syndrome conservative �
-I
c
syndrome transversum - os paresthesia, night surgical (neu rolysis)
increase, Tinel +,
- atrophy musculorum
:::J
pisiforme
Froment- + :::J
spinalis progressiva
ro
os pu bis - membrana n. obturatorius +
'<
Pelvis - Obturator thigh adductor - hernia obturatoria conservative � Vl
a. + w. obturatoriae :::J
c..
hip tunnel obtu ratoria paresis, - ostitis p u bis surgical (neu rolysis)
syndrome
(3
thigh medial side
3
ro
paresthesia
Nervus gluteus hiatus n. gluteus su perior + gluteus medius - L disc hernia conservative � Vl

superior tunnel suprapiriformis a. + v. glutea sup. insufficiency, positive - L spine tumor surgical (neurolysis) ....
syndrome Trendelenburg sign
-'
N
1.0
 w
-'

o
17.1.
---'
Tunnel syndromes
......
Location Name Tunnel Involved structures Symptoms Differential diagnosis Treatment

'-J
-'
Piriformis conservative ---+
-I
hiatus infrapiriformis n. ischiadicus sciatica without low - L disc hernia

c
tunnel back pain, no gluteal surgical (neurolysis)
�
- L spine tumor
�
syndrome atrophy, Lasegue
sign + rt>

'<
VI

�
Ligamentum m. iliacus fascia - n. cutaneus femoris lateral thigh pain, - ilioinguinalis syndrome conservative ---+
a.
inguinale ligamentum lat. burning feeling, - sciatica surgical (neu rolysis)
o
syndrome inguinale fascia paresthesia ....

3
Knee - leg Nervus m. vastus medialis - n. saphenus continuous medial meniscus tear conservative ---+ rt>
VI
saphenus m. adductor longus - knee pain surgical (neurolysis)
syndrome membrana
vastoadductoria -
m. sartorius
Peroneus m. peroneus longus n. peroneus comm. sciatic pain, Lasegue - vertebrogenic causes conservative ---+
tunnel tendinous canal test elicits pain at - piriformis tunnel syndrome surgical (neurolysis)
syndrome fibula head - tibialis anterior syndrome
Foot Anterior tarsal dorsal surface of n. peroneus sharp, burning pain - ankle injury conservative ---+
tunnel hindfoot - profu ndus + a. + v. at 1-11. toes, if motor - hindfoot edema surgical (neurolysis)
syndrome ligamentum dorsalis pedis branch is involved,
cruciatum the painful area is
extended
Morton- surfaces of the n. + a. + v. digitalis sharp pain at Ill-IV. - forefoot osteoarthritis conservative ---+
metatarsalgia metatarsus head - comm. toes, i ncreased by surgical (neurolysis)
ligamentum loading, night pain
metatarsi spfc. +
prof.
 1 7. T u n n e l sy n d r o m e s � 131

�______���_______ 3
-------.30..
0

.. ---- 2
....+-

�+-- 3

Fig. 17.1.
Compression of the n. suprascapularis nerve in a

1: incisura scapu lae, 2: n. suprascapular is, 3: a.

case of incisura scapulae syndrome (K6mar).

suprascapularis, 4: lig. spinoglenoidalis

pronator teres syndrome. 1: m. pronator teres, 2: n.

Compression of the median nerve in a case of

medianus, 3: the humeral origin of the m. pronator

teres
132 � 1 7. T u n n e l sy n d r o m e s

1· ,CjK:/

Fig. 17.3.
Compression of the median nerve i n a case of car­
pal tunnel syndrome, and compression of the u l nar
nerve in a case of Guyon tunnel syndrome. 1: n.
medianus, 2: n. u lnaris, 3: lig. carpi transversu m, 4: a.
ulnaris, 5: a. radialis

;
,' '..7. J::==r:=:=:a\;R'j \ 1
Fig. 17.4.
Compression of the n. cutaneus femoris lateralis in a
case of meralgia paraesthetica.1: n. cutaneus femoris
�:::;��'S':"��:N\l /J I
I C�l
3 lateralis, 2: arcus iliopectineus (lig. iliopectinea)
2

4 \ \/', �- .

Compression of the sciatic nerve in a case of

piriformis tunnel syndrome. 1: m. gluteus medius, 2:
m. gluteus maximus, 3: m. piriformis fascia, 4: n.
ischiadicus
 1 7. T u n n e l sy n d r o m e s � 133

1-t---1!--H-- 1

---++- 4

"0
"
"
"

,,/:/ "--
I-++--Tt-t-- 3
1

\\-+---+--+- 3

2
Compression of the peroneus nerve in a case of an­
terior tarsal tunnel syndrome. 1: n. peroneus
profundus, 2: lateral branch of the n. peroneus
profundus, 3: medial branch of the n. peroneus
Fig. 17.6. profu ndus, 4: retinaculum extenso rum
Compression of the peroneus nerve in a case of
peroneus tunnel syndrome. 1: n. peroneus com­
munis, 2: n. peroneus superficialis, 3: n. peroneus
profundus, 4: nerve compressed by fascia
 Zo ltan Cse r n at o n y

18. Disorders of tendons, tendon sheaths,

bursae, fasciae a nd lig a ments

The vanous elements of the musculo­ treatment of tendon injuries are discussed in
skeletal system are the contractile muscles connection with traumatology.
and the non-contractile other constituents.
The latter may be connected in series or in � Tendinopathies
parallel. Those connected in series are the ten­ The tendinopathies may be divided into a
don ongms and attachments, fascia, number of groups :
aponeurosis, retinaculum, tendon sheaths,
while those connected in parallel are the con­ 1 . Mechanical edematous tendinopathy. The
nective tissue cover of the muscles and their tendon is thickened, looses its white shine,
framework, the epimysium, perimysium and
endomysium, and the ligaments. Common or­
thopedic diseases of these elements are dis­
cussed below.
All anatomic structures are designed for
optimal function: the maximal efficacy is at­
tained with minimal mechanical effort. The
muscle - tendon ensemble acts in complete
harmony. If a structural disturbance or a par­
tial injury occurs, a reflex-like overall func­
tion failure may develop.

18. 1. Disorders of the tendons

a
In view ofthe fact that the tendons are sub­
jected to a heavy mechanical load even in nor­ b
mal use, it is not surprising that they can be in­
volved in disturbances due to multiple micro­
trauma. This is obviously more common in
heavy physical laborers and athletes, and
some tendons are particularly involved, in the
shoulder, knee and heel region (Fig. 18.1).
In certain illnesses, or following long­
lasting immobilization, inflammation, or inju­
ries loose connective tissue may build up in
the tendon making it weaker, fragile. The dis­
eases of the tendons may be divided into vari­
ous groups, affecting of the tendon itself, the
osteo-periosteal attachment or the peri­ Rupture of the Achilles tendon in an occasional ten­
tendineal connective tissue. The onset and nis player. b: I ntraoperative picture
136 � 1 8 . D i s o r d e r s of t e n d o n s, t e n d o n s he a t h s , b u rsae, fa s c i a e a n d l i g a m e n ts
and becomes gray and matte. Histology re­
veals edema of the collagen fibers.
2 . Mechanical edematous tendinopathy. Af­
ter opening of the tendon sheath, the ten­
don is widened with fissures of various
depth and length. Connective tissue scar­
ring and hypervascularization appear in
the tendon sheath.
3 . Mechanical nodular tendinopathy. Hyper­
trophic nodules appear in the tendon, as an
indication of an advanced stage of degen­
eration. Fibrosis may develop in the ten­
don-sheath (e.g. trigger finger, Chapter
27).
4. Mechanical necrotizing tendinopathy.
This is the gravest stage, some fluid secre­
tion may start in the tendon sheath.
Histologically various extent of necrosis
may appear in the tendon and it may be se­
questrated. Usually, there are no inflam­
matory signs.
� Enthesopathies
In the diseases classified here, the patho­
logical state is located in the bony anchorage
of the tendons or ligaments. Five types are dif­
ferentiated:
1 . Inflammation (spondylarthritis ankylo-
poetica, spondy larthropathy)
2. Degenerative (Forestier' s illness).
3. Metabolic (gout, chondrocalcinosis).
4. Toxic and iatrogenic (synthetic vitamin A
abuse, fluoride intoxication).
5 . Mechanical (overload).
18.2. I l lnesses of the paratenon and
the tendon sheath
The tendon sheath has multiple tasks : it
protects the tendon, decreases friction, nour­
ishes, produces synovial fluid and ensures the
blood and lymph supply. The structure and
composition of the joint synovial membrane
and the lining of the tendon-sheath is almost
the same, and their pathologic processes
therefore are very similar.
� Tenosynovitis, peritendinitis, peritendi­
nitis crepitans, tendovaginitis crepitans
Tenosynovitis is inflammation of the in­
ner, synovial layer of the tendon sheath. The
origin may be irritative, mechanical or bacte­
rial.
Irritative tenosynovitis can be induced by
an overload. The synovial layer of the tendon
sheath shows evidence of mild inflammation
and a small amount of exudate is excreted. In
places, where tendon is covered only by the
paratenon instead of by the tendon, such in­
flammation is called peritendinitis.
� Tenosynovitis purulenta, tenosynovitis
tuberculosa
Bacterial infection of the tendon sheath
may take place in acute or chronic form. The
chronic form is usually due to tuberculosis.
� Tendovaginitis
In tendovaginitis, the outer fibrous wall of
the tendon sheath is thickened. The etiology is
unknown, but bacterial infection is not in­
volved. The inflammation is located at the ori­
fice of the tendon sheath (see trigger finger
and de Quervain' s disease) .
18.3. Diseases of the bursae
At positions, where the skin, muscles and
tendons slide over each other or exhibit major
angulations, the synovial bursae ensure the
undisturbed motion and mechanical protec­
tion (e.g. between the heel bone and the Achil­
les tendon, or over the olecranon and the pa­
tella) . Accessory bursae can develop at any
time, e.g. over the medial side of the 1 st meta­
tarsal head in hallux valgus, or over exostoses,
around implants etc.
� Bursitis
1 . Traumatic bursitis. Swelling of the bursa
may develop in consequence of a single
mechanical effect or repeated microtrau­
ma (long-lasting kneeling or elbowing,
e.g. a roofer, paver) . The bursa may con-
 1 8 . D i s o r d e r s of t e n d o n s, t e n d o n s he a t h s , b u r s a e , f a s c i a e a n d l i g a m e n t s
tain blood (trauma, hemophilia) or syno­
vial fluid. It may be triggered by a foreign
body.
2 . Microcrystal bursitis. Urate crystals may
accumulate in gout (Fig. 1 8.2.), while apa­
tite may accumulated as a result of bleed­
ing or chronic bursitis, producing the ra­
diological picture of bursitis calcarea.
3. Infectious bursitis. This condition most of­
ten follows percutaneous infection and
takes place either in the prepatellar or in
the olecranon bursa. It is most frequently
caused by classical pyogenic bacteria
(bursitis purulenta), but it may also due to
a fungal infection.
4. Rheumatoid and reactive bursitis. Bursitis
may be initiated by rheumatoid arthritis,
spondylarthritis ankylopoetica, or Reiter ' s
disease.
Fig. 18.2.
Gout-induced olecranon bursitis
18.4. Illnesses of fascia
• Muscle hernia
Following injury or operations the fascia is
not closed, and the underlying muscle pro-
� 137
trudes into the soft tissues. Apart from the es­
thetic discrepancy, in more severe cases this
may be a source of functional disturbances
and pain.
• Necrotizing fasciitis
This severe condition occurs mainly in
tropical areas and is caused by streptococcus.
It may involve any fascia, causes extensive
soft tissue necrosis and demands surgical in­
tervention. It can often cause a severe disfig­
urement.
18.5. Disorders of ligaments
The j oint ligaments contain tendinous or
compact collagen fiber connective tissue bun­
dles, which in some cases are purely the stron­
ger parts of the fibrotic joint capsule, but they
also may be independent ligaments with a flat
or cylindrical shape, located intra- or ex­
tra-articularly. Their role is to stabilize the ar­
ticulating bones.
• Degeneration, rupture, insufficiency
Various ligaments may degenerate either
due to physiological aging, or as a result of
certain rheumatologic disorders. Intact liga­
ments often undergo rupture following inju­
ries, and degenerated ligaments may be tom
due to a minor trauma. The consequence may
be instability and pathological j oint motion,
inducing early osteoarthritis. The ligaments
may become insufficient either because of
tearing, or due to relative lengthening. An ex­
ample is the insufficient function of the spinal
ligaments following disc degeneration: the
vertebrae come close together, pathologic
vertebral motions appear and the ligament be­
comes subj ected to strain.
 Zo lta n (se r nato ny, J6zsef La katos,

19. Ta mas Mesza ros, Gyu l a Po6 r, M i kl6s Sze n d r6i

Diseases of joints

The bones constituting the skeleton are
connected (juncturae ossium) to each other in
two ways : either adj acent bones are connected
continuously by connective tissue, cartilage or
bone tissue (synarthrosis, syndesmosis,
synchondrosis, synostosis); or there is a nar­
row gap Ooint space) between the bones.
Bones are connected by a membrane consist­
ing of connective tissue, the joint capsule, the
connections being called joints. (articulations,
diarthroses) .
are made up of protein molecules and attached
glucose-aminoglycan chains. Their role in­
volves consolidating water (70% of the carti­
laginous tissue is water). Cartilaginous tissue
is completely avascular and does not contain
nerve endings either. It receives its nourish­
ment partially through the veins surrounding

19. 1. The structure of the joints

Bone endings connected to joints have to
satisfy opposing criteria simultaneously: they
must withstand pressure, but ensure mobility
and stability. The shape of bone endings con­
nected to joints is fundamentally determined
by the subchondral bone. The bone endings
are enclosed in hyaline cartilage. The size of
the articular surface is always proportional to
the range of motion of the joint and is always
larger on one of the bone endings connected to
the joint.
The hyaline cartilage is made up of
chondrocytes and the intercellular matrix pro­
duced by these cells. Chondrocytes are capa­
ble of mitosis only while still in growth, and
during this period minor cartilaginous injuries
may heal . During adulthood, biomechanically
much less valuable fibrous cartilage tissue is
produced in the area of cartilage defects . Car­
tilaginous cells mainly produce type II colla­
gen and proteoglycan. Collagen accounts for
50% of the dry substance ofthe cartilage. Col­
lagen fibers are built up in a systematic fash­
ion: parallel to the surface and close to it, and Structure of hyaline cartilage. 1. lamina splendens, 2.
in deeper segments perpendicular to the i ntercellular matrix, 3. chondrocytes, 4. borderline, S.
subchondral bone (Fig. 19.1). Proteoglycans calcified cartilage, 6. subchondral bone
140 � 1 9 . D i se a s e s of j o i nts
the subchondral bone, but mainly via diffu­
sion from the synovial liquid. Cyclical pres­
sure increases the intake and metabolism of
cartilage cells, but above a certain pressure
level the cartilage cells die.
The hyaline cartilage surrounding the
bone-cortex of the j oint is made up of 4 layers :
1 . Tangential zone (small, round, flat cells
parallel to the surface)
2. Transitional zone (irregular round cells)
3. Radial zone (perpendicular cell position­
ing)
4. Calcification zone (the layer in contact
with the bone)
The surfaces of the joints are comple­
mented by various structures composed of fi­
brous cartilages partially independent of the
bone, increasing the pressure surface area of
or even the stability.
The joint capsule surrounds the j oint to­
gether with all of its structures. It is slim and
loose in joints which have a higher range of
motion, but in parts of the j oint that demon­
strate limited movement it is tighter and has
segments that strengthened so as to become
ligaments. Within the j oint capsule are the
proprioception nerve endings, which are fun­
damental in proprioception.
The cavity of the joint is padded by the
synovial membrane. This is a very simple tis­
sue structure that includes an inner cell layer
made up of synovial cells, with a fibrous layer
rich in blood supply beneath it. It has two
functions : assisting in the mobility between
internal structures, as well as producing
synovial fluid which has similar ingredients to
those of the plasma: it is high in protein and is
responsible for supplying the interior of the
cartilaginous j oint and for lubricating the joint
itself.
19.2. The biomechan ical aspects of
joints
The mobility of joints and to some extent
their stability are determined chiefly by the
shape of the bone endings. Basically there are
6 different shapes, namely spherical (hip), el­
liptical (wrist joint), snail (elbow), cylinder
(radius head), flat (smaller joints of the foot),
and saddle (carpo-metacarpal joint of the
thumb) (Fig. 1 9.2.). The possibilities of
movement and their permanent range, how­
ever, are determined by the soft tissues mak­
ing up the joint and the muscles responsible
for bringing the joint into motion.
Apart from the movement possibilities of
j oints, the positions of the participating bones
relative to each other and the limb axis are of
maj or significance. Both the upper and lower
limbs define anato mic and constructional
axes. The anatomic alignment is determined
by the axis of the diaphysis, while the con­
structional axis is that around which the ex­
tended limb can be rotated.
19.3. Arthritis
Arthritis is the collective term used for in­
flammatory diseases that affect the joints of
the spine and the limbs. The reasons for these
diseases vary: immune deficiencies, infective
agents and pathogenic crystals may all be
causes. Degenerative arthrosis, which is also
often inflammatory, is not included here de­
spite the fact that in the Anglo-American
medical literature it is (incorrectly) referred to
as osteoarthritis.
Nonbacterial arthritis can be divided into
the following main groups: autoimmune loco­
motor diseases, seronegative spondarthritis,
juvenile chronic arthritis (beginning in child­
hood), polymyalgia rheumatica, arthritis re­
lated to infections and arthritis induced by
crystals.
19.3. 1. Autoimmune locomotor
diseases
Among the real auto immune rheumatoid
diseases, rheumatoid arthritis primarily oc­
curs within joints, while the other diseases
listed below often affect organs other than the
j oints.

Fig. 19.2.
Various joints.
19.3. 1 . 1 . Rheumatoid arthritis
The causes of rheumatoid arthritis (RA) are
unknown. It is a chronic progressive a rthritic
disease, with an autoimmune pathomecha­
nism that affects multiple joints. Since the dis­
ease practically destroys the joints it affects, it
is also responsible for causing serious disabil­
ity, often coupled with extra-articular
changes. The serological characteristics in­
clude the positive rheumatoid factor (RF) and
antifilaggrin levels.
Occurrence. The prevalence is between
0.2 and 1 . 5%. The number of RA patients in
Hungary is between 50 - 1 00 thousand. Even
though it may also occur in childhood and in
the elderly, it primarily commences after the
third or fourth decade and mainly affects fe­
males (female - male ratio : 2-3 : 1 ) .
Etiopathogenesis. A maj or role i n the
emergence of the disease is played by a ge­
netic predisposition. The human leukocyte
antigen (HLA) DR4 and DRl loci, and to a
1 9 . D i s e a s e s of j o i nt s � 14 1
a: sadle (carpo-metacarpal
b: spherical (hip)
c: flat (smaller joints of foot)
d: cylinder (atlanto-axial)
e: snail (elbow)
f: elliptical (wrist)
lesser extent the polymorphism of certain
cytokines are related to the disease. Among
the HLA DR4 subtypes, the DRB l * 040 1 ,
DRB 1 * 0404, DRB 1 * 0405 and DRB 1 * 04 1 0
alleles make one more prone to the disease. If
subtypes pertaining to certain amino-acid se­
quences ("shared epitope " ) are present, the
disease may be expected to be more severe.
External factors that are considered to have an
impact on the disease include viruses (e.g.
hepatitis B and C virus, Epstein-Barr virus,
parvovirus) and bacteria (e.g. enteral bacteria,
mycoplasmas ) .
The essence o f t h e disease is t h e T a n d B Iym­
phocytes, resulting of the autoimmune pro­
cess, as well as the macrophage activation,
which leads to an i ncreased production of
proinflammatory cytokines (chiefly TNFa and
interleukin- l), the secretion of specific anti­
gens, pathologic cell adhesion, migration and
angiogenesis, and finally joint pannus forma­
tion, cartilage and bone destruction (Fig.
19.3.).
142 � 1 9 . D i se a s e s of j o i nt s

I L-l
I L-12 --""
/ TNF
I L-l
IU8

I FN-y
I L-2
I L-12

"- r;;l � M Ps. PGE2. etc.

�
..
a utoanti bod ies
"-t. +
inflammati o n , tissue injury
rh e u matoid factor
i m m u n co m p l ex , d i rect ce l l to ce l l contact

The role of activated T and B cells, macro phages and proinflammatory cytokines in initiating the cellular and
humoral immune response i n rheu matoid a rthritis.

Clinical picture. In most cases the disease

commences with symmetric polyarthritis, af­
fecting even the hands. The onset may be
rapid, or the disease may develop slowly as
oligo- or a monarthritis. Apart from the gen­
eral symptoms (fatigue, fever, loss of weight,
nausea), morning stiffness in the smaller
joints of the hand is also typical. During the
evolution of the disease, remissions and exac­
erbations alternate, while the joints are slowly
destroyed and various deformities appear, to­
gether with the consequent changes in the soft
changes in the wrist joint and hand in
tissues. In the most frequently affected hand
rheumatoid arthritis.
j oints, ulnar deviation and interosseous atro­
phy develop, while the fingers assume a
swan-neck shape or buttonhole deformity any j oint, even the temporomandibular or thy­
and, due to the destruction of the ligaments in roid joints, but the back, the lumbar spine and
the wrist joints, the ulnar head becomes dor­ the distal interphalangeal joints usually re­
sally dislocated (Fig. 1 9.4.). The shoulders main unaffected.
exhibit a painful restriction, while the elbow The disease often produces extraarticular
and knee joints often show signs of flexion manifestations as well. Subcutaneous rheu­
contracture. A Baker cyst may develop in the matoid nodules appearing mainly in the
popliteal fossa and its rupture may resemble extensor sides are indicative of a poor progno­
the symptoms of deep vein thrombosis . sis. Scleritis, episcleritis and secondary
Chronic inflammation of the metatarso­ Sjogren syndrome may occur. Pleuritis,
phalangeal joints in the leg leads to sub­ bronchiolitis and fibrosis may appear in the
luxation. Very rarely atlantoaxial subluxation lungs, the latter possibly due to the frequently
develops, which may lead to grave and even applied methotrexate therapy. In case of
fatal consequences. The disease may affect chronic and high inflammatory activity,

amyloidosis may occur, which primarily af­
fects the kidneys. Necrotizing vasculitis of
small and medium veins is a rare, but severe
manifestation may lead to limb gangrene. Os­
teoporosis can take place, due to the basic dis­
ease and the frequent and lasting cortico­
steroid treatment. Cardiovascular complica­
tions significantly increase the mortality rate,
as direct (e.g. coronary vasculitis) or indirect
consequences of the basic disease. Due to
immuno-suppressive treatment and long last­
ing steroid administration, various infections
are common and relatively serious. The life
expectancy for individuals with RA is 5 to 1 0
years less than for their healthy contemporar­
ies. The Felty syndrome is an unusual and rare
form of RA and also includes the following:
splenomegaly, hypersplenia and consequent
leucopenia and thrombocytopenia.
Diagnosis. Apart from the general inflam­
mation symptoms (increased We, CRP) labo­
ratory signs of RA include mild anemia and in
80% of the cases rheumatoid factor positivity.
The seronegative form is rarer and has better
prospects. The presence of antifilaggrin anti­
bodies (filaggrin, or cyclical citrullinated
antipeptide) demonstrates high (above 95%)
specificity in RA. The disease may be diag­
nosed if at least four of the following seven
symptoms are present; the first four must per­
sist for at least six weeks (criteria set up by the
American College of Rheumatology) :
� Morning stiffness for more than an hou r
� Inflammation o f three or more joint regions
� Inflammation of the hand joints in at least
one region
� Symmetric arthritis
� Presence of rheu matoid nodes
� Radiological alterations Ouxtaarticular
porosis, marginal erosion)
� Presence of rheu matoid factors in the
serum
Therapy. If treated early, if the treatment
starts 4-8 months after the symptoms first ap­
pear,- and adequately (aggressively), i.e. the
1 9 . D i s e a s e s of j o i n t s � 143
method applied is a disease-altering basis
therapeutic treatment, the destruction ofjoints
can be significantly reduced or in some cases
even halted. For such medication, metho­
trexate is the primary choice (in a dosage of
7,5-25 mg/week), which is well tolerable and
has relatively few side-effects. However, due
to its potentially dangerous hepatotoxicity
and the damage it causes to the blood produc­
ing system, regular clinical and laboratory
monitoring is required. Lejlunomid is a simi­
lar frontline medication. The following too
are used in some cases as base-therapeutic
medication, often in combination: hydroxy­
choloroquine, suJfasalazin, azathioprin and
cyclosporine-A . If therapy refraction or seri­
ous disease progression should occur, biologi­
cal therapy (primarily TNFa-blockers) is an
option which is very effective, though rather
expensive. Additionally, local (intra-articu­
lar) or systemic corticosteroid administration
is often required; non-steroid anti- inflamma­
tory drugs (NSAID) are often not enough to
relieve the symptoms. In order to prevent
bone loss due to the effects of the basic illness
and lasting steroid treatment, the administra­
tion of calcium and vitamin D3 is necessary,
while for therapeutic purposes, the adminis­
tration ofbisphosphonates is advised. Regular
physiotherapy and related exercises, and the
application of hand orthoses and various ap­
pliances can greatly reduce the progression of
the disease and contribute to the preservation
of the patient' s health. If conservative treat­
ment does not lead to an improvement and the
progressive j oint alterations persist, surgery is
advised. All patients with rheumatoid arthritis
require treatment in which all the involved
parties should act together as a team: the fam­
ily doctor, the rheumatologist, the physiother­
apist, the psychotherapist, the orthopedic sur­
geon, the patient and the family should all be
involved in the long therapy, sometimes last­
ing a decade. In order to diagnose the disease
at an early stage and to administer adequate
treatment, an arthritis center was established
in Hungary in 2004.
144 � 1 9 . D i s e a s e s of j o i nts
19.3. 1.2. Polysystemic a utoimm u ne
diseases affecting the locomotor
organs
These include the Sjogren syndrome,
dermatomyositis/polymyositis, progressive
systemic sclerosis, mixed connective- tissue
disease and systemic lupus erythematosus
(SLE) which will be discussed in detail below.
� Systemic lupus erythematosus (SLE)
This is an autoimmune d isease that affect
women of rep roductive age. It attacks several
organs and its causes are unknown. It may
a lso occur but much more rarely in childhood
and old age and in males (female : male =
10 : 1) The average p revalence is 0. 1-0.5%.
The essence of the pathogenesis is the cel­
lular defect, the pathologic antibody and the
immune complex secretion and deposition
observed in genetically prone individuals fol­
lowing a T and B cell function disturbance, in­
ducing severe destruction of the organs and
emergence of an extraordinarily colorful syn­
drome.
Skin and mucosa symptoms (sensitivity to
light, butterfly erythema, urticaria, vasculitis,
discoid lesion, oral ulcers, alopecia, reticular
livedo, and Raynaud 's syndrome) are com­
mon. The joints are also often affected, mostly
in the form of symmetric non-erosive poly­
arthritis. Besides recurrent serositis (pleuritis,
pericarditis), parenchymal alterations, pneu­
monitis and myocarditis may also occur. The
neuropsychiatric symptoms can vary and may
include psycho-organic syndrome, psychosis,
epileptic seizure, central or peripheral neurop­
athy, lupus headache, mononeuritis multi­
plex. The kidney is generally affected in vari­
ous clinical forms and to different extents, the
degree of this significantly influencing the
prognosis of the disease.
Of all the autoantibodies typical of SLE,
the antinuclear antibodies, the antibodies tar­
geting the DNA double helix, the anti­
phospholipid antibodies and the anti-Srn anti­
bodies are worth noting because of their
diagnostical significance. Further laboratory
discrepancies are leucopenia, anemia and
thrombopenia, a low complement level and
non- specific inflammatory parameters.
The treatment of SLE is determined by the
degree of inflammatory activity, the extent to
which the organs are affected, and by other
coexisting diseases. The therapy is individual
and is based on the employment of immuno­
suppressive agents, corticosteroids and non­
steroid anti-inflammatory drugs.
19.3.2. Seronegative spondarthritis
A common feature of seronegative spond­
arthritis (SNSA) also known as spondyl­
arthropathy is the arthritic transformation of
the spinal and sacroiliac joints, which from a
serological perspective means the lack of
rheumatoid factor. Another common feature
of the group is the solid or loose association
with HLA (human leukocyte antigen) B27.
Among these clinical entities spondylitis
ankylopoetica is considered to be the most
significant due to its frequency and severity.
19.3.2. 1. Spondylitis a n kylopoetica
(an kylosing spondylitis)
Spondylitis ankylopoetica (SPA), also known
as ankylosing spondylitis (AS) is a chronic and
progressive inflammation of the sacroiliac
joints and the spine, which is usual ly associ­
ated with calcification of the ligaments, and fi­
nal ly the whole spine stiffens.
Occurrence. The prevalence is around
2% and there are around 20 thousand patients
with this disease in Hungary; it is approxi­
mately seven times more frequent among
males than among females. It usually appears
in early adulthood but it has been known to
occur in childhood.
Etiopathogenesis. The clinical picture
was independently described by Bechterew
( 1 892), Striimpel ( 1 897) and Marie ( 1 898).
It has two types:

- Bechterew 's variant in which the
alteration is concentrated in the spine;
- Striimpel-Marie 's variant, in which
apart from the spine, the peripheral
joints also participate in the process.
There are also two groups depending on
the direction in which it spreads :
1 . the ascending variety: This starts off from
the sacroiliac j oint and the lumbar vertebra
and progresses toward the cranial parts,
sometimes incorporating the hips and the
knees.
2. the descending variety: This starts off at
the cervical spine toward the caudal seg­
ments, usually it does not affect the large
joints.
More than 90% of the patients are HLA
B27-positive, however the role of this in the
pathogenesis has not yet been established. Be­
sides the genetic association, it is assumed
that infective agents (such as enteral bacteria)
also play a part in the development of the dis­
ease. The role of TNFa in the development of
SPA has been confirmed by successful bio­
logical therapy. The major feature of the
pathomechanism is the synovitis coupled with
proliferation and hyperplasia primarily in the
fibrocartilaginous gaps, the intervertebral
discs, the symphysis, the sternoclavicular
joint and the sacroiliac j oint.
As the disease progresses, the erosion of
the cartilage of the joint and the destruction of
the bone beneath it , lead to the development
of fibrous and finally bony ankylosis. One of
the pathological characteristics is enthesitis.
The inflammation ofligaments, tendons origi­
nating from or attached to bones heals with
bony apposition ( "hairy " sciatic bone or
calcaneus ).
Clinical picture and diagnostics. This
disease usually occurs after the age of 20, but
it has been known to affect youngsters and the
elderly as well. Its development is rather in­
sidious. 1t begins with symptoms and ailments
that are difficult to obj ectify for a long period.
1 9 . D i s e a s e s of j o i n t s � 145
Fig. 19.5.
Lateral narrowing o f dorsolumbar spinal movements
in ankylosing spondylitis.
Years can go by between the first symptom
and the correct diagnosis. SPA usually starts
with pain in the lumbosacral spine, in some
cases in the hip or heel. The pain escalates
during the night, but usually subsides in re­
sponse to movement. Thoracic tightness, with
the sensation of wearing armor, may also oc­
cur. Among the general symptoms, sub­
febrility, loss of appetite and weight, and fa­
tigue are most common. Besides the charac­
teristic symptoms, an increase in the sedimen­
tation rate of the red blood cells helps to dis­
tinguish this condition from banal lumbar spi­
nal pains. The diagnosis is based upon the
New York criteria: painful immobility in the
dorsolumbar spine in all three directions (pri­
marily lateral) (Fig. 1 9.5.), a decrease in chest
expansion while breathing in, and the radio­
logical signs of sacroileitis. The sacroileitis is
usually bilateral and is coupled with special
radiological alterations in the spine
(syndesmophyte formation, cube vertebrae,
spondylodiscitis, facet joint arthritis, etc).
Signs of ligament calcification also appear,
which are so typical. Calcification of the
146 � 1 9 . D i s e a s e s of j o i n t s

ligamentum longitudinale anterius and

posterius appear in the X-ray image in the
form of syndesmophytes. The complete de­
velopment of this is what leads to a "bam­
boo-like" spinal transformation (Fig. 1 9.6.).
Additionally, enthesitis is often present and in
one third of the cases the peripheral joints,
also show signs of the disease.
The extra-articular symptoms include
iritis, aortitis, pericarditis, lung fibrosis, rarely
neurological symptoms; in cases of prolonged
inflammatory activity amyloidosis may also
develop. Of the laboratory findings RF
negativity, and HLA B27 positivity are typi­
cal, the latter is used in differential diagnostic
problems . High inflammatory parameters are
frequent, but these can be negative in the ac­
tive phase as well. In such cases the clinical
signs and a specific questionnaire filled out by
the patient (BASDAI index) help determine
the severity of the activity.
As the costovertebral joint may be affected
at an early stage, the chest expansion during
inspiration is significantly limited. By the fi­
nal stage only abdominal breathing may be
observed. The breathing expansion may be
limited to 1 -2 cm at the level of the nipples,
which is an early sign and can lead to estab­
lishment of the correct diagnosis. With the
gradual limitation of the spinal mobility and
with spinal stiffening the extent of dorsal
kyphosis also intensifies.
Because of the curve of the spine, and the
occasional hip j oint involvement, with flexion
contracture, the patient is able to look forward
only below the horizon. To look forward, the
patient bends his/her knee j oints. It can occur, Fig. 19.6.
that the spine of the patient is deformed to a: Syndesmophyte development, " bamboo " shaped
such an extent that the patient looks back­ spine, and
wards from between the legs (Fig. 1 9.7.). b: sacroiliac joint synostosis in advanced ankylosing
Patients with SPA can only lean to the side spondylitis.
if the opposite limb is lifted from the ground
and abducted. The patient' s condition is inten­
sified if the hip is affected, especially if posture even in cases of complete spinal stiff­
ankylosis develops in the hip joints in flexion. ness are essential; of course, the patient must
also cooperate fully to succeed. Non-steroid
Therapy. The most important part of the anti-inflammatory drugs are important, not
therapy is regular and proficient physiother­ only as symptomatic drugs, but also to exert a
apy. Teaching and the attainment of a correct positive influence on the outcome. If the pe-

Fig. 19.7.
Stiffened Bechterew's spine in incorrect posture,
leading to radical reduction of field of vision
ripheral joints are affected, basic therapy
(methotrexate or sulfasalazin) may also be
beneficial . Lately, TNFa blockers have been
used in cases where SPA was not responding
to drug treatment and have achieved excellent
results. If arthrosis is affecting the larger
joints, a hip arthroplasty is necessary (see
later) .
SPA seriously impairs the quality of life.
Active and extra-articular forms are espe­
cially difficult to treat and the related mortal­
ity rates are close to those for RA.
19.3.2.2. Other forms of seronegative
spondarthritis (SNSA)
In the following section the clinical pic­
tures for this group as well as SPA will be
1 9 . D i s e a s e s of j o i nt s � 147
briefly reviewed. A common feature i n con­
trast to SPA is that the sacroiliac joint is usu­
ally affected on one side and there is
paravertebral ossification of the ligaments
(development of parasyndesmophytes) on the
spine. The clinical picture indicates a milder
course than in SPA.
� Arthritis psoriatica
1 0-20% of the cases of psoriasis are usu­
ally combined with arthritis.
This can lead to :
1 . most commonly to asymmetric oligo- or
monarthritis and sausage fingers,
2 . distal interphalangeal j oint involvement,
3 . mutilation of fingers,
4. symmetric RA-like polyarthritis and
5 . spinal involvement resembling SPA.
Treatment involves basic therapeutic
drugs (mainly methotrexate, leflunomid and
more recently biological drugs) and non­
steroidal, or if needed, steroidal anti-in­
flammatories. Skin alterations are treated si­
multaneously.
� Reactive arthritis
Urogenital or enteral infections followed
by sterile arthritis are generally categorized as
belonging to SNSA group. Arthrites follow­
ing urethritis are categorized as SARA (sexu­
ally acquired reactive arthritis). Reactive ar­
thritis caused by gonococcus, similarly to the
gonorrhea infection process, is nowadays
comparatively rare. No causative agents can
be identified in the j oint. Forms relating to
urogenital infections can occur at any age, but
are most frequent in early adulthood. Mostly
the lower limb are involved in oligoarthritis
and sacroileitis, sometimes with lumbar spi­
nal pain. Reactive arthritis showing signs of
the clinical triad urethritis, conjunctivitis and
arthritis is termed Reiter disease.
�
Enteropathic arthritis
1 5% of the patients suffering from Crohn
disease or colitis ulcerosa develop arthro­
pathy, primarily in the lower limbs. Non-
148 � 1 9 . D i s e a s e s of j o i n ts

erosive asymmetric oligoarthritis with spinal

and/or sacroiliac involvement often develops
independently of the bowel symptoms.

19.3.3. Juvenile chronic arthritis

Juvenile chronic arthritis (JCA) recently also

termed juvenile idiopathic a rthritis (JIA), is a
chronic a rthritic d isease beginning before the
age of 16 (all other arthritic diseases must be
excluded). It includes j uvenile SNSA's as well
and is a heterogeneous disease group. An ac­
curate diagnosis can be established only dur­
ing later p hases of the disease.

Occurrence. The prevalence of lCA IS

around 1 : 1 000 while the incidence is
1 : 1 0,000.

Etiopathogenesis. Etiology of the dis­

ease, similarly to RA has not yet been firmly
established, and due to its heterogeneous na­
ture it differs as a subcategory too. Immuno­
pathogenetic roles of genetic factors (e.g.
HLA association) and inductive infective
agents are understood to be responsible.

Clinical picture and diagnosis. Accord­ Fig. 19.8.

ing to the initial symptoms, three diagnostic Clinical picture of polyarticular juvenile chronic ar­
subcategories are distinguished: thritis in a boy treated with steroids.

� systemic,
� polyarticular and
� oligoarticular form.
The symptoms within these subcategories
differ considerably, often the only common
factor is chronic arthritis, which interestingly
does not cause significant pain as it does in
adults (Fig. 1 9.8.).
Therapy. The disease can only be influ­
enced appreciably if medication, physiother­
apy and, if needed, orthopedic surgery are ap­
plied concurrently. The multidisciplinary
therapeutic team headed by the pediatri­
cian-rheumatologist must also include the
teacher and the parents. The drug treatment
policy varies according to the subcategory
and the severity of the disease. The first step is
usually non-steroidal anti-rheumatoid and,
following j oint drainage, steroid medications.
If this fails, systemic steroid treatment fol­
lows. In active or progressive cases, basis
therapy (primarily methotrexate, azathioprin,
sulfasalazin, chloroquine, etc.) is recom­
mended. , Excellent results have recently been
achieved with etanercept, a biological sub­
stance inducing TNF blockade. Physiother­
apy and especially exercises are indispensable
when it comes in the treatment in order to pre­
vent j oint deformities, contractures and im­
mobility. The activity of the disease can be
stopped in children suffering with lCA

through professional treatment; the progres­
sion may also be slowed down and, through
the avoidance of severe consequences the
child may become a useful member of the
family and society.
19.3.4. Crystal-induced arthritis
This category includes gout (arthritis
urica) which is a well-defined and perfectly
treatable disease, plus the less significant cal­
cium pyrophosphate dihydrate and hydroxy­
apatite arthropathies.
19.3.4. 1. Gout
Gout is a joint disease caused by the accumu­
lation of monosodium-urate-monohydrate
(M5U) crystals in the tissue. It is often coupled
with extra-articular and visceral involvement.
Hyperuricemia arises, when the uric acid level
in the serum exceeds its solubility, this occurs
in males over 20 IJmol/l, and in females over
360 IJmol/l.
Occurrence. Gout is the most common
type of arthritis to affect overweight men
above the age of 40. Its prevalence among
men is 5 -2 81 1 000, while that among women is
only one-fifth of this. The prevalence of gout
is on the rise worldwide, primarily due to en­
vironmental factors (diuretics, alcohol, obe­
sity etc).
Etiopathogenesis. Purine nucleotides are
components of DNA and RNA molecules and
the end-product of the human purine metabo­
lism is uric acid. The development of the dis­
ease is due to three significant factors: a high
serum uric acid level, the formation of MSU
crystals and the interaction between the crys­
tals and the inflammatory system. Gout is said
to be primary, when the hyperuricemia, ini­
tially responsible for the precipitation of uric
acid is a consequence of a hereditary meta­
bolic disease. Gout is considered secondary, if
the hyperuricemia is a consequence of some
1 9 . D i s e a s e s of j o i n t s � 149
Fig. 19.9.
Typical gout attack in the 1st metatarsophalangeal
joint and the back of the foot.
other factor (e.g. increased cell disintegration
in myeloproliferative diseases, or a decreased
uric acid secretion due to an impaired renal
function) . The pathomechanism of the hyper­
uricemia is related to the overproduction of
uric acid (approximately 1 0%) or more fre­
quently (approximately 90%) to a deficiency
in the transport of tubular urate which leads to
a decreased excretion. The urate crystals ac­
cumulating in the tissue activate the inflam­
matory mediator systems and the effector
molecules thereby released cause acute in­
flammation.
Clinical picture. The clinical appearance
of an acute attack of gout is typical. It usually
occurs in the first metatarsophalangeal joint
(podagra) (Fig. 1 9.9.). Intense pain in the j oint
with a swelling often commences in the early
morning hours, the skin is of purple-red and
there is often fever. Acute gout is frequently
induced by a purine-rich diet, alcohol, surgi­
cal intervention, trauma, medication and is of­
ten accompanied by general symptoms (fever,
elevated sedimentation rate and white blood
cell count). Following a period of acute at-
1 50 � 1 9 . D i se a s e s of j o i n t s
u lcer. a: Clinical and b: radiological picture.
tacks chronic gout gradually develops accom­
panied by soft-tissue and bone tophus, serious
joint destruction and changes in the visceral
organs (Fig. 19.10. a, b). The most common
internal complications are tubular nephro­
pathy gout and uric acid nephrolithiasis. Met­
abolic syndrome (obesity, hyperiipidemia, di­
abetes mellitus, hypertension, arteriosclero­
sis) often accompany gout.
Diagnosis. The absolute criterion for a
laboratory diagnosis is the presence of the
negative bi-refractive needle-shaped MNU
crystals in the synovial fluid. If this is not ob­
served, gout may be suspected in case a of a
joint being affected, tophus, hyperuricemia,
urate nephrolithiasis and remission within 48
hours after the intake of colchicine.
In the X-ray image the bone edges are
usurated, a urate deposit is formed, which is
not absorbed. A circumscribed radiolucent
area is visible, with a sharp edge and smooth
contour, as though created by a punch. Dif­
fuse idiopathic skeletal hyperostosis may de­
velop in the spine and sometimes on the
limbs, as part of the complex metabolic dis­
turbance.
a b
Therapy. It is important to distinguish be­
tween the treatment of an acute gout attack
and a steady decrease of the serum uric acid
level. In cases of secondary gout causative
therapy is possible. Colchicum salicylate is
perfect for the treatment of joint seizures. An
initial dose should be 1 mg followed by 0.5
mg every 2 hours till the seizure abates or di­
arrhea occurs. If colchicine is ineffective,
non-steroidal anti-rheumatic drugs or if
needed, steroid may also be administered. As
part of the non-medical treatment a local cold
pack and a tranquil environment are essential.
If an intense attack persist, a daily dose of
0 . 5 - 1 .0 mg of colchicine salicylate may be ad­
ministered for a longer period. During sei­
zure-free periods, the serum uric acid level
have to decrease below the safe level of 360
!lmol/l. A daily dose of300-600 mg of the uric
acid synthesis inhibitor allopurinol is effec­
tive. Less commonly used uricosuria medica­
tion is applied if the tubular transport of uric
acid is decreased. Non-drug therapy includes
the elimination of environmental riskfactors
such as obesity, a purine rich diet and alcohol
consumption. Medication options for the
treatment of chronic locomotor transforma-

tions are supplemented by mechano-, electro­
and balneotherapeutic methods and, if
needed, by orthopedic interventions.
19.3.4.2. Other forms of crysta l induced
arthritis
� Calcium pyrophosphate dihydrate
(CPPD)-arthropathy
This arthropathy is caused by crystal de­
posits of CPPD in the joint cartilage or in the
periarticular area. The disease has numerous
clinical appearances. The synovial fluid con­
tains non-inflammatory, small, rod-like,
slightly positive, bi-refractive CPPD crystals.
The X-ray picture of the affected joint reveals
linear, pearl-like spottiness (chondro­
calcinosis) or irregular cartilage calcification
(a few calcified nodes) During an acute attack
of pseudo-gout colchicine, non-steroidal
rheumatoid or steroid treatment may be ap­
plied. Rest, a cool compress and evacuation of
the synovial liquid are necessary. The chronic
forms are treated symptomatically with phys­
iotherapy and non-steroidal anti-inflam­
matory agents. It is important to treat the
background disease.
� Hydroxyapatite arthropathy
This involve the peri-articular and intra­
articular deposition of crystals of calcium­
hydroxyapatite. It appears most frequently in
connection with inflammatory calcifying bur­
sitis (e.g. bursitis calcarea subacromialis)
Radiological picture of calcified subacromial bu rsitis.
1 9 . D i s e a s e s of j o i n t s � 151
(Fig. 1 9. 1 1 .). I n extreme cases, it causes de­
position similar to tophus or calcifying gout.
The pathogenic crystals can be seen in the
synovial liquid with an electron microscope.
The treatment is symptomatic, X-ray irradia­
tion is often effective.
19.4. Arthropathies
19.4. 1. Neurogenic arthropathy
Neurogenic arthropathy (neuropathic arthro­
pathy, Charcot joint) is a disorder involving se­
rious articular destruction caused by damage
to the central or peripheral nervous system
leading to the loss of pain sensation deep and
proprioceptive sensation.
Etiopathogenesis. As a result of the sen­
sory neurological disorder, due to loosening
of the overloaded j oint, micro traumas, de­
struction of the cartilage and reactive hyper­
trophic bone transformations occur. Atrophic
bone transformations are more characteristic
in cases of peripheral nerve damage, while
hypertrophic phenomena are primarily attrib­
uted to the central forms. Arthropathy is pri­
marily caused by insulin-dependent diabetes
mellitus and alcoholism-induced polyneuro­
pathy, tabes dorsalis and syringomyelia, but
the latter two nowadays are rather rare.
Clinical picture and diagnosis. Neuro­
genic arthropathy induced by alcoholism and
diabetes mellitus chiefly affects thefoot joints,
while transformations caused by tabes
dorsalis primarily involve the h ip and knee,
and the syringomyelia form develops in the
upper limbs. The process may in some cases
be bilateral and symmetrical . Foot transfor­
mations caused by alcoholism and diabetes
are accompanied by chronic edema, hyper­
emia, trophic skin and nail alterations, pain­
less ulcers on the soles of the feet, foot defor­
mities (see also chapter 30). Chiefly the tarso­
metatarsal and the metatarsophalangeal j oints
are affected, and the joints of the ankle to a
152 � 1 9 . D i s e a s e s of j o i nts
much lesser extent. The central leg and meta­
tarsal heads sink, causing hammer toes and
serious deformations, leading to walking dif­
ficulties. Pain sensation is disturbed, numb­
ness and spasms develop, often coupled with
motor disorders leading to muscle atrophy
and weakening. Crepitation and subluxation
may be detected, articular liquid collection
develops which may occasionally be blood.
There is great inconsistency between the seri­
ous clinical symptoms and the lack of pain
sensation. Precise neurological examination
is vital and must include tests of the sensation
of position and vibration sense as well as re­
cording of electrophysiological parameters .
Radiologically
� Stage I.: osteoporosis, cortical defects and
subluxations.
Stage 11.: osteolysis, fractures, fragmentation
�
and incipient periosteal reactions.
� Stage I ll.: severe hypertrophic arthrotic
phenomena, serious bone defects, and in
certain cases a n kylosis (Fig. 19. 12).
In the differential diagnostics, the distinc­
tion of septic arthritis, osteomyelitis and
osteonecrosis is of primary importance .
Therapy. Treatment of the basic illness,
alcohol prohibition, correct control of the car-
Fig. 19.12.
Neurogenic arthropathy caused by tabes dorsalis in
both hip joints, with significant destruction and bi­
zarre bone formations.
bohydrate metabolism and elimination of the
risk factors responsible for the damage of the
vessels of the nerves (e.g. lipid metabolism
disorder, hypertension, smoking) are all im­
portant. Medication of value for the symptom­
atic treatment includes various B vitamins,
tricyclic antidepressants and anticonvulsive
agents if needed. The limbs must be relieved
from weight-bearing with the help of orthope­
dic insoles and shoes and protected from in­
jury. If the illness has progressed, orthopedic
intervention will be the only effective treat­
ment.
19.4.2. Hemophilic arthropathy
Hemophilic arthropathy is a degenerative ar­
ticular alteration due to repeated intra­
articu lar bleeding. It is mostly due to the lack
of factor VI II. (hemophilia A) or factor IX. (he­
mophilia 8).
The prognosis of the disease depends on
the serum level of the factors : the condition is
critical if the serum concentration level is be­
low 1 % of, intermediate, if it is between
1 -5%, and mild if it is between 5 - 1 5%. A mi­
nor trauma, or no trauma at all, may still ac­
company continuous articular hemorrhage
(hemarthros). As a result the nourishment of
the hyaline cartilage breaks down and chronic
pigmented villonodular synovitis-like inflam­
mation develops on the synovial membrane
which in turn extends to the cartilage (pannus
development) and destroys it. Later cysts de­
velop in the subchondral bone leading to seri­
ous destructive arthrosis at a rather young age
(Fig. 19.13 a-c). Hematological care and fac­
tor supplementation are important. If arthrosis
or articular destruction has already occurred,
arthroplasty has to be taken into consider­
ation. Serious contractures and axial deformi­
ties are contraindicated and it is important to
note that the surgical complications are much
more frequent than in normal cases of old-age
arthroplasty.
 1 9 . Diseases of joi nts � 153

19.5. Performing surgery

i n arthritis
Rheumatoid symptoms chiefly indicate
chronic multisytemic diseases affecting the
cartilage, bone, tendon, muscle and internal
organs. In rheumatoid arthritis, synovial inva-

5%

3%

1%

11%

24%
c

32% ----4

24% -----t
Fig. 19.13.
a: Swollen knee joint of a hemophilic child
b: X-ray image of the seriously destroyed knee joint
Fig 19.14.
Distribution of ailments of 200 patients with some
in the same patient
c: Surgical picture of articular destruction, pannus
development. type of articular disease.
1 54 � 1 9 . D i s e a s e s of j o i nt s

sion destroys the articular surface, while the
rheumatoid pannus damages adj acent tissues.
Bone destruction, erosion, cyst formation and
osteoporosis j eopardizes the skeletal integrity
and, since ligaments and tendons are also af­
fected (destruction, rupture), the result is ar­
ticular instability. The aim of the treatment
concerning patients with rheumatoid arthritis
are to eliminate the pain and improve the j oint
function and hence the quality of life for the
patient.
From a surgical point of view, rheumatoid
patients pose numerous problems (Fig.
19.14):
� If many joints are affected it is difficult to
choose the "target joint".
� In order to rehabilitate the patient, a series
of surgical interventions are often
necessary, cooperation of the patient in
such cases is indispensable.
� As internal organs are affected, pre­
operative preparation and postoperative
care are necessary.
� Rheumatoid patients frequently exhibit
atlanto-axial articular synovitis, sub­
luxation and luxation, which requires
anesthetic vigilance. Insertion of the
intratracheal tube can pose a threat due to
the possible neurological complications .
Examination and if needed, stabilization
of the neck segment of the spine for
patients with RA are therefore essential.
� Steroid treatment and the basic illness
itself can damage various structures.
Accordingly, the most gentle surgical
techniques are required.
� Because ofthe skin changes that occur in a
rheumatoid patient, wound healing is
often a problem.
� Because of the immunodepressed state,
rheumatoid patients are more vulnerable
as concern septic complications.
� For patients who have received steroid
treatment, surgery is performed with an
elevated amount of medication and the
initial amount is gradually restored during
the post operative phase.
� The preparation of the patient, defining the
indication, forming the strategy, the post­
operative therapy and the long-term
treatment is largely based on the close
cooperation of the surgeon and the
rheumatologist.
From a surgical aspect, the disease has an
early, an intermediate and a late stage, corre­
sponding to the Steinbrocker stage classifica­
tion (Table 1 9. 1 .) .
Surgical procedures can b e classified to
operations performed on soft tissues, bones
and joints .

Table 19.1.
Surgical interventions according to the stages of rheumatoid diseases
Surgical stages Early Intermediate Late
Main symptoms I and (11) (11) and Ill. IV.
(Steinbrocker stages) No radiological destruction, Various degree of Rbrous or bony ankylosis,
osteoporosis may occur osteoporosis, cartilage and significant deformities,
locally bone destruction, extra-articular soft tissue
deformities, subluxation, alterations
restricted mobility
Nature of surgery Preventive - curative Plastic - reconstructive Salvage
Type of surgery Synovectomies Ligament reconstruction, Resection, arthroplasty,
tenosynovectomies tendon transpositions, arthrodesis
tendon reconstructions
i nterpositional arthroplasty,
prosthesis implantation
 1 9 . D i se a s e s of j o i n t s � 155

� Surgery on soft tissues:

synovectomies
tenosynovectomies
ligament transpositions
ligament reconstructions
� Surgical procedu res on bones and joints:
arthrodesis
osteotomies
resection arthroplasty
interpositional arthroplasty
alloarthroplasty (endoprosthesis im­
plantation)
a

b
As regards to local preventative/curative
interventions in rheumatoid arthritis, the main
mean intervention is early synovectomy to
eradicate the synovial invasion; this solves the
ongoing complaints of the patient, and at the
same time prevents or hinders the progression
of the process in the specific joint.

Hand surgery. When synovitis that has

been present for longer than 6 months and has
not responded to conservative treatment af­
fects the metacarpophalangeal (MCP) joint,
early stage synovectomy can help prevent the
tri-articular system from reaching the col­
lapsed deformity stage (Fig. 1 9 . 1 5) .
Correction ofthe typical ulnar deviation o f
the fingers is achieved by centralization of the
middle extensor tendon and its fixation at the
level of the MCP j oint. If the "button hole" or
"swan neck" deformities have already devel­
oped, surgery is possible only if it is supple-
Fig. 19. 16.
a : Serious ulnar deviation of fingers o n a rheumatoid
hand
b: Arthroplasty of I I-V. MCP joints with silicon pros­
thesis implantation: the correction of the axis of the
MCP joints can be clearly seen.

mented with ligament-plasty and tendon­

Fig. 19.15.
Synovitis of the metacarpophalangeal joint and mild
palmar subluxation of the fingers.
plasty. In order to restore the function of the
hand arthroplasty has to be performed in the
metacarpophalangeal articular line with the
insertion of silicone prostheses (Fig. 1 9 . 1 6. a,
b) This is also possible with the proximal
interphalangeal (PIP) joints but arthrodesis in
156 � 1 9 . D i se a s e s of j o i nts
a functional position may also be performed.
Stability of the distal interphalangeal (DIP)
j oint in a pathologic position can be restored
by performing arthrodesis.
Wrist j oint surgery. Dorsal tenosynovitis
requires synovectomy, whereas palmaris
tenosynovitis causing median nerve compres­
sion requires tenosynovectomy and decom­
pression of the median nerve by incision of
the transverse carpal ligament.
The invasive synovial tissue destroys the
tendons, if bone destruction occurs, the un­
even, protruding end of the ulnar bone (Lister
tuberculum, or ulnar head syndrome) may in­
duce extensor tendon ruptures . It is extremely
difficult to restore the continuity of the ten­
dons . When the tendons are fibrillated and de­
stroyed, uniting the ends is practically impos­
sible. Reconstruction can be performed only if
the distal ends of the tendons are attached to
the remaining mobile motor. This can be done
with the tendon of the m. indicis proprius but
also by suspending the distal parts of the de­
tached tendons IV. and V. to the remaining
Ill. and 11. tendons in the shape of a
roller-blind. If the rupture was caused by the
ulnar head syndrome by the protruding ulnar
end (piano key sign), ulnar head resection has
to be performed. This involves retinaculum
plasty and repositioning of the m. extensor
carpi ulnaris. In case of ulnar head syndrome,
ulnar head silicone prosthesis implantation
surgery is also an option.
Arthrodesis and wrist prosthesis implants
are the key procedures when it comes to han­
dling wrist joint problems . If the wrist is af­
fected by pain, destruction or subluxation, re­
section of the destroyed surfaces, radiocarpal
arthrodesis may be performed in a slight dor­
sal flexion and midposition. This is a favor­
able setting considering the function of the
hand as the flexor, and the extensor muscles
can therefore function optimally.
If the patient does not engage in laborious
physical work and does not have to exert large
forces, and ifthere is a significant destruction,
complete wrist joint silicone prosthesis im­
plantation may be performed, but it must be
borne in mind that this is a compromise be­
tween stability and mobility.
Elbow j oint surgery. Synovectomy can
be performed with radius head resection. In
these cases, synovectomy is possible more ex­
tensively. However, some authors restrain
from removing the radius head as it may cause
instability.
In the event of serious destruction, resec­
tion arthroplasty was earlier an accepted sur­
gical method which eliminated the pain, but it
resulted in a limited functional outcome and
in instability.
If the degree of resection is minimal,
interpositum can be used to cover resection
surfaces. (e.g. fascia, dura mater). Some au­
thors report success, but the long-term func­
tional results are uncertain and instability and
subluxation often occur.
Arthrodesis used to be a standard surgical
intervention, but today (especially if the prob­
lem is bilateral) it is avoided. Development in
recent decades have had the aim of compen­
sating for the deficiencies of the firs­
generation wrist joint prostheses.
Erosive destruction, a narrowed joint and
significant pain are all indicatory.
In summary, synovectomy and arthro­
plasty are currently the most accepted meth­
ods for the treatment elbow deformations in
RA.
Shoulder surgery. Surgery on rheuma­
toid shoulders also include various proce­
dures similar to those mentioned above. In
rheumatoid arthritis extended inflammation
of the subacromial bursa and the subdeltoid
bursa, destruction and rupture of the rotator
cuff and glenohumeral synovitis we are often
observed. These conditions can be treated by
arthroscopic synovectomy or open surgery.
Reconstruction of the rotator cuff and
acromioplasty can be performed similarly us­
ing both methods.
In cases of major articular destruction,
glenohumeral arthrodesis is a possibility, but
from a functional point of view the most suit­
able procedure is shoulder joint endo­
prosthesis implantation.

Hip surgery. Synovectomy has not be­
come very widespread in treating h ip joint
transformations due to the special anatomic
factors and the unique hip processes. The hip
is the joint that is subjected most frequently to
prosthetic procedures.
Knee surgery. Arthroscopic or open sur­
gical synovectomy of the knee may delay the
destruction of the cartilage for quite some
time. If destruction should occur, arthrodesis
is performed much less frequently nowadays.
From a functional aspect, the best solution lies
in the implantation of a total surface replacing
endoprosthesis.
Following a certain progression of the dis­
ease, the foot is also damaged in 80-90% of
the cases.
Foot surgery. The hindfoot is involved in
50-70% of advanced cases of the disease, and
in 70-80% of the cases the forefoot. If the tar­
sus is involved, synovectomy and subtalar and
Chopart arthrodesis may be necessary.
Apart from the generally known proce­
dures mentioned above, complex rheumatoid
forefoot deformities may be treated by resec­
tion of the metatarsus heads according to
Clayton-Hibinette or by subcapital metarsal
osteotomies according to Helal.
Ankle and hallux j oint endoprostheses are
not very popular. Arthrodesis may be per­
formed only if strict certain individual criteria
are strictly satisfied, since, besides achieve-
b a
Fig. 19.17.
Picture of open wedge osteotomy.
1 9 . D i se a s e s of j o i n t s � 157
ment o f stability, the ability to bear weight
and painlessness, the articular mobility is lost.
This leads to walking difficulties, which in
turn means a limited environment, as well as
overloading and acceleration of the degenera­
tive processes of the neighboring joints.
Surgical treatment of spondylarthritis
ankylopoetica (SPA). From a surgical as­
pect, the treatment of SPA poses a special
problem. In about one-third of the cases
arthrosis develops in the hip (usually bilater�
ally) which ends up as a fibrous or bony
ankylosis. If the hip is in a flexion position,
this further worsens the limited ability to look
forward, caused by the kyphosis.
Conversion arthroplasty may be per­
formed by implanting a hip endoprosthesis. In
such cases the flexion contracture of the hip
joint resolves, extension becomes possible
and the straightening of the torso increases the
distance of forward vision.
In certain cases, it may be necessary to
consider bilateral hip arthroplasty in one ses­
sion. An applicable surgical procedure in
cases of a maj or degree of kyphosis is spinal
osteotomy. All procedures have the aim of
wedge resection of the vertebral arches of the
involved spinal segment CL 2-3 -4), exposure
and preparation of the dura and the roots, then
reclination and stabilization of the spine (Fig.
1 9 . 1 7 and 1 9.8).
b
Fig. 19.18.
Picture of closed wedge osteotomy.
158 � 1 9 . D i se a s e s of j o i n t s
19.6. S}'novial chondromatosis
Synovial chondromatosis is a rare tumor-like
transformation of the synovial membrane
which usually occurs between the 2nd and
5th decade of l ife and is typically accom pa­
nied by cou ntless cartilaginous loose bodies
p roduced by the synovial membrane.
Etiopathology. Initially microscopic
metaplastic cartilage tissu begin to accumu­
late in the synovial membrane, later these
islands grow to such an extent that clusters be­
come visible to the naked eye and are finally
detached into the joint and behave as free bod­
ies. In the later phase of the disease, the ex­
panded articular cavity may contain many
hundreds of cartilaginous free bodies, mea­
suring 0 . 5-2 cm.
Clinical symptoms. The knee, hip, elbow
and shoulder joints and the temporo­
mandibular j oint are most often affected. The
j oint becomes swollen, the articular mobility
is reduced and the patient complains of articu­
lar blocks and clicking.
X-ray symptoms. Initially, the cartilagi­
nous bodies are not visible in the X-ray pic­
tures. They become visible only later when
they become calcified (Fig. 1 9 . 1 9.). The MR
Fig. 19.19.
Widened elbow capsule surrounding numerous ar­
ticu lar free bodies with typical calcification
and eT images display typical intra-articular
free bodies.
Treatment. The surgical solution is expo­
sure of the joint, the removal of cartilaginous
free bodies from the joint and subtotal
synovectomy to prevent further development
of the cartilaginous bodies. Very rarely, ma­
lignant transformations can occur in the carti­
laginous substance. In such cases, the process
accelerates and becomes destructive.
19.7. Pigmented villonodu lar
synovitis (PVNS)
This disease i nvolves alterations in the
synovial membrane in the joints, bursae and
tendon sheaths and is categorized today as a
benign tumor rather than an inflammatory
disease. The proliferative tissue collection re­
sults in swelling of the joint, expansion of the
capsule, but it may destroy the bone too.
Etiopathology. It is quite a rare condition.
There are two forms : nodular and diffuse, also
known as benign synovioma and primarily
starts from the tendon sheaths of the fingers.
From a histological aspect, both involve the
proliferation of the cells lining the synovial
membrane and the mesenchymal elements .
Macroscopically, the synovial membrane
padding the joint becomes thickened and the
synovial villi, which are discolored brown­
ish-red from the haemosiderin also thicken to
resemble the fingers of a glove.
Clinical symptoms. It can occur at any
age but is most frequent in young adults. It
primarily affects the knee, the hip and the
shoulder. The major symptoms include swell­
ing of the joints, pain, immobility, and in 75%
of the cases intraarticular bleeding and
hemarthros also develop.
X-ray signs. Initially, there are no charac­
teristic X-ray alterations. In the later phase of
the disease cystic defects may develop in the
 1 9 . D i s e a s e s of j o i n t s � 1 59

bones making the joint in question (Fig. 19.8. Arthrosis (osteoarthritis)

1 9.20. a-c) . An MRI demonstrates a charac­
teristic change in signal intensity due to the
tissue proliferation and its increased haemo­
siderin content.
Treatment. Exploration of the joint and
removal of tissue proliferation by subtotal
synovectomy are required; in some cases ra­
dio-orthesis is also an option, which in prac­
tice means that an isotope is inj ected in the
cavity of the joint. Irradiation of the lesion is
rarely used nowadays, due to the side-effects.
Recurrence of the process is not rare. In case
of serious bone destruction, synovectomy is
combined with endoprosthesis implantation.
As concerns all of the tissues making up
the skeleton, degenerative (regressive) trans­
formations, are most common and most pro­
nounced in the cartilaginous tissue. Damage
to the metabolism of the cartilaginous cells
and to the structure of the intercellular sub­
stance also causes the mechanical characteris­
tics of the cartilaginous cells to change. As a
result ofthe degenerative processes relating to
the tissue, the structure of the cartilaginous
tissue may be completely disorganized and
destroyed.
The degenerative transfo rmations affecting
the joint that are manifested i n typical clinical
symptoms a re referred to as arthrosis

Fig 19.20.
a. Acetabulum destroyed by PVNS (area illustrated by arrows).
b. The involvement of the femoral head and the acetabulum can be well observed in a a picture.
c. The swollen synovial membrane removed from the joint.
160 � 1 9 . D i se a s e s of j o i n t s
a b
Fig 19.21.
Arthrosis of the hand, I. (PM joint in an X-ray and demonstrated in an overview histological picture.
deformans. The degeneration and g radual
devastation of the articular cartilage is accom­
panied by significant changes in the bone
structure of the joint endings, d isordering of
the synovial membrane (synovitis) and modifi­
cation of the structure of the tissues of the
joint capsule. Besides degenerative processes,
proliferative tissue development may also be
observed at the joint endings (e.g. the devel­
opment of osteophytes). Alterations of this
kind give rise to deterioration of a rticular
function and pain (Fig. 19.21.).
The frequency of arthrosis increases rap­
idly after the age of 40. However, it also de­
pend on the joint. It is more common in the
hand, hip, knee and spine, than in the ankles,
wrist, elbow and shoulders. Arthrosis can de­
velop simultaneously in many joints, conse­
quently, 70% of people over the age of 65
have some kind of arthritic ailment.
If some alteration in the metabolism or
structure of the articular cartilage is related
causatively only to a constitutional defect in
the articular cartilage or to old age, it is re­
ferred to as a primary degenerative alteration
(primary arthrosis). On the other hand, if the
degeneration of the joint is initiated or pre-
ceded by a disease (arthritis, osteochondrosis,
etc.) trauma or the incongruence of articular
surfaces, it is called a secondary arthrosis. In
the latter case, the articular transformation
(disease) initiating the arthrosis is called a
state of prearthrosis.
The character of the structural change in
articular cartilages and bone endings in joint
areas, where the pressure is more intense
(weight-bearing zones) differs from that in
other areas where there is only limited or no
pressure (non-weight-bearing zones).
19.8. 1. Alterations in various parts
of the joint
�Weight-bearing zone, alterations of the
hyaline cartilage
The articular cartilage is the primary indi­
cator of the start of degeneration. In some
places, the surface of the articular cartilage
splinters and cracks, its chondroitin-sulfate
content decreases, and the surface loses its
smooth, shiny character. The structure of the
deeper layers also changes, in some places
this means swelling, in others the structure be­
comes crumbly. The number of cartilage cells

a
b
Fig 19.22.
a: Normal and b: degenerative articu lar cartilage.
gradually decreases and irregular, island-like
cell groupings develop at certain places. Cal­
cified salt deposits may also develop in cer­
tain layers of the articular cartilage. Conse­
quently, the integrity of the cartilaginous tis­
sue gradually ceases in these spots, and the
forces affecting the surfaces of the j oint cause
1 9 . D i s e a s e s of j o i n t s � 161
smaller parts o f the articular cartilage to drift
off or become detached from the surface, and
ulcers develop on the articular cartilage. In the
degenerated areas ofthe j oint cartilage, newly
formed blood vessels enter the basal layers of
the cartilage from the medullary cavities of
the cancellous bone through the subchondral
bone, the hyaline tissue begins to degrade and
new cartilage tissue of lower quality (fibrous
cartilage tissue) commences to develop in the
vicinity (Fig. 1 9.22. a, b).
Impairment ofthe cartilaginous tissue usu­
ally starts in an overloaded segment of the ar­
ticular surface and later spreads to other areas.
Various stages of the degenerative process
may therefore be simultaneously observed in
different segments of the articular surface.
The situation is similar in the joint sur­
faces; if the degenerative process begins at
one joint surface, the affected cartilage is ini­
tially in contact with the healthy cartilage of
the other joint surface, but after a certain pro­
gression, degeneration appears here as well.
As a result of the degeneration of the articular
cartilage, the bone endings of the joints par­
tially or completely lose their cartilaginous
coating, i.e. their surfaces consist of bare bone
tissue.
During arthroscopic examinations, the ar­
ticular cartilage can be examined. According
to the above characteristics, such examina­
tions reveal four stages of articular cartilage
alterations (chondropathies):
• 1st stage: the surface is rough, has lost its
gleam and is soft
• 2nd stage: there a re cavities and fissures on
the surface of the a rticular cartilage
• 3rd stage: the surface of the a rticular
cartilage is cracked, discontinuous, some
segments can be mechanically separated.
• 4th stage: there is an u lcer-like defect of
cartilaginous tissue on articular surface, the
subchondral bone appears (eburneation).
Extensive transformations, corresponding
to 2nd-4th stage can be considered symptoms
of arthrosis if they emerge during old age.
162 � 1 9 . D i s e a s e s of j o i n t s

a b

Degenerative cysts in the femur head

a: Overview histological picture, b: intra operative picture.

19.8.2. Alterations in the articular tory area of the cartilage synovial membrane,
multipotential cells begin to generate new
endings of bones
bone tissue, and osteophytes develop (Fig.
The internal structure of the articular end­ 1 9.24.). The gradual growth of osteophytes
ings of bones is also modified due to the changes the shape of the endings of the joints
change in the pressure conditions. In the vi­ and the j oint congruence as well as causes
cinity of the j oint surface the cancellous strain in the articular capsule.
trabeculae thicken as a result of lamellar new
bone formation in certain areas (sclerosis). In
other regions bone cavities of various extent
are formed due to the active resorption of the
bone trabeculae (degenerative cysts, Fig.
1 9.23, a, b). Therefore, when the joint is
placed under pressure, there is a morphologi­
cal possibility for the articular liquid to be
squeezed toward the cysts and the cavities of
the spongiosa which in turn will lead to an in­
crease of the intraosseal pressure and thus the
development of pain.

19.8.3. Transformations in the

non-loaded zone, osteophytes
Even in the early phase of articular degen-
eration next to the articular edge in the transi- Osteophytes in the edge zone of the femoral head.
 1 9 . D i s e a s e s of j o i n t s � 163

19.8.4. Changes in the articular movement and load-bearing and ceases

during rest. In most serious cases, a blunt
capsule
or piercing pain becomes permanent and
The cell layer covering the synovial mem­ its intensity prevents all activities for the
brane becomes multilayered, thickens, and patient. The pain originating from the
the villi become larger. The detached and de­ straining muscles in contracture also
generated cartilage particles embed them­ contributes to the increase in articular
selves in the synovial membrane, where they pain.
� Immobility (contracture). Due to the
are finally absorbed. In the connective tissue
layer of the synovial membrane, aspecific defensive contracture of the muscles and
chronic inflammation develops (synovitis) . the shrinking of the articular capsule, the
Similar processes take place i n the fibrous articular mobility range gradually
layer of the articular capsule: the articular decreases. Deformation of the joint
capsule thickens and shrinks. Calcium salts endings leads to a decrease of articular
may also be deposited develop in the articular movement and to changes in movement
capsule or in its vicinity (capsule calcifica­ direction.
� Deformation. The shape of joints that are
tion).
in a superficial position (elbow, fingers,
knee) become more weighty and deform
19.8.5. Symptoms due to the osteophytes and the swelling of
the joint. During examination the osteo-
Clinical symptoms. Arthrosis may de­
velop in any joint, but it occurs most fre­
quently in the vertebrae, in the smaller j oints
of the hand and in the weight-bearing joints of
the lower limbs (hip, knee, foot). Arthrosis
rarely develops exclusively in a single joint, it
usually affects numerous j oints.
The clinical progression of the disease is
indicated by the repeated appearance of
symptoms and by an increase in their fre­
quency, so that they may even become perma­
nent.

� Pain is the primary indicator of arthrosis.

In most cases the onset, intensity and type
of the pain changes as the degenerative
process progresses. The earliest, but not
typical symptom is fatigue or blunt pain
following strenuous work. The genuine
early pain form is the initiating pain which
occurs when the patient starts to move
hislher j oints, e.g. when taking first the
steps in the morning. During continuous
movement the intensity of the pain
subsides and it may even cease entirely: Fig 19.25.
the joint has "warmed up". Simultan­ Degenerative cysts and sclerosis in the femoral head
eously the articular stiffness also gradually and the acetabulum, the joint space has narrowed in
abates. Later, the pain arises during the pressure lone.
1 64 � 1 9 . D i se a s e s of j o i n t s
phytes can be palpated and the patient is
usually sensitive to pressure at these sites.
� Crepitation. A cracking can often be heard
while the joint is in motion, which can be
uncomfortable and painful for the patient.
X-ray signs. In the initial phase of the de­
generative process, no changes can be de­
tected on X-ray examination, however sec­
ondary arthrosis displays an abnormal articu­
lar picture (dysplasia, subluxation). Early
signs of arthrosis include narrowing of the
j oint space, subchondral sclerosis and
osteophyte development. As the condition
progresses, the articular gap steadily narrows
and degenerative cysts of different sizes de­
velop in the subchondral zone. The j oint space
in the pressure zone progressively contracts
and the structural changes in the bone esca­
late. Finally, the joint endings are signifi­
cantly deformed, the j oint space disappears
and the bone surfaces converge (Fig. 1 9.25).
19.8.6. Treatment of arthrosis
The patient should be informed about the
chronic and progressive character of the dis­
ease, as well as its expected outcome, and all
possibilities which could lead to an improve­
ment, or with a reduction, or even complete
termination of the complaints. The patient 's
cooperation is vital to provide successful
treatment.
General advice. A reduction of the load
(overload) on arthrotic joints is significant
step. Overweight patients should therefore
lose weight (by dieting) under medical super­
vision. A weight gain usually leads to an esca­
lation of the complaints. Exercises involving
non-weight-bearing or swimming promote
the metabolism of the cartilage cells and thus
reduce pain. Permanent immobilization of an
arthrotic joint should be avoided as it leads to
a further deterioration in the metabolism of
the joint and intensifies destruction.
After pregnancy and child-bearing, a dete­
rioration of hip arthrosis and the escalation of
related symptoms are to be expected.
The conservative and surgical procedures
during treatment are closely harmonized.
Conservative treatment
� Medication. No specific medication is
currently available to cure for arthrosis,
however chondroitin-sulfate medications
does exert a positive effect on the
progression of arthrosis. Pain killers,
salicylates, non-steroidal anti-inflam­
matory drugs and muscle relaxants have
beneficial effects. The use of steroids is
very rarely necessary (in arthritis that does
not respond to any other medical treatment
and there is a large fluid collection in the
j oint) .
� Physiotherapy. This too has a beneficial
effect and can be of great help in the
treatment of arthrotic patients. It is
especially useful in reducing articular
contractures and pain as are curative
massages and underwater exercises.
� Extension treatment. This treatment can
provide relative comfort and relief from
pressure leading to a decrease in pain and
possibly even termination of the
contractures. It is primarily used in
degenerative diseases involving the lower
limbs and spine.
� Therapeutic X- ray irradiation. This may
assist in relieving pain completely.
However, depending on the patient' s
radiation exposure this treatment can
usually be repeated only after an interval
of some years .
The various conservative treatment meth­
ods are usually employed simultaneously.
Surgical treatment. The aims here are to
reduce pain as well as, to improve the joint
function and to correct the deformities that
have developed. Accordingly, osteotomy,
arthroplasty and arthrodesis may be per­
formed. Surgical procedures relating to spe­
cific joints are dealt with in the appropriate
sections .
 J6zsef La katos
20. Bacterial infections of bones and joints
Inflammation of the musculoskeletal sys­
tem may be of various origins.
� Reactive, sterile arthritis types:
transitory
after abdominal surgery
caused by a metabolic disturbance
(gout)
accompanying arthrosis
� Viral, mycosis
� Specific (tu berculosis or syphilis)
� Accompanying rheumatic disease
� Bacterial
� Iatrogenic
The above classification is an arbitrary
one. Transitory arthritis which appears in
childhood and generally ceases spontane­
ously, will be discussed separately (see Chap­
ter 28. 1 ).
Sterile inflammation can also be experi­
enced in degenerative diseases with articular
erosion and as a result of the deposition of
metabolic products in the j oints. In rheumatic
diseases, one of the sites of the pathological
process is the synovial membrane. The vari­
ous types of synovitis with quite different eti­
ologies mostly cause similar symptoms and
have to be taken into consideration in the dif­
ferential diagnosis .
Inflammations of the osteoarticular sys­
tem are from some respects different from
those of the parenchymal organs. Some parts
of the bone act as bradytrophic tissue and it is
therefore difficult to attain a therapeutic con­
centration of antibiotics. There are a limited
number of antibiotics that accumulate at an
optimal concentration in the bone. Articular
movement makes the elimination of the in­
flammation more difficult. An inflammatory
process (mainly bacterial in origin), in some
circumstances can recrudesce in the bone or in
the synovial membrane after years or decades.
Hence, in many cases it is not possible to be
certain whether the process has merely
paused, or the healing is permanent.
Acute and chronic inflammation of bones
and j oints is often caused by bacterial infec­
tion . Before the discovery of antibiotics, these
diseases were life- threatening. Since the in­
troduction of antibiotic treatment however,
the results are more favorable, though the
ability of the pathogens to resist antibiotics
makes the selection of a potent antibiotic dif­
ficult.
20. 1. Osteomyel itis
This is the inflammation of bone and bone
marrow, caused by pyogenic pathogens. It
can develop as a consequence of
hematogenic or external (fractures or surgery)
infection.
20. 1 . 1 . Acute hematogenic
osteomyelitis
This condition can occur in infancy, in the
neonatal period, in childhood and in adult­
hood equally. The course depends on the age
of the patient, the virulence of the pathogen,
the immunological state of the organism, the
localization of the infection, and the features
of the bone blood supply.
Staphylococcus aureus is most frequently
responsible for the development of acute
hematogenic osteomyelitis; it can be demon­
strated in about 90% of the cases. However
this disease can also be caused by any kind of
pyogenic, mycostic, parasitic or viral infec­
tion.
166 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i n ts
In 75% of the cases, acute hematogenic
osteomyelitis is encountered in children and
adolescents, though in recent years the acute
form of vertebral osteomyelitis has been seen
more often among adults and the elderly too,
as a complication of inappropriately treated
pneumonia, cystopyelitis, abdominal and mi­
nor pelvis surgery and after discus hernia sur­
gery with a febrile postoperative period.
Etiopathology. Rosenbach found evi­
dence that the hematogenic osteomyelitis is
caused by infection ( 1 878), and the predomi­
nant role ofthe Staphylococcus strains was re­
vealed by Becker in 1 88 3 .
The behavior o f Staphylococcus strains in
the pathogenesis of hematogenic osteo­
myelitis was examined a century ago by the
surgeon Laxer. It is typical of the Staphylo­
coccus strains that the pathogenic organisms
adhere together in the shape of a bunch of
grapes. These conglomerates (bacterial
emboli) can collect in the capillary system of
the bones and even in the nutrient arteries.
The characteristics of the blood supply have
been known since the researches of Treat
( 1 959). Because of the terminal arteries, the
vascular system of the metaphysis provides
the greatest potential for the bacterial emboli
to adhere and thus the danger of the develop-
Fig. 20.1.
Epiphyseal osteomyelitis i n infancy, with destruction
of the left hip metaphysis.
ment of osteomyelitis caused by pyogenics is
most pronounced in the metaphysis.
The extent and the course of the process
and its localization in the bone depend on the
anatomical features of the blood supply.
� Among neonates and infants (under the
age of 1 year) the blood vessels penetrate
the growth cartilage for the epiphysis.
This makes it possible for the inflamma-
tory focus in the metaphysis to break into the
epiphysis, causing purulent arthritis in the
joint (see Chapter 20.2).
It is extremely important if the infection
occurs at the proximal end of the femur, be­
cause of the intra-articular localization of the
metaphysis (Fig. 20. 1 .).
At this age severe destruction of the joint
and the growth cartilage and longitudinal
growth disorder are typical (Fig. 20.2.).
Fig. 20.2.
Late result of epiphyseal osteomyelitis that occurred
in infancy: the right hip joint has been destroyed,
the head and neck of the femur are damaged, the
metaphysis is widened, and the limb is shortened.
 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i n t s � 167

I n infancy, osteomyelitis I S called in the proximal metaphysis of the tibia and the
epiphyseal osteomyelitis. humerus, and in the distal metaphysis of the
The process generally develops in the radius (Fig. 20.3. a, b, c) .
proximal and distal metaphysis of the femur,

a b

Fig. 20.3.
a: Result of distal femora l metaphyseal osteomyelitis that
began in a neonate: serious bone defect and deformity at
the age of 2.
b: At the age of 2 1/2, the medial femur condyle was re­
placed by a bone graft taken from the iliac crest.
c: The redeveloped, sufficiently congruent femoral condyle
at the age of 6 1/2.
168 � 2 0 . B a cte r i a l i nfect i o n s o f b o n e s a n d j o i nts
� In childhood (after the age of I) the
growth plate, except for a narrow peri­
pheral part, becomes impenetrable for the
blood vessels, and accordingly the j oint is
protected from the spreading of the in­
flammation that has developed in the
metaphysis.
After the course of the disease, growth im­
pairment is rarely experienced. Because ofthe
excitement of the growth zone, hypertrophy
of the bone can occur, similarly as in inflam­
mations caused by nonpyogenics e.g. in rheu­
matoid arthritis.
� In adulthood, the growth cartilage
ossifies, and because of the developed
vascular system the purulent bone process
can break into the joint.
The periosteum adheres tightly to the
bone, and therefore cannot be elevated easily
from its base by the pus. The process mainly
occurs inside the bone. In the absence of re­
storative ability, a chronic course and relapse
are frequent.
In general this condition can develop in the
extremity bones, the short, flat bones, the pel­
vis, and the vertebrae.
B acterial emboli that have entered the
blood stream can become blocked in the capil­
lary system of the metaphysis, causing an
acute inflammatory bone process and various
extents of osteonecrosis. An abscess can de­
velop and can spread through the Haversian
and Volkmann canals to the medullary space
and the periosteum. The periosteum is lifted
by the abscess, and in severe. cases it can break
out into the soft parts .
C linical symptoms. The onset of hemato­
genic osteomyelitis often remains unnoticed;
the patient and the relatives merely recall the
septic condition accompanied by fever. The
patient frequently presumes a connection be­
tween the condition and some traumatic
event. Thorough questioning generally re­
veals information concerning dermatological
or dental illnesses, urinary infections, pneu­
monia or previous operations involving com­
plications.
The pain persists even when the patient is
in a resting position, and at night it can inten­
sify and pulsate. The reason for this is the in­
creasing pressure in the medullary space,
which is unable to expand.
The limb can display pasty consistency, or
a hard substance; if the abscess breaks
through the bone into the soft parts, fluctua­
tion can be observed.
An atypical, but not rare symptom in in­
flammations in deep-lying bones, is that
above the infected area the temperature of the
skin increases (calor heat); this may be de­
=
tected by comparative palpation of the two
sides.
The impaired function ofthe inflamed area
(functio laesa), is caused by the pain and also
by the muscular spasm induced by the pain.
This is manifested in the resting of the
limb, contracture, and restricted movement of
the involved j oints.
The disease usually begins rapidly and
dramatically, with a high fever, shivering and
prostration. Because of the frequent usage of
antibiotics, these symptoms may be compara­
tively slight, assuming an atypical form, and
this can impede an early, correct diagnosis.
Laboratory examinations show numerous
typical differences. In acute hematogenic
osteomyelitis, the erythrocyte sedimentation
rate ( l OO mmIh), and the protein level in the
acute phase (C-reactive protein) can be ex­
tremely high. Besides a high leukocyte count,
the differential blood count is noticeably
shifted to the left.
In a septic condition, the patient easily be­
comes anemic; a decreased red blood cell
count, the hemoglobin level, the renal func­
tion, and the liver function are therefore im­
portant features.
X-ray signs. In inflammatory bone pro­
cesses, the X-ray signs appear after 8- 1 0 days
in children, and 2-3 weeks later in adults. The
bidirectional X-ray image demonstrates typi­
cal alterations .
The inflammatory process is surrounded
by an atrophic bone structure. The purulent,
confluent foci induce the resorption of the
 2 0 . B a ct e r i a l i nfect i o n s of b o n e s a n d j o i n t s
bone trabeculae, and i n these cases, an exten­
sive rarefied area can be seen inside the atro­
phic environment, with an indistinct periph­
ery and an elevated periosteum, which ap­
pears as a continuous, narrow strip (Fig. 20.4.
Fig. 20.4.
a: In the femur diaphysis, a lytic rarefaction can be
observed, with a sclerotic edge at some sites, with a
shadow that may indicate the presence of a
sequestrum with a periosteal reaction.
b: The MR image (horizontal reconstruction) reveals
the edema of the neighboring muscles.
� 169
a, b). Later, the focus is surrounded by a scle­
rotic area with increased X-ray density; the
thickness of the bone is increased by the
periosteal reaction. Some parts of the bone
can necrotize, and later the necrotic cortex is
surrounded by a sequestrum: the necrotic
bone part sequestrates (see: Chapter 20. 1 .2).
If the inflammation breaks into a j oint, the
articular surfaces can be rough, and the effu­
sion increases the distance between the sur­
faces. Later, if the process becomes chronic,
since the chondronecrosis narrows the j oint
space, the articular destruction increases. In
destructive inflammatory processes of the tu­
bular bones, pathological fractures can occur.
Bone scintigraphy is a useful, but nonspe­
cific examination technique which is per­
formed by the administration of 99mTechne­
tium phosphonate or 67Gallium citrate.
Though the technetium procedure is non­
specific, it shows a technetium isotope con­
centration in inflammatory areas, even in the
early stage of the inflammation, when the
X-ray image still appears negative. This con­
centration is as a result of the increased circu­
lation and osteoblast activity.
Fistulography is a radiological examina­
tion technique, adopted for the determination
of advanced processes, the expansion of bone
cavities or the presence of a sequestrum. For
this examination, contrast material is inj ected
into fistulous canals, after which X-ray exam­
ination is performed.
Inflammatory bone destruction can be
checked by means of CT, while the intra­
medullary extent of the process and the edema
of soft parts can be demonstrated through
MR!. These examinations have led to a great
advance in the differential diagnosis of in­
flammatory bone processes, and especially in
the distinction from other, generally tumorous
processes.
Diagnostic algorithm. The clinical pic­
ture, the X-ray signs, and the laboratory ex­
aminations provide a sufficient basis for the
correct diagnosis. Identification of the patho­
gen is followed by the establishment of the an­
tibiotic sensitivity of the bacterium in order to
1 70 � 2 0 . Bacte r i a l i nfect i o n s of b o n e s a n d j o i nts

formulate a treatment plan. Material including
pus, tissue and detritus, taken by puncture or
instrumental biopsy, is subj ected to general
bacteriological examination, Koch culture,
cytological examination and histological ex-
amination, so as to confirm the diagnostic sus­
picion.
In a septic condition, with febrile periods,
hemoculture examinations help to reveal the
pathogen.

Table 20/1.
Antibiotic treatment
Generic name Commercial name Mode of administration Most frequent side-effects

Clindamycin Dalacin C iv. or orally Abdominal pain, nausea, vomiting,

diarrhea, esophagitis
Ciprofloxacin Ciprobay, Cifran iv. or orally Gastrointestinal side-effects, impaired
renal function, psychic effects,
chillorhexis, pseudomembranous colitis
Amoxicillin + Augmentin iv. or orally Diarrhea, nausea, pseudomembranous
clavulonic acid colitis, candidiasis, hepatitis, cholestatic
icterus, erythema mu ltiforme
Cefu roxim Zinacef, Zinnat, iv. or orally Allergic reactions, erythema multiforme,
Xorim pseudomembranous colitis
Vancomycin Vancocin CP iv. Urticaria, pru ritus, asphyxia, hypotension,
shock, nephro- and ototoxicity,
thrombocytopenia
Teicoplanin Targocid iv. or im. Exanthema, pruritus, fever, colic,
anaphylaxia, shivering, u rticaria
Carbapenem Meronem, Tienam iv. Inflammation at the site of
administration, thrombophlebitis, pain.
Allergic reactions, abdominal pain,
pseudomembranous colitis, convulsions,
candidiasis, headache, paresthesias
Netilmycin Netromycine iv. or im. Nephro- and ototoxicity, headache,
weakness, visual disturbance,
disorientation, fever, tremble
Gentamycin Gentamycin iv. or im. Nephro- and ototoxicity, vertigo, tinnitus,
nystagmus, deafness, peripheral
neuropathy, articular pains, psilosis
Linezolid Zyvoxid iv. or orally Abnormal liver fu nction values,
dysgeusia, diarrhea, nausea, vomiting,
vaginal moniliasis, other laboratory
divergences
Rifampicin Rifamed, Tubocin orally (in combination) Colors the body fluids orange, hepatitis,
nausea, vomiting, diarrhea,
pseudomembranous colitis, pru ritus,
ataxia
Fusidic acid Fucidin orally or iv. (in Gastrointestinal complaints, jaundice,
combination) allergic reaction, granu locytopenia,
thrombocytopenia
 2 0 . B a ct e r i a l i nfect i o n s of b o n e s a n d j o i nt s
Differential diagnostics. The clinical pic­
ture must be distinguished from Ewing tumor
which gives similar laboratory and radiologi­
cal results. In Ewing tumor, the periosteal re­
action reveals an "onion leaf' structure and
the roughness of the cortex from the direction
of the medullary space. These can be distinc­
tive features.
Treatment. One of the most important
methods for the treatment of acute bacterial
inflammatory processes of bone articulations
is rest.
Depending on the age, the general condi­
tion and the region, this can be bed rest, exten­
sion treatment, the application of plaster (im­
mobilization splints), a plaster bed, corset,
etc.
The treatment should start with a broad­
spectrum antibiotic that accumulates in ap­
propriate concentration in the bone tissue. Af­
ter this in the knowledge of the results of the
bacteriological culture, directed antibiotic
treatment should be initiated (Table 20/1 .).
Antibiotics, most freq uently used to treat
bone-articular infections, osteomyelitis and
puru lent arthritis, together with the mode of
administration and the most frequent
side-effects (the dosages are specified in the
usage instructions).
If a few days after antibiotic therapy is be­
gun there is no spectacular clinical improve­
ment and regression of the laboratory data
surgical treatment of acute hematogenic
osteomyelitis may be recommended (verte­
bral processes can be exceptions; see Chapter
24).
Important surgical interventions:
- Exposure of an abscess, and drainage of
the pus
- Exposure of an enclosed bone abscess
by trepanation
- Focus cleaning
- Sequestrotomy
The healing process is indicated by the
subsidence of the clinical symptoms and the
� 171
regression of the radiological picture, with the
laboratory findings progressively becoming
negative.
20.1.2. Chronic osteomyelitis
Despite appropriately performed surgical
treatment and massive a ntibiotic therapy, it
can occur that
� the treated acute form becomes chronic;
� acute osteomyelitis relapses years later and
becomes chronic;
� the acute period is passed in a subclinical
form, which is manifested as a chronic
condition.
Chronic osteomyelitis does not endanger
the life of the patient, because a balance sets in
between the inflammatory bone process and
the resistance capacity of the organism. In the
cancellous bone, bacteria can survive in a
larvate condition, encapsulated by the scarry
environment, and can subsequently become
virulent again, thereby reactivating a process
thought to have been healed. In the area of old
inflammation, the skin becomes tight and red,
the limb swells up, and fluctuation may be ob­
served, accompanied by fever, a high sedi­
mentation rate, and typical blood test alter­
ations. A fistula can develop in the soft tis­
sues, leading to regression of the clinical
symptoms. In the event of retention, a re­
peated febrile condition must be expected.
The frequency of acute hematogenic
osteomyelitis in childhood and also that of the
ensuing chronic bone processes have de­
creased in the last few decades. Strong new
bone formation is typical of chronic
osteomyelitis in childhood. The affected limb
can thicken, and can even be longer than the
contralateral limb.
The X-ray image may reveal that the bone
structure is altered throughout the entire
length of the diaphysis. In adulthood the
X-ray symptoms exhibit a rather varied pic­
ture. However the bone thickening due to the
sclerosis is always typical (Fig. 20.5. a, b). In
the sclerotic areas, sequestra are frequently
1 72 � 2 0 . B a cte r i a l i n fect i o n s of b o n e s a n d j o i n ts
found. If the lytic and restorative processes
are in equilibrium, the X-ray picture resem­
bles a honey comb. Depending on the viru­
lence of the pathogens, large bone abscesses
can emerge, with a tendency to distal spread­
ing. As this process progresses, pathological
a ing cavities can be filled with a muscle lobe,
b
Fig. 20.S.
a: Picture of chronic femur osteomyelitis, with thick­
ening of the femur, and with a variety of lytic and
sclerotic bone structure changes.
b: The a scan depicts the extended periosteal reac­
tion.
fractures can develop. Fistulography and a eT
scan can reveal the location and size of the in­
flammatory focus and the position of the
sequestrum.
Treatment. The process can drag on for
decades, and the treatment therefore demands
patience on the part of both the patient and the
physicien. A good result can be achieved only
from radical surgical intervention . Opening
of abscesses, excochleation, and excision of
fistulas afford only a temporary solution. Af­
ter the removal of necrotized tissues and
sequestra, suction and irrigation drainage may
be the best surgical choice, but success is not
guaranteed.
For the local antibiotic treatment of a
cleaned bone cavity, polymethyl metacrylate
beads (PMMA Septopal chain) containing
gentamycin or some other antibiotic may be
implanted. This is required, because the struc­
ture of the walls of the bone cavities (inflam­
mation barrier) impedes the achievement of
an effective local antibiotic concentration if
general antimicrobial treatment is applied.
After focus-cleaning surgery, the remain­
autologous bone, etc.
However, it may be taken as a principle,
that in cavities involving active inflammation
and containing purulent fluid, the implanta­
tion of foreign material or bone is not expedi­
ent until the process has subsided.
20. 1.3. Special forms of primary
chronic osteomyelitis
Some types of primary chronic osteo­
myelitis can diverge from the general in ap­
pearance and course. Etiologically, the possi­
bility of the presence of a pathogen of low vir­
ulence may arise, though in most cases the
agent cannot be isolated. These types occur in
adolescence or at a young age, which suggests
that intense tissue-protective mechanisms
may play a role in the process. The history of
the patients does not reveal septic or febrile
episodes.
 2 0 . B a ct e r i a l i nfect i o n s of b o n e s a n d j o i n t s � 1 73

� Brodie abscess in a spindle-like form on the metadiaphysis of

long tubular bones.
This is an inflammatory focus that develops in
the metaphysis of the bones. The typical signs Symptom: pain.
are pain, limited joint movement, and a
Occurrence : in childhood and adoles­
marginated, round or oval region of lytic de­
cence.
struction with a sclerotic margin in the
Clinical symptoms: there is a subdued
metaphysis demonstrated by X- ray. This a b­
onset that drags on, with uncertain pain and
scess is often observed as an accidental find­
ing.
f�brility, but without particular laboratory
sIgns.
Brodie distinguished this alteration from In the X-ray image the diaphysis of the
other types of osteomyelitis long ago as 1 863 . tibia or the femur exhibits an expressed scle­
Because of the relatively strong resistance rosis, with a spindle-like thickening and con­
of the organism and the low virulence of the siderable narrowing or the possible disappear­
pathogen, the inflammation process inside the ance of the medullary cavity.
bones appears in a larvate form. The cavity Differential diagnosis : the possibility of
contains necrotic cells, granulation tissue and osteoid osteoma must be considered (Fig.
a dense mucous liquid, from which bacteria 20.6.).
can not be isolated. The cavity is enclosed in a
connective tissue envelope.
Clinical symptoms. Mild swelling (in the
event of a superficial location), tenderness,
and sometimes a dull, dragging pain can be
observed. The neighboring j oint can respond
to the close-lying inflammatory focus with ef­
fusion and limited movement. Fever is not
typical, and the laboratory findings are rarely
informatory.
X-ray signs. In the metaphysis of the fe­
mur, tibia or humerus, a round or oval, scle­
rotic-edged lesion, 1 -2 cm in diameter can be
seen. Rarely there is a mild periosteal reac­
tion. Depending on the size, it may be neces­
sary to consider osteoid osteoma, osteo­
blastoma, benign chondroblastoma and bone
cyst in the differential diagnosis.
The essence of the treatment is exposure,
excochleation of the cavity and autologous
bone implantation, all under in antibiotic pro­
tection.
� Garre osteomyelitis

This disease, described by Garre in 1893, is

also called sclerotizing osteomyelitis, because
of the predominance of sclerotizing and
proliferative elements in the inflammation of
Fig. 20.6.
long tubular bones. The major feature of this
Garre's sclerotizing osteomyelitis in the mid part of
disease is the sclerotic thickening, developing
the tibia
1 74 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i nts
Treatment. Longitudinal sawing of the
thickened cortex and opening of the
medullary cavity can relieve the complaints,
and the process may subside. A culture of the
removed bone tissue is generally negative.
The process can recrudesce years later,
and the complaints and the pain can recur.
20. 1.4. Special forms of
inflammatory bone alterations
� Iatrogenic infections
� Posttraumatic osteomyelitis
� I nfections in the environment of stabilizing
and mobile implants
� An iatrogenic infection is a result of a
medical intervention.
It can develop after surgery or intra­
articular inj ection treatment. Despite the in­
fection being a consequence of medical activ­
ity, it is difficult to determine in which phase
of the intervention, or where and when the in­
fection occurred or even whether it was
caused exogenously at all. These questions
may be answered only if all of these issues are
considered.
� As concerns post-traumatic infections,
open fractures are regarded as potentially
infected.
In these cases any complications that oc­
cur after the surgical interventions are held to
be iatrogenic.
� Infections, developing in the environ­
ment of stabilizing devices (metal
plates) or mobile implants (endo­
prosthesis).
Infections emerging early after the treat­
ment of closed fractures (after surgical stabili­
zation) should be considered iatrogenic.
In the environment of implants, even if the
operation is performed under ultra sterile cir­
cumstances, infection occurs in 0.3-0.5% of
the cases, despite the sterile air flow, the spe­
cial surgical exhaust suit and the antibiotic
prophylaxis making the contamination virtu­
ally impossible.
Under the customary conditions, the fre­
quency of septic complications after hip pros­
thesis operations can reach 2%.
It is generally accepted in the literature
that infections developing within 6 months af­
ter surgery are due to contamination, and can
therefore be regarded as iatrogenic, while
those that develop after 6 months (late infec­
tions) due to hematogenic dispersion. Never­
theless the cause of a late infection may also
be contamination, but the virulence of the
pathogen and the resistance of the organism
are such as to allow only a late manifestation,
so that can be classified as so called low-grade
infection.
The recognition ofpost-traumatic bone in­
fection does not pose any problem, because
this is indicated by the open fracture or the fe­
brile condition after the osteosynthesis, and
the laboratory alterations proving the inflam­
matory process
The pain in the injured limb does not de­
crease, but becomes stronger. Inflammatory
symptoms develop in the operated area. X-ray
signs appear later. The first sign is bone atro­
phy, which is followed by a callus formation
failure. The fractured bone-ends may undergo
destruction and condensation, indicating that
avascular necrotized bone parts have taken
the place of live tissue.
Broken-off bone pieces can sequestrate.
Lytic, rarefied areas evolve around the
screws, which therefore loosen and the fixa­
tion becomes insufficient. The inflammatory
process is maintained and in many cases in­
tensified by the instability.
Septic pseudarthrosis is a serious compli­
cation, treatment of which is a quite compli­
cated task. If it is accompanied by bone loss, it
is defined as defect pseudoarthrosis (Fig.
20.7. a, d). The essential feature of the solu­
tion is the stabilization of the broken ends. As
it is often necessary to remove all foreign ma­
terials from the area of the inflammatory fo­
cus, the method of choice is extrafocal osteo­
synthesis. The process can often be solved
only by a series of surgical interventions. If
 2 0 . B a cte r i a l i n fe ct i o n s of b o n e s a n d j o i n t s � 1 75

a c

b d

Fig. 20.7.
a: Septic pseudarthrosis with a severe leg deformity, involving shortening and an u nstable limb.
b: A bone defect can be observed in the X-ray image, with a fibula deformity.
c: Postoperative picture: fibula implantation i nto the lateral condyle of the tibia, with screw fixation and
spongiosa plasty, has been followed by bony consolidation.
d: After surgery, the limb is stable, with a more favorable alignment.
1 76 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i nts
the fixing pins of the externalfixateur are po­
sitioned distant from the inflammation, in the
healthy parts, the broken ends can be stabi­
lized which leads to subsidence of the inflam­
mation. Remodeling of the bone is often pos­
sible only by bone transplantation.
Infections of mobile implants and articu­
lar endoprostheses belong in a different brand
of orthopedic surgery.
Infections can be either early or late com­
plications of surgery occurring within a year.
The course can involve an acute, subacute or
chronic inflammatory process, with the corre­
sponding clinical and laboratory symptoms.
Clinical symptoms. A late haematoma,
which is a subcutaneous or subfascial clot
mass evolving as a result of a slowly oozing
hemorrhage, and which can diffuse between
the tissue layers, develops around postopera­
tive day 7. It may be accompanied by sub­
febrility or febrility. This gives rise to the sus­
picion of an early infection.
Accordingly the prevention of hematoma
formation is very important. If it does develop
the earliest possible evacuation under sterile
circumstances is mandatory.
Acute infection can develop within 1 2
weeks after the operation. The patient suffers
from permanent pain, and subfebrility or fever
can be observed. In the operated area,
induration and edema may be noted. Labora­
tory examinations indicate the infection. Be­
fore the healing of the wound, a purulent dis­
charge begins that can be long-lasting and
abundant.
In cases of subacute infection the erythro­
cyte sedimentation rate is slightly high. In ret­
rospect as concerns the early postoperative
period, the former moderate fever, sub­
febrility, the edemic wound periphery nor­
malize spontaneously. The patient may feel
well for months before the appearance of pain
in the hip area. The occurrence of the com­
plaints is generally associated with the ap­
pearance of the radiological and isotopic signs
of prosthesis loosening.
Initially X-ray alterations can not be ob­
served, or they are merely very slight: peri-
prosthetic bone atrophy, a periosteal reaction,
and a narrow discrete radiotransparent stripe
at the boundary of the implant and the bone, or
at the boundary of the cement and the bone.
Laboratory examinations demonstrate a
high sedimentation rate, CRP (C-reactive pro­
tein), and a differential blood count shifted to­
ward the left. Ultrasonography of the joint
demonstrates an articular effusion, with in­
creasing density, which is a result of the tissue
detritus in the fluid. An urgent puncture fur­
nishes information on the nature of the articu­
lar fluid (hematoma, infected hematoma or
pus) . It is expedient to perform an immediate
bacteriologic examination, and if the case is
not acute, immediate exposure under antibi­
otic protection, cleaning and drainage are sug­
gested; if the case is subacute, after the culture
and resistance results have been obtained, it is
sensible to perform the exposure and lavage
and to insert a suction drain as early as possi­
ble: experience indicates that an early expo­
sure (within some weeks) helps solve the pro­
cess meanwhile the prosthesis is retained.
Slow infection generally appears months
or even years after surgery that was thought to
be successful. It is manifested as insidious,
dull, monotonous pain, which cannot be eased
by motion.
When late septic complications develop,
the leading clinical signs are a painful limita­
tion of movement, a sensation of instability,
fever, and subfebrility. The operated area can
be infiltrated and warm, but it can even be
normal if the infection is of low grade.
The laboratory tests may detect the classi­
cal signs of inflammation, depending on the
virulence of the pathogen, but in a torpid case
or if there is a fistulous process, they can be
negative.
Signs of septic implant loosening can be
observed in the X-ray image: a periosteal re­
action, cirrus-cloud-like focal osteolysis in
the bone structure, snowflake-like para-arti­
cular calcified shadows, and a transparent
layer between the bone surface and the im­
plant (Fig. 20.8.).
To clarify the diagnosis, articular puncture
and bacteriologic examination are recom-
 2 0 . B a cte r i a l i n fect i o n s of b o n e s a n d j o i nts
Fig. 20.S.
a: X-ray picture of the septic loosening of the hip endoprosthesis. Septic hip prosthesis: solution in two
steps.
b: Excisional arthroplasty - Girdlestone situation.
c: After the healing of the process, reimplantation of hip endoprosthesis.
mended besides the above examinations . Un­
fortunately, after puncture, one-third of the
traditional bacteriological cultures prove to be
negative.
There is a modem molecular biology
method for isolation of the DNA of the bacte­
rial wall from the aspirate.
If the typical symptoms are absent, per­
forming a gallium isotope scan can facilitate
the differentiation of sterile and septic loosen­
ing.
Treatment. Some decades ago, septic
complications were solved by total reveal of
the prosthesis, and Girdlestone surgery
(pendular hip).
After removal of the prosthesis and the
head and neck of the femur, the remaining
proximal part of the thigh bone was inclined
against the pelvis, resembling the way in
which a thinking individual bends the head
into the palm. This led to a well-moving, pain­
less but unstable hip : the patients could walk
� 177
only with a walking aid. Nowadays, the solu­
tions chosen that are more appropriate for the
patient. After the subsidence of the process
and the eradication of the infection the aim is
implantation of a new prosthesis. This can be
performed as an immediate reimplantation, or
in a two-step process. This latter also gives
two options:
1 . Implantation of an antibiotic spacer, with
reimplantation after the subsidence of the
process.
2 . The removal of all foreign materials, with
reimplantation 4-6 months after antibiotic
treatment (Fig. 20.8. a, b, c).
20.2. Puru lent arthritis
A condition of articular inflammation caused
by pyogenic pathogens is called purulent a r­
thritis.
1 78 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i nt s

The inflam mation o f t h e joint can b e primary
if the process begins initial ly in the a rticula­
tion, and secondary if the process com mences
in a d ifferent part of the organism and
spreads to the joint. Primary infections usually
occur in childhood, at which age it is twice as
frequent as osteomyelitis.
Etiopathology. The pathogenic agent in
90% of the cases is Staphylococcus hemo­
lyticus, but all pathogens that may be in­
volved in osteomyelitis must be taken into
consideration.
Pathogens may enter an articulation in var­
ious ways:
� Via the blood stream, as a result of an
infectious process in a different part of the
organism.
� By direct spreading, as a result of sup­
puration occurring up in the metaphysis.
� As a result of an injury penetrating the
joint.
� In an iatrogenic way, during an intra­
articular inj ection, and especially a steroid
inj ection (sterility! ) .
The process in the joint depends consider­
ably on the virulence of the pathogen and on
the resistance of the organism.
� In serous arthritis, the synovial membrane
is purple and sanguine, while the exudate
is transparent and serous.
� In serofibrinous arthritis, the joint is filled
with a fibrin-containing turbid exudate, in
which bacteria multiply.
� In purulent arthritis, which results from a
severe infection, the j oint is filled with
thin, yellow pus. The articular capsule and
the synovial membrane are thickened:
inflamed, sanguine and in some places
necrotic. The articular cartilage is lifted
from its base. The pus can easily break
through and form a periarticular abscess or
fistula.
Clinical symptoms. The general symp­
toms are manifested early: Prostration, fever,
shivering and a loss of appetite. The labora­
tory findings reveal a high sedimentation rate
and CRP and leukocytosis, with the blood
count shifted to the left. The pain increases in

a b

Fig. 20.9.
a: Septic arthritis induced by Staphylococcus in the left knee-joint. The articular surface has been destroyed,
and deep destruction of the subchondral bone can be observed.
b: Surgical exposure with serious destruction of the femur condyles and disorganization of the joint.
 2 0 . B a ct e r i a l i n fect i o n s of b o n e s a n d j o i n t s
response to the slightest movement of the ar­
ticulation. The joint is in a mid-position or a
forced position; this is how the patient tries to
ease the pain. The articulation is swollen, and
its contours are blurred. The skin is warm to
touch, and reddish. If the local symptoms
worsen, the general condition can also deteri­
orate, and a serious septic, toxic state can de­
velop.
X-ray signs. Initially, there are no signs,
but the great amount of exudate enlarges the
articular cavity. After 2-3 weeks, the bone
structure becomes atrophic (local osteopenia) .
The damage to the chondral surface leads to
unevenness and tightening of the articular
cavity. Subchondral cysts can appear (Fig.
20.9. a, b).
Treatment. Before the discovery of anti­
biotics, a purulent inflammation of a large,
weight- bearing j oint could threaten the life of
a patient.
Nowadays, thanks early diagnosis and the
availability of modem antibiotic and surgical
treatment, there is even an opportunity for the
healing the process to end up with a moving
articulation.
By puncture of a diseased articulation, the
tense, painful joint can be relieved, and mate­
rial can be collected for laboratory, bacterio­
logic examinations of the synovial fluid and
exsudate. Until the results are obtained the pa­
tient should be treated with a broad spectrum
antibiotic .
Punctures can be repeated. The need for
radical surgical exposure depends on the gen­
eral condition of the patient and on the local
alterations. In some cases, rinsing and lavage
performed under arthroscopic intervention,
with the application of a suction-rinsing
drain, may be sufficient.
In a serious, purulent process, however ar­
ticular exposure, subtotal synovectomy and a
suction or suction-rinsing drain should be
chosen as a solution.
When the clinical symptoms have sub­
sided, extremely careful moving of the articu­
lation is allowed.
� 1 79
In advanced cases involving severe de­
struction a loss in function of the affectedjoint
and sometimes the development of ankylosis
should be expected.
20.3. Bone and joint tuberculosis
This is a secondary tuberculotic process in the
bone o r articulation. Symptoms are slowly de­
veloping pain, swelling and limitation of
movement.
Occurrence. As for pulmonary and extra­
pulmonary (lymph node, urogenital, meningi­
tis) manifestations, articular tuberculosis was
earlier a serious widespread disease in Hun­
gary, as elsewhere.
Thanks to the organizations established in
the 1 950s within the anti-tuberculosis net­
work, this grave illness was almost totally
eradicated.
In the past few years, however various
negative changes, such as the increasing num­
ber of unemployed, the social problems of the
homeless, alcoholism, and the spreading of
immunodeficiency states (AIDS), have led to
a resurgence of tuberculosis worldwide. Ac­
cordingly, the number of newly diagnosed
extrapulmonary cases has moderately in­
creased.
The data published in 2000, gave the inci­
dence of tuberculosis in Hungary as 36 per
100 000; the number of extrapulmonary cases
was 246, of which 33 were newly diagnosed
articular tuberculosis (Fig. 20.10.). Hence, al­
though relatively rare, the possibility of a rticu­
lar tuberculosis must be thought of, and suc­
cessful treatment demands a knowledge of
the clinical pictu re.
Etiopathology. Articular tuberculosis is
caused by Mycobacterium tuberculosis (Koch
bacillus), discovered by Robert Koch in 1 882.
In 90% of the cases, the primary focus is
induced by bacteria invading the lungs and in­
fecting the regional lymph nodes (primary
complex). As a result of hematogenic disper-
180 � 2 0 . Bacte r i a l i nfect i o n s o f b o n e s a n d j o i nts
pleuritis a n d others 42%
bone and joint 13%
Fig. 20.10.
Tubercu losis pie chart, showing the d istribution of extrapu lmonary forms.
sion, the bacteria can spread to other parts of
the body (bones and urogenital organs), giv­
ing rise to post-primary tuberculotic lesions.
Tuberculosis bacteria induce tuberculosis
gemmation and caseous necrosis. The disease
is caused by bacteria ofhuman or bovine type.
The pathomorphologic alterations and the
clinical pictures caused by the two types of
bacteria do not differ.
Bone and joint tuberculosis is a severely
destructive disease. The mycobacterium ad­
heres in the terminal arteries of the epiphysis
or in the vessels of the synovial membrane,
which is where the focus develops (primary
osteitis or primary synovitis) .
The bone foci therefore appear first on
bones and bone parts with a rich blood supply
and with terminal arteries, that are rapidly
growing and mechanically affected (meta­
physis of tubular bones, vertebrae and ossi­
cles) .
In productive granulating processes, the
tuberculous granulation tissue destroys the
surrounding bone.
In exsudative bone processes, a consider­
able proportion of the marrow undergoes
caseation, and the blood supply and nutrition
of the bone are therefore blocked.
In the event of widespread necrosis, the
necrotic detritus can dissolve, and a bone ab­
scess develops, often with sequestra. If the ab­
scess breaks through the bone, passing into
the surrounding tissues, it can cause a cold ab-
meningeal 2%
scess (abscessusfrigidus) . This can move fur­
ther from the focus, sinking by gravitational
force, so it is also called a migrating abscess
(e.g. psoas abscess).
If the abscess breaks through to the skin
surface, a fistula develops.
Clinical symptoms. The early diagnosis
of articular tuberculosis is not easy. The de­
velopment of the symptoms is latent, with no
typical signs.
The pain of the affected joint differs in in­
tensity from other sharp pains, induced by
acute bacterial inflammation. The pain in the
articulations of the lower limb intensifies dur­
ing walking or weight-bearing, whereas in an
inactive position it weakens or disappears .
The patient easily becomes fatigued, suffers
from night sweats, and is permanently subfeb­
rile. The erythrocyte sedimentation rate is
slightly elevated, but only in very rare cases
does this reach the level of acute inflamma­
tion. The differential blood count indicates
relative lymphocytosis.
If the bone destruction is severe and the
treatment is delayed, the pus can break
through the articular cavity, forming ab­
scesses in soft tissues at some distance from
the focus. The fistula can be superinfected by
banal pathogens.
A microscopic smear, histological exami­
nation, culturing and animal inoculation facil­
itate an early diagnosis. These examinations
 2 0 . B a ct e r i a l i n fect i o n s of b o n e s a n d j o i n t s
should be performed if possible before the
start of medicinal treatment, though the toxic
condition of the patient may necessitate im­
mediate antibiotic treatment.
The presence of acid-fast rods in the smear, a
positive animal inoculation test and the histo­
logical demonstration of Langhans cel ls help
clarify the diagnosis.
The tuberculin test is not as important as in
the era before BCG vaccination. An organism
that has recovered from an earlier infection
can even react hyperergically in the tuberculin
test. The skin reaction in the tuberculin test of
an infected organism rather indicates the ac­
tivity of the process or the result of the treat­
ment.
X-ray signs. The X-ray signs are varied
and change with the pathomorphologic alter­
ations. They are not always pathognostic, not
even in advanced cases. The earliest X-ray
sign is bone atrophy. This is a result of the
toxin effect and inactivity. Prognostically,
progressing bone atrophy is an unfavorable
sign. As the process advances, the articular
cavity can shrink, but if effusion is present it
can expand too. The articular surface becomes
rough, and in some places the subchondral
bone may even crack, which suggests exten­
sive destruction of the articular cartilage.
X-ray signs of articular deformities and se­
vere bone destruction may be observed. Cal­
cified abscess shadows can be seen at some
sites distant from the bone.
Reductions in the level of atrophy and of
the local sclerotizing changes, and ossifica­
tion of the joint are radiological signs of heal­
ing. This is bony ankylosis of the articulation.
If combined treatment with anti tuberculo­
sis inhibitors, and focus cleaning-surgery is
carried out sufficiently early, the articular sur­
faces are not seriously damaged, and may re­
main intact, especially in processes limited to
synovial primarily the knee-joints.
Thus, the articulation can heal with a good
function. However, even in these cases, the
development of arthrosis few years later
should be reckoned with.
� 18 1
Treatment. In an early stage, the treat­
ment of the disease is primarily medicinal.
The drugs most frequently used today are
Rifampicin, INH, (isonicotinic acid hydra­
zide) PZA (pyrazin amide), Ethambutol,
Streptomycin and PAS (para-amino salicyl
acid) . Drug treatment is always carried out
with a combination, of 2-3 medicines or in a
serious case even four.
Drug resistance may develop during
chronic treatment, which necessitates drug
sensitivity examinations on the discharges
and the pathogens from time to time.
Drug treatment alone is not always suffi­
cient to eliminate a bone focus, but it is an es­
sential addition to conservative or surgical
treatment. If the general condition of the pa­
tient rules out surgical intervention, long-term
drug treatment and conservative therapy are
recommended. However the safe way to solve
abscess and sequestral bone processes is to
combine medicinal therapy with focus­
cleaning surgery.
Sanatorium care in special institutes re­
mains important with the provision of
heliotherapy dietetic treatment and climato­
therapy.
Immobilization, rest, and freedom from
stress must be continuous . Immobilization of
the affected limb during the treatment of
osteoarticular inflammatory processes has
been regularly used since the observations of
Jimos Balassa. The pain can be decreased and
severe deformities can be avoided with plaster
casts and traction. Appropriate weight- bear­
ing prevention can be achieved with walk­
ing -caliper treatment. In Hungary, the use of a
sciatic-tuber supporting walking-caliper was
introduced by Gyula Dollinger. If the pain in­
creases at any stage of the treatment, immobi­
lization must be ensured.
The goal of the therapy is to achieve the
maximum, possible painless movement, or
perfect ankylosis in a proper position. Move­
ment limited to 1 5-20° indicates permanent
pain and can induce the relapse of the process.
The process can not be considered healed if an
abscess or sequestrum is present, since these
are frequent causes of relapse. The most im-
182 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i nts
portant goal is the permanent eradication of
all possible sources of relapse by radical sur­
gery, focus cleaning, joint resection and verte­
bral focus cleaning. Abscesses and fistulae are
accompanying signs of an osteoarticular
a
b
Fig. 20.11.
a: Caries trochanterica and coxitis tubercu losa on the
left side. Because of the specific destruction of the
g reater trochanter, a large, protruding cold abscess
has developed in the area of the trochanter major
and the gluteal region.
b: The X-ray picture demonstrates that the a bscess
and sequestral process of the trochanter has broken
into the joint, destroying it.
tuberculotic process, and must be treated to­
gether with the bone focus. The connection
between the fistula and the bone focus can be
proved by fistulography.
The role of surgical procedures in the
treatment of osteoarticular tuberculosis is in­
creasmg.
The widening of the indication of intra­
focal interventions is due to the availability of
antituberculotics. However it is a general
principle that active osteoarticular tuberculo­
sis demands a major surgical intervention
only if the condition is life-threatening (sep­
sis, an abscess or a sequestral process, and the
danger of development of paraplegia) as long
as the process is not demarcated. In such a
case, massive (a combination of 4 drugs)
antituberculotic drug treatment should be car­
ried out to prevent the danger of tuberculosis
dispersion (meningitis, pleuritis and wound
suppuration).
20.3. 1. Coxitis tubercu losa
<tuberculosis of the hip)
Clinical symptoms. The first clinical
symptoms of coxitis are limping, articular
pain and limitation of movement.
The obturatory irritation caused by the hip
process provokes pain that radiates to the knee
joint. In such a case, the clinical picture can
give rise to the suspicion, of a knee joint dis­
ease. The first sign of the process breaking
into the articulation is the restriction of
hyperextension and rotation.
The hip is initially in abduction and in
flexion - external rotation, but later ad­
duction, flexion and an internally rotated posi­
tion are observed.
The patient feels that the limb is shorter.
The hip area may be warm, the skin and the
underneath layers pasty. This latter sign oc­
curs especially in trochanter caries situated
close to the surface, with swelling and fluctu­
ation (Fig. 20. 1 1 . a, b).
If the hip process is not treated in time, it
can break into the pelvis or the soft parts. The
concomitant abscesses can cause the thigh to
thicken.
 2 0 . B a ct e r i a l i n fect i o n s of b o n e s a n d j o i n t s
Children often cry out while sleeping, be­
cause rel axation of the muscles leads to relax­
ation of forced position and the return of the
pam.
X-ray signs. Comparative films reveal
bone atrophy around the hip on the affected
side. The articular cavity shrinks, and the
bone atrophy later spreads to the femur. Foci
can appear in the bones forming the articula­
tion. X-ray and CT scans demonstrate the de­
struction. When the cartilage and the
subchondral bone too are destroyed, radiolog­
ical recognition is not difficult. As concerns
the differential diagnosis, coxarthrosis, septic
coxitis and Perthes-disease should be consid­
ered. In such cases, the fever course, the labo­
ratory examinations, bacteriological findings
and the X-ray features can be points of refer­
ence.
Treatment. If the focus is situated in the
greater trochanter (caries trochanteric a ), and
has not yet broken into the joint, a combina­
tion of inhibitors and focus-cleaning tro­
chanter resection may solve the process. In
this way, there is a hope that the movements
of the hip can be preserved.
After the operation, medicinal treatment,
immobilization and traction therapy are sug­
gested. After careful exercises, the patient can
Fig. 20.12.
Condition after left hip-joint resection, arthrodesis
and subtrochanteric osteotomy for coxitis
tuberculosa.
� 183
walk with crutches, without loading. In the ac­
tive state of inflammatory processes, physio­
therapy can be harmful.
If the focus has broken into the j oint, good
results can be expected only from surgery. Af­
ter exposure synovectomy, cleaning of the fo­
cus may enhance the blood supply and attain­
ment of the necessary concentration of the
medicines in the j oint. If the articular surface
of the acetabulum remains intact, and the pa­
tient receives inhibitors for an appropriate
time, the range of motion (ROM) of the artic­
ulation may well remain sufficient. As men­
tioned earlier, the development of osteo­
arthritis can cause problems later.
From the aspect of the aftercare, it is im­
portant for the patient to stay in bed, until
movements improve, the pain disappears and
the process subsides. As the X-ray signs re­
gress later than the clinical symptoms, deci­
sions must be based on the latter indicators.
When an intra-articular process, causes
severe destruction, fistulae and painful atro­
phy, h ip joint arthrodesis should be per­
formed (Fig. 20.1 2.).
20.3.2 Gonitis tuberculosa
(tuberculosis of the knee-joint)
The leading symptoms are the swelling of
the joint, flexion contracture and pain. In
terms of frequency, it is second after coxitis,
but it has a more benign form.
Clinical symptoms. Pain, during weight­
bearing and because of the inflammation of
the capsule, limping and flex ion contracture
are typical. The patient walks on tiptoe.
In the primary synovial form, inflamma­
tion of the articular capsule is one of the main
signs; some articular effusion is typical. Be­
cause of the thickening of the capsule and the
femoral muscle atrophy, the knee j oint swells
in a spindle form, and is slightly warm to the
touch. A temporary, brief improvement and a
decrease in pain during rest are typical. If
there is a primary para-articular focus, sec­
ondary articular effusion can develop, with a
184 � 2 0 . B a cte r i a l i nfect i o n s of b o n e s a n d j o i nts
negative Koch culture result. If the destruc­
tion increases, the caseous mass can break
into the articulation. In untreated or mis­
treated cases, besides the serious knee-j oint
contracture, the articulation may be destroyed
and a secondary axial deformity (genu
valgum or genu varum) may result. There is
also the danger of the development of a mi­
grating abscess or fistula.
X-ray signs. In the initial stages, osteopo­
rosis can be observed. The para-articular
osseal foci are visible in the X-ray or eT scan.
Later, the valgus or varus position causes ar­
ticular surface damages especially in areas of
greater loading. On the tibia and the femur,
very large foci are to be found. The X-ray pic­
ture in the late stage can resemble that in se­
vere arthrosis. Under favorable circum­
stances, osseal ankylosis can develop in a
proper position.
An early synovial form, recognized in
time and treated properly, can be healed with
the maintenance of moving articulation.
Differential diagnosis. It may be neces­
sary to consider rheumatoid arthritis,
monarthritis and arthrosis.
Treatment. In the early stages of the dis­
ease, when the process has not yet advanced
into the articulation and only the synovial
membrane is affected, healing with a moving
j oint may be hoped for. Medicinal treatment,
rest and plaster cast fixation of the joint with
are important. If such conservative treatment
is unsuccessful, the inflammatory parts
should be removed by subtotal synovectomy.
If the cartilage is intact, and no necrosis can be
observed on the articular surface, ex­
cochleation of the bone foci may be effective.
 Mi kl6s Szendr6i

21. Tumors of the musculos keletal system

21.1. General patients to specialist centers, where the so­

Musculoskeletal tumors are benign or ma­
lignant tumors of the bones and soft-tissues.
These tumors are quite different from the
other tumors of the body and are relatively un­
common: bone tumors account for 0 .5-1 %,
soft-tissue tumors for 1.5-3 % of all malignant
tumors . Bone tumors are often highly malig­
nant and occur at an early age, they change or
destroy the structure of the bone, and accord­
ingly they show up as characteristic changes
in the X-ray films.
The chances for the limb to be saved and
for the patient to survive depend on the early
discovery of the tumor. Considering the rare
occurrence, it is advisable to refer suspected
phisticated diagnostic tools, the necessary ex­
perience and the conditions of interdisciplin­
ary treatment (chemo- and radiotherapy, bone
bank and tumor endoprostheses, etc.) are
available.
Clinical signs. The maj ority of patients
seek medical help because of a palpable tu­
mor, swelling, pain or the restriction of mo­
tion. It is not uncommon that particularly in
cases of tumor-like changes, benign tumors
(e.g. enchondroma), and malignant tumors in
early stages are discovered by chance, e.g. in
X-ray films recorded because of trauma. It is
essential to decide if the symptoms are caused
by the trauma sustained or by the tumor. A

age (yes)
70 +---
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0 Cl> 0 0 -0 m :::T

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o
(J) 3 3 Cl () - '"
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--
"'< '<. (J} Cl> (J)-

!'e. (3 '"
0. ,<
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a.
=.: � �
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(J)

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Fig. 21.1.
Age distribution of primary bone tumors, tumor-like lesions
186 � 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l system

proportion of soft-tissue tumors and the ma­
j ority of bone tumors appears in the first two
decades of life (Fig. 2 1 . 1 .), and the patients
often relate their complaints to sports injuries
and traumas. In cases involving unexpectedly
long-lasting and increasing symptoms follow­
ing a trauma, tumor is to be considered and
X-ray tests must be performed.
The period of the complaints is dependent
on the rate of growth of the tumor and may
vary extremely wide. In cases of a rapidly ex­
panding Ewing 's sarcoma or a highly malig­
nant central osteosarcoma it is often a few
weeks or months, but in cases with less malig­
nant chondrosarcomas it can be many years.
The benign tumors and turnor-like lesions
usually grow slowly.
Malignant soft-tissue tumors may remain
undetected for a long time, especially if they
are localized deep down, covered by muscles.
In these cases, dull pain is the only symptom,
which does not disappear even at rest. Those
tumors that penetrate the superficial fascia are
detected earlier, they cause large swelling, the
skin is warmer above them and the venous
network is expressed.
The most significant symptoms of bone tu­
mors are also swelling, a warmer skin and
pain. The latter does not depend on the load, in
the beginning it is intermittent and pricking,
later radiating, constant pain is perceived.
Both benign as well as malignant tumors in
the vicinity of joints may induce a restriction
of j oint motion and reactive synovitis with
j oint fluid collection. If a soft-tissue or bone
tumor expanding from the bone involves ves­
sels, the significant symptom is radiating pain
and edema of the limb.
Pathologic fractures may occur as the first
symptoms in cases of benign tumors (e.g.
enchondroma) or turnor-like lesions (e.g. ju­
venile bone cyst), which may have been pres­
ent for a long time and extenuated the cortex.
The fracture arises spontaneously or in re­
sponse to a minimal trauma. Malignant bone
tumors, bone destructive soft-tissue tumors
usually start with pain.
X-ray and other imaging procedures. In
the diagnosis of soft-tissue tumors X-ray tests
are of modest help. Only the fat-tissue tumors
(lipomas, liposarcomas) can be differentiated
as they are more radiolucent than the sur­
rounding muscle and bone tissue.
If a primary tumor is discovered, antero
posterior (ap) and lateral chest X-ray is man­
datory to detect possible lung secondaries.
As bone tumors grow, they change the
shape and the structure of the bone, producing
typical (but not absolutely specific) X-ray
changes, so X-ray therefore plays a decisive
role in the preoperative diagnosis and treat­
ment planning.
Benign bone turnors usually grow slowly
and there is time of the shape of the bone to
change, and for a demarcation reaction to de-

Benign _______Malignant
/'
� Benign:
1: the cortical bone is protruded and thin
2: sclerotic rim, well-defined border between normal
bone and lesion

1=:
Malignant:

\\ \'(
3. (od man's triangle
2 I
I 4: "onion-peel" lamellar periosteal reaction
5: il-defined border between tumor and normal bone
6: destructed cortical bone
- "
- 8

Fig. 2 1.2.
I
I
I
I
: i,
I
1(
X -ray features of benign and malignant bone tumors
�
7: invasion into the soft tissue
8: spicula formation
 2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l system
velop. The border o f the intact and affected
bone tissue is usually well-defined, and the le­
sion is often surrounded by a sclerotic margin
(non-ossifying bone fibroma, osteoid
osteoma) (Fig. 2 1 .2.). Occasionally the bone
appears "inflated" (e.g. enchondroma in pha­
langes of the fingers) (see Fig. 2 1 . 1 5.).
With malignant tumors, the border be­
tween intact and affected bone tissue is vague
il-defined, the sclerotic margin is missing. If
the bone shows fusiform widening (e.g.
chondrosarcoma), this is a sign of slow expan­
sion. Nevertheless the cortex and also the
Fig. 21.3.
a: A- P film of the right pelvis, with a lmost invisible
changes the iliac bone is slightly more radiodense
b: The a image shows a tumor originating from the
iliac bone, interspersed with calcifications invading
both the gluteus muscles and the pelvic soft-tissues
� 187
neighboring soft-tissues are soon infiltrated.
There is often a periosteal reaction, such as
spiculum formation in osteosarcomas (Fig.
2 1 . 1 2.),laminated "onion peel" reaction in
Ewing 's sarcoma (Fig. 2 1 .2 1 .) .
Indirect signs of extraosseal invasion o f
the tumor are shown o n traditional X-ray
films, as calcified foci deposited in a hyaline
matrix (Fig. 2 1 .3. b), or spiculum fOrmation
in osteosarcomas.
It is important that the penetration of cer­
tain types of tumors (giant cell tumor of bone,
metastasis, bone fibrosarcoma) into the cortex
is never accompanied by a periosteal reaction.
The availability of modem non-invasive
imaging modalities, such as CT and MR,
angiography and arteriography have lost
much of their significance, but they are still
useful diagnostic or therapeutic tools in cases
of soft-tissue tumors and expansive,
infiltrative bone tumors. Angiography can
provide information on the extent, blood sup­
ply of a tumor, its relation to important ves­
sels, displacement or invasion of vessels (Fig.
2 1 .4. a). Arteriography may be supplemented
by therapeutic interventions. Cytostatic drugs
can be inj ected intra-arterially in high concen­
tration, directly into the tumor, or the supply­
ing vessels can be embolized. In this way, the
tumor temporarily shrinks, its removal is eas­
ier and the bleeding can be reduced to a great
degree.
Computer tomography (CT) is useful di­
agnostic procedure mainly in cases of bone tu­
mors, and less for soft-tissue sarcomas.
Tomographies are done in a horizontal plane,
and even a very small bone destruction, intra­
and endosteal propagation can be demon­
strated. CT is mandatory in tumors of the
shoulder girdle, pelvis and spine, where single
bones, bone structures are proj ected in tradi­
tional X-rays to cover each other, and thus the
localization of a tumor can not be accurate
(Fig. 2 1 .3. a, b). The most significant method
is the MR (magnetic resonance) imaging,
which is based on a non ionizing radiation
source, and is therefore harmless to the organ­
ism. It is excellent for visualization of the
intramedullary and of bone tumors, and their
188 � 2 1 . T u m o rs of t h e m u s c u l o s k e l eta l system
intraosseal extent, metastases (skip meta­
stases) (Fig. 2 1 .4. b). Since it is less sensitive
than CT depicting possible cortex break­
through by the tumor, it is often applied in
combination with CT.
Common isotope test used for bone tumors
is the 99 Tc-polyphosphate bone scan.
Pathologically high osteoblast activity is
indicated by a high uptake of the isotope (Fig.
Fig. 2 1.4.
a. Angiography: hypovascularized periosteal
osteosarcoma dorsally on the d istal metaphysis of
the femur, d islocating the a rtery
b. Sagittal MR image of the same periosteal
osteosarcoma: the tumor has broken through the fe­
mur cortex in the area marked with arrows and has
spread into the intramedullary cavity
2 1 .5.). The test is extraordinarily sensitive,
but less specific : Apart from tumors, in­
creased activity can be induced by degenera­
tive, traumatic or inflammatory changes. Its
main indication is the early detection of multi­
plex bone tumors or metastases and observa­
tion of the effectivity of chemotherapy. Fol­
lowing effective preoperative chemotherapy
the isotope uptake of the turn or decreases. Iso­
tope tests are of little value in the diagnosis of
soft-tissue tumors .
In the recent years ultrasound investiga­
tions developed considerably and became
more sensitive. They play no role in bone tu-
Fig. 2 1.S.
Bone scan: apart from the growth plates, there is
high isotope uptake shown in the proximal meta­
diaphysis of the right femur (Ewing's-sarcoma).
 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system
Fig. 21.6.
Ultrasonografic image: the base of the picture is the
femoral cortex, seen as a white line, above it, a re­
current chondrosarcoma is seen in the vastus mus­
cle, the calcifications in the tumor su bstance are visi­
ble as white spots
mor cases, since bone tissue "blocks" out
intraosseal processes. With soft-tissue tu­
mors, it is an essential non-invasive and al­
ways repeatable examination method, the size
of the tumor can be identified in three dimen­
sions, and the tumor can be differentiated
from fluid-containing cysts (Fig. 2 1 .6.). A
connected use is the ultrasound guided needle
biopsy taking from soft-tissue tumors.
Laboratory investigations. Bone tumors
do not initially cause bone metabolism
changes, which can be detected by laboratory
tests. An early sign is the increased sedimen­
tation rate, but this is not specific. The serum
alkalic phosphatase level is characteristically
high in osteosarcomas (it is excreted by the tu­
mor cells), its decreased level proves the effi­
cacy of chemotherapy, whereas a repeated in-
� 189
crease may be indicative of recurrence. This
level is also high in disseminated cancer
metastases, when high quantities of calcium
and phosphorus dissolve in the serum from
the diluted bone tissue and the ion level may
be elevated. The acid phosphatase level is in­
creased in metastases of prostate cancer,
while in cases of neuroblastoma the excretion
of vanillic-amygdalic acid and homovanillic
acid are considered characteristic. Important
diagnostic indicator in myeloma patients are
the appearance of Bence-Jones protein in the
urine, and increased levels of serum proteins.
Paraproteins are generally detected by im­
mune-electrophoresis. Sternal puncture can
be performed to detect affected plasmocytes,
however a normal bone marrow does not ex­
clude this condition.
Biopsy, histological test. Regarding to the
diversity and the relative rarity of bone and
soft-tissue tumors, which hampers a differen­
tial diagnosis, establishment of the exact diag­
nosis and surgical planning, almost always re­
quire biopsy and histological examinations.
Amputation is contraindicated without a
histological opinion !
In cases of soft-tissue tumors aspiration
cytology is possible, the specimen is obtained
by needle biopsy.
In the overwhelming maj ority of bone tu­
mor cases open surgical biopsy is performed,
with adherence to strict rules. The scar should
be positioned so that the surgeon might re­
move it during the definitive surgery (there is
a risk of recurrence in the scar) .
Regarding the numerous differential diag­
nostic problems close cooperation and con­
sultation is essential between the pathologist,
radiologist and surgeon.
Treatment. The variety of surgical inter­
ventions depends on a number of factors for
both soft-tissue and bone lesions, the age and
general condition of the patient, the localiza­
tion of tumor, and particularly the surgical
stage of the tumor.
Latent, active and aggressive forms of be­
nign tumors are differentiated. A latent tumor
190 � 2 1 . T u m o rs of t h e m u s c u l o s ke l e t a l system

usually does not cause clinical signs, it ap­ 2 . The relation of the tumors t o their ana­
pears coincidentally, it does not grow, or only tomic compartment, intracompartmental
very slowly, and occasionally it may heal forms (T l ) and extracompartmental forms
spontaneously. Examples include lipoma, that broke through (T2).
osteoma, etc. An active benign tumor grows 3. Absent (MO) or apparent metastases (M I )
slowly, does not extend from its anatomic at the time o f discovery.
sheath, has its own capsule. Examples are
enchondroma, chondromyxoid bone fibroma. The above 3 factors have the highest prog­
Aggressive benign tumors can expand gradu­ nostic value, so there are significant differ­
ally, or rapidly, emerging from their anatomic ences between the surgical stages regarding
casing (fascia border, periosteum), but usu­ the 5 year survival rate. The radicality of the
ally do not metastasize (or very rarely). Ex­ surgical intervention should be chosen in ac­
amples are desmoids and giant cell bone tu­ cordance with this. Group I contains tumors
mor. of low malignancy (e.g. chondrosarcoma,
The surgical classification of malignant fibrosarcoma, differentiated liposarcoma);
bone and soft-tissue tumors is based on three If A: tumors have not broken out from the
considerations (Table 2 1 . 1 .) : compartment (e.g. intraosseal chondrosar­
coma), or lIB : have broken out from the com­
1 . The level o f malignancy o f the tumor (low partment (e.g. soft-tissue infiltrative chondro­
malignancy: G 1 , high malignancy: G2 sarcoma). Group 11 includes lesions with high
malignancy (osteosarcoma, Ewing 's-sar­
coma, malignant fibrous histiocytoma, etc.),
which (IlIA stage) are intracompartmental, or
Table 21.1. ,
(IIIB stage) have already broken out from
Surgical classification of benign and malignant
tumors (Enneking)
their compartment. Group III includes those
tumors of low or high malignancy, where the
Classification by GO = benign prognosis is very poor, since distant meta­
histological g rade G1 low malignancy
=
stases are already present at the time of diag­
G2 = high malignancy nOSIS.
Classification by TO = intracapsular Beside modem therapy (chemo-, ra­
localization T1 = i ntracompartmental diotherapy) complete removal of the tumor is
T2 = extracompartmental essential . Intralesional curettage or marginal
resection is permitted only for benign lumps,
Classification by MO = no metastasis since tumor cells may be left behind. In cases
metastasis M1 regional or distant
=
of malignant tumors, wide (the incision is di­
Benign tumors rected in a few centimeters deep in the healthy
tissue) or radical resection must be performed
1. latent GO TO MO
or if the tumor has already invaded the ves­
2. active GO T1 MO sels, amputation or exarticulation is to be per­
3. aggressive GO T1-T2 MO formed (Fig. 2 1 .7.). Now that effective che­
motherapy is available, limb-saving opera­
Malignant tumors tions are done in more than half of the bone tu­
LA G1 T1 MO mor cases, resection of tumor being followed
I. B G1 T2 MO by reconstruction of the bone defect. The
bone defect, which is usually large and in­
11. A G2 T1 MO
cludes a j oint, can be replaced with the pa­
11. B G2 T2 MO
tient' s own bone ( autograft), or bone from a
Ill. A G l-2 T1 M1 bone bank ( homograft), or by modular tumor
Ill. B G l-2 T2 M1 endoprosthesis system.
 2 1 . T u m o r s of t h e m u s c u l o s ke l et a l syst e m � 191

;��:=======:=::i��H"---':I-=--=--=-=
25
20
15 30 12
1 10
5 l5j) 19 27 32
0
1985 1995 1998 2000

o a m putation
2
o l i m b- saving o peration s
'\\\---/---- 3

�\'-0JIf---/--- 4
Fig. 2 1.S.
Changes in the proportions of amputations and limb-saving pro­
cedures performed in the Dep. of Orthop. of the Semmelweis
University

Bone cavities are filled with homologous

Fig. 21.7.
Type of surgical procedures according their
radicalities. 1: radical intervention: amputation,
exarticu lation, 2: wide resection, 3:marginal excision,
4: intralesional (cu rettage of the defect)
or autologous ground bone, or bone cement
(Fig. 5.8. a, b).
If the tumor has destroyed the j oint sur­
face, the joint must be replaced or the defect
may be bridged over by a bone plate to stiffen
it (resection arthrodesis). This has an advan­
tage that, after the bony union, the limb will be
stable and can bear weight, the disadvantages
are the stiff joint and the poor quality of life.

100
-- li mb-saving o perations
90 - - - - - - a m putation

80

70
�'"
60

� 50
ro
>
.�::J
40
'" 30

20

10

0
0 20 40 60 80 100 120 140 160

time (mounth)
Fig. 21.9.
Survival rates after amputations and limb-saving proced ures in patients with osteosarcoma (Dep of Orthop.
Semmelweis Univ.)
192 � 2 1 . T u m o rs of t h e m u s c u l o s k e l eta l syst e m
The defect can be replaced b y a n osteo­
articular graft from bone a bank, but integra­
tion is slow (6 months- l year), and both early
and late complications are common (ligament
insufficiency, bone collapse, fatigue frac­
tures, etc.). More recently the use of tumor
endoprostheses has become popular, they
have the advantage that the moving joint pro­
vides good function, but the prosthesis may
work loose after some years and the bone de­
fect will then be even larger.
Chemotherapy. The overwhelming ma­
j ority of bone tumors were earlier considered
as chemotherapy-resistant. In the most recent
decades it became clear that besides Ewing 's
sarcoma and central osteosarcoma, which lat­
ter were thought to be resistant are indeed sen­
sitive to adriamycin and to high doses of
methotrexate. As a result of the introduction
of pre- and postoperative chemotherapy the
numbers of lung metastases and local recur­
rences have diminished and limb-saving oper­
ations have become possible even in cases of
osteosarcomas, that have broken through the
bone (Fig. 2 1 .8.). The survival rates are not
significantly worse than in the amputation
group (Fig. 2 1 .9.), and the 5 years survival
rate has elevated from 20% to 60-70%.
Chemotherapy is also being used in the
treatment of soft-tissue tumors increasingly
frequently, however the results are not so
clearly beneficial as in osteosarcoma. The
maj ority of soft-tissue sarcomas are moder­
ately sensitive to chemotherapy, and a tempo­
rary remission or an increased survival rate
can be achieved through the use of very ag­
gressive protocols. Chemotherapy can be
used as palliative treatment in cases of lung
metastases.
Radiotherapy. Bone tumors are generally
radiotherapy resistant, except Ewing 's sar­
coma, where irradiation of the aimed bone
may be effective to support chemotherapy and
surgical therapy.
At one time the maj ority of soft-tissue tu­
mors were considered to be radiotherapy-re­
sistant. Recently however, the available so-
phisticated radiation sources (linear accelera­
tors, high energy gamma-ray beams) permits
the attainment of a greater deep dose that is
better focused with a milder skin reaction.
In soft-tissue tumors radiotherapy is used
as an adjunct of surgical treatment preopera­
tively, to decrease the volume of the tumor
and postoperatively, to expose the tumor bed,
to inhibit local recurrence, specially following
margin�l resections. In some forms oftumors,
irradiation of the regional lymph glands may
be needed.
2 1.2. Primary bone tumors
2 1.2.1. Histological classification of
bone tumors
This classification, based on histogenetic
aspects, was prepared by a working party of
the WHO (2002), it is generally approved and
it has an advantage of an internationally stan­
dardized approach (Table 2 1 -2.).
2 1 .2.2. Bone forming (osteogenic)
tumors
Table 2 1.2.
Histological classification of bone tumors
I. Bone-forming tumors
A) benign
1. osteoma
2. osteoid osteoma
3. osteoblastoma
B) malignant
1.
conventional osteosarcoma
(central, high-malignant)
2. central, low-malignant osteosarcoma
3. parosteal Ouxtacortical) osteosarcoma
4. periosteal osteosarcoma
5. su perficial, high-malignant
osteosarcoma
6. teleangiectatic osteosarcoma
7. small cell osteosarcoma
8. secondary osteosarcoma
 2 1 . T u m o rs of t h e m u sc u l o s ke l eta l syst e m � 193

11. Cartilage-forming tumors IX. Lipogenic tumors

A) benign A) benign
1. osteochondroma lipoma
2. chondroma (enchondroma, periosteal B) malignant
chondroma) liposarcoma
3. chondroblastoma
4. chondromyxoid fibroma
X. Neural tumors
8) malignant Neurilemmoma
1. chondrosarcoma XI. Notochordal tumor
(primary and secondary) chordoma
2. juxtacortical (peripheral)
chond rosarcoma
XII. Other tumors
1. adamantinoma
3. dedifferentiated chondrosarcoma
2. metastatic tumors
4. mesenchymal chondrosarcoma
5. clear cell chond rosarcoma Tumor-like bone lesions
1. solitary bone cyst
Ill. Giant cell tumor of bone (osteoclastoma)
2. aneurysmal bone cyst
1. giant cell tumor (intermediate)
3. juxtacortical bone cyst (intraosseal
2. malignant giant cell tumor
ganglion)
IV. Bone marrow (hematopoietic) tumors 4. metaphyseal fibrous defect
1. plasma cell myeloma(solitary, multiple) (non-ossifying bone fibroma)
2. malignant lymphoma (reticulum cell 5. eosinophilic granuloma
sarcoma) 6. fibrous dysplasia
IVIA Ewing's sarcoma I primitive 7. "myositis ossificans "
neuroectodermal tumor 8. hyperparathyroidism-induced
Ewing's sarcoma family "brown tumor"
9. intraosseal epidermoid cyst
V. Vascular tumors
A) benign
hemangioma
2 1.2.2. 1 . Benign bone forming tu mors
B) malignant
angiosarcoma � Osteoid osteoma
VI. Fibrohistiocytic tumors
A) benign This is a benign, osteoblastic lesion, with a
characteristic small size (ordinarily less than 1
benign fibrous histiocytoma
cm), and reactive sclerosis surrounding the
central radiolucent "nidus" (Fig. 2 1 . 10. a, b).
B) malignant
malignant fibrous histiocytoma
The thickened hard cortex contains the nidus
VII. Connective tissue (fibrogenic) tumors as a soft redd ish-gray a rea.
A) benign
desmoplastic fibroma It is not uncommon, about 1 0 % of the be­
B) malignant nign bone tumors. The tumorous origin of
fibrosarcoma osteoid osteoma is not uniformly approved,
VIII. Smooth muscle tumors some authors regard it as a reactive lesion.
A) benign
Symptoms. The disease starts usually at
the age of 1 0-25 years. The special kind of
leiomyoma
pain is the leading symptom, it is severe, often
B) malignant
occurs in the night and reacts well to salicy­
leiomyosarcoma
lates (salicylic test).
194 � 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syst e m
About 1 0% are intracapsular, i n these
cases a j oint-effusion, muscle spasm, cont­
racture are often present. Vertebral osteoid
osteoma may result in an antalgic posture, and
scoliosis. Femur and tibia are the most com­
mon sites of its occurrences.
X-ray signs. The center of an osteoid
osteoma is a radiolucent area 0,5-2 cm in di­
ameter ("nidus"), surrounded almost always
by reactive sclerosis (Fig. 2 1 . 1 0. a, b). The
nidus is often invisible due to the enhanced re- '
Fig. 21.10.
a: A 16 year-old boy: osteoid osteoma in the
metaphysis of the right femur
b: a image: the intramedullary localization of the
nidus and the surrounding sclerotic margin is clearly
visible
active sclerosis on regular films, in these
cases a CT scan is advised. A bone scan shows
a highly positive isotope uptake.
Treatment. The aim is the total removal
ofthe nidus. If this fails, the process may recur
and the pain may persist. Surgical possibili­
ties: curettage or resection ofthe nidus. On the
surface of the resected specimen the nidus is
seen as a soft bluish spot. Very recently CT
-guided drilling or radio-frequency thermo­
coagulation has been introduced.
� Osteoblastoma
This is a benign lesion, that is histologically
equivalent to osteoid osteoma, but this is
larger, exceeding 2 cm in the large tubular
bones, and the sclerotic margin is usually
missing or much less prominent.
Symptoms. This tumor occurs under the
age of 20 years, it is much less common than
osteoid osteoma. It is localized to the verte­
bral arches, long tubular bones, ribs, pelvis.
The symptoms depend on the localization: the
forms in the vertebral arches may cause neu­
rological complications, while osteoblasto­
mas in long tubular bones may be symptom­
less for a long time.
X-ray signs. The X-ray image of osteo­
blastoma is not very distinctive. It generally
causes extensive osteolytic destruction. The
sclerotic margin is usually much less marked
than in osteoid osteoma, occasionally it is
mlssmg.
Treatment. Osteoblastoma is one of the
tumors that display active expansion. The
choice of treatment should be curettage and
filling with cancellous graft, or en-bloc exci­
sion in normal tissue.
2 1.2.2.2. Malignant bone forming
(osteogenic) tu mors
� Osteosarcoma
This is a malignant tumor, distinguished by
the direct bone mass and osteoid production
 2 1 . T u m o r s of t h e m u sc u l o s ke l eta l system
9%
-t---- 8 %
-f--rf.""'--- 5 %
-+--'-liHH/--- 5 %
f_�-+------ 32 %
�¥�----- L5 %
, vasive tumor excrete osteoid in the soft­
-tt---- 3 %
Fig. 21.11.
Incidence of central osteosarcoma
of the tumor cells. Osteosarcoma is the most
common primary malignant bone tumor,
comprising 25-30% of all bone sarcomas.
� 195
It appears in many forms, its histological
picture can be very variable.
The conventional central osteosarcoma is
most common, and is of greatest importance.
In 60% of the cases, it appears in the first 2 de­
cades of life. It may involve any bone, but in
about 50% of the cases it occurs around the
knee, in the distal femoral or proximal tibial
metaphysis (Fig. 2 1 . 1 1 .) .
Symptoms. These are initially rather in­
significant: intermittent pain sets in, which
later becomes persistent. The swelling ex­
pands rapidly, the skin is warmer, swollen
subcutaneous veins appear. In an advanced
stage pathologic fracture follows in app. 1 0%
of cases.
X-ray signs. Because of its fast progres­
sion, the osteosarcoma has usually broken
through the cortex and infiltrated the
soft-tissues even on the first X-ray film. Less
frequently in forms producing a high volume
of tumorous osteoid tissue, the involved bone
is sclerotic, very radiodense (Fig. 2 1 . 1 2 . a, b).
Fibroblastic, teleangiectatic or anaplastic
osteosarcomas, that show hardly any bone
production, induce osteolytic destruction.
Most frequently mixed lytic -sclerotic forms
occur. The border between the intact and in­
volved tissue is il-defined, with a "moth­
eaten" appearance. The growth plate, and the
periosteum inhibit the expansion of the tumor
somewhat. Similarly, the tumor destroys the
joint cartilage and invades the joint only
rarely. The pathological osteoblasts of the in-
tissues, this appearing as spiculum creation.
eT and MR tests provide great help in as­
sessing the soft-tissue and intramedullary
penetration (Fig. 2 1 . 1 2 . b, c).
Pathology. The material of the tumor is
determined by its osteoid content and by its
calcification. Its texture may therefore vary
from soft up to bone-hard.
The cut surface often demonstrate visible
bleedings, necrosis and cyst-like changes. At
a distance of few cm from the tumor, inde­
pendent tumor islands, the so called "skip"
metastases, are occasionally visible even
macroscopically.
196 � 2 1 . T u m o rs o f t h e m u s c u l o s ke l et a l syst e m

Fig. 2 1. 12.
a: Sclerotic osteosarcoma invading the soft-tissue
with spicula formation, located in the distal
metaphysis of the femur
b: a image showing the tumor invading to the joint
in the level of patella
c: MR image shows the soft-tissue extent of the
osteosarcoma
d: Histology of the osteoblastic osteosarcoma
e: Limb-saving operation after resection of this
osteosarcoma and implantation of a tumor prosthe­
sis (antero-posterior and lateral X-rays).
 2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system � 197

The histological structure o f the tumor is
variable, and often causes differential diag­
nostic difficulties. Osteoblastic (Fig. 2 1 . 1 2 .
d), chondroblastic, fibroblastic, giant cell­
loaded, teleangiectatic, small cell and mixed
forms are distinguished, depending on the dif­
ferentiation of the primitive connective tissue
mesenchymal cells. Unfortunately, between
osteosarcomas with a different tissue struc­
tures, the prognostic difference is negligible.
Treatment. Central osteosarcoma is ra­
diotherapy-resistant, and accordingly chemo­
therapy and surgery are to be attempted.
Cytostatic therapy is used both pre- and post­
operatively. Preoperative (neoadjuvant) che­
motherapy decreases the chances of the
spread of tumor cell and metastasis formation
during biopsy, the tumor shrinks, ("ripens"),
and can be resected more easily.
Thanks to the effective chemotherapy, the
5-year survival rate increased considerably, to
60-75 % (Fig. 2 1 .9.), and in more than half of
the cases limb-saving procedures are success­
ful (Fig. 2 1 .8.).
Some decades ago radical amputation pro­
cedures were almost always preferred, but
nevertheless the 5 year survival rate did not
exceed 20%. Today, surgical treatment, with
wide removal of the tumor in the intact tissues
is recommended, following preoperative che­
motherapy. Regarding metaphyseal location
of the tumor the involved bone segment is re­
moved together with the j oint surface . Bone
defects may be replaced by the patients own
bone, or by bone from a bone bank, most often
by tumor endoprostheses (Fig. 2 1 . 1 2. e).

Table 21.3.
Other rare osteosarcomas
Diagnosis Age Most frequent X-ray appearance Malignancy Therapy 5 year
(years) Localization survival
rate

Parosteal 20-60 Femur, popliteal Intensely radiodense Grade 1 surgical 90%

osteosarcoma area, humerus, tumor on the bone
tibia surface
Periosteal 15-30 Distal femur, tibia Blu rred, slightly Grade 2 surgical 70%
osteosarcoma (meta-diaphyseal) mineralized tumor on (chemotherapy
the bone surface ?)
Central 15-45 Distal femur, I ntramedullary Grade 1 surgical 80-95%
osteosarcoma, proximal tibia lytic-sclerotic lesion,
low malignancy usually with a marked

[-
border
-

Superficial, 10-30 Distal femur, Poorly mineralized Grade 3 chemotherapy 55-65%

highly malignant proximal tibia, superficial tumor, surgical
osteosarcoma humerus spicula formation
Teleangiectatic 10-25 Distal femur, Lytic destruction with Grade 3 Chemotherapy 65-75%
osteosarcoma proximal tibia blu rred border surgical
metaphysis
Secondary 30-60 Metaphysis of Lytic-sclerotic Grade 3 Chemotherapy 30-40%
osteosarcoma long tubu les destruction with surgical
bones blurred border
(+ basic disease Paget's
disease, fibrous
dysplasia, bone infarcts,
postirradiation, etc.)
198 � 2 1 . T u m o rs of t h e m u sc u l o s ke l e t a l syste m

a: Parosteal osteosarcoma
growing dorsally on the distal
femoral metaphysis
b: Operative specimen cut in
half: it is clear, that the cortex
is intact
c: a images showing that the
lump is situated on the outer
surface of the bone, has not
broken through the cortex.

� Parosteal osteosarcoma (j uxtacortical � Periosteal osteosarcoma

osteosarcoma)
This is an osteosarcoma localized on the
surface ofthe bone, from the parosteal tissues,
with relatively better prognosis (Fig. 2 1 . 1 3 . a,
c).
This is located on the outer surface of the
bone, in the periosteum, and usually in the
diaphysis (Fig. 2 1 . 1 4.).
 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
Fig. 21.14.
Periosteal osteosarcoma on the surface of the tibia,
the surgical specimen has been cut in half with a saw
21.2.3. Cartilage-forming tumors
2 1 .2.3. 1. Benign cartilage-forming
tu mors
� Enchondroma
This is a benign tumor containing mature
hyaline tissue. It is common, accounting for
20% of all benign bone tumors. It occurs in
d h
the 2n _4t decades, primarily involves the
phalanges and the metacarpals of the hand.
Symptoms. It does not necessarily cause
symptoms, and is often observed with un­
changed size for many years or expands only
slowly. Stabbing pain may sometimes occur,
or pathological fracture may be the first sign.
The X-ray sign is specific, well delineated
destruction is visible centrally in the meta­
diaphysis of the metacarpals, which inflates
the bone without a periosteal reaction (Fig.
2 1 . 1 5.).
� 199
Fig. 2 1. 15.
Lytic destruction in the base and mid-phalanx of the
index finger inflating the bone -enchondroma
Pathology. Elevation of the cortical plate
reveals a grey-white, debris-like material that
fills up the medullary cavity. Micro­
scopically, this tumor is built up from hyaline
cartilage, its structure is lobular. Occasionally
it is difficult to differentiate the less malignant
chondrosarcomas from the benign ones. One
should not rely merely on the histology, but
also consider the X-ray, the location and the
clinical picture. It is essential to know that
chondrosarcoma is very rare in the short tubu­
lar bones of the hand. It can present in a multi­
ple form as a systemic bone disease. In such
cases, the risk of malignant transformation is
higher, at 1 0%.
Treatment. Excochleation and cavity fill­
ing the with autologous cancellous bone. In
the active stage lesions may recur.
About 0,5 - 1 % of solitary enchondromas
produce secondary chondrosarcomas.
200 � 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l s y s t e m
� Osteochondroma (exostosis
cartilaginea)
This is a bony lump covered with a hyaline
cap, which p resents from the surface of the
bone.
Osteochondroma is rather a hamartoma,
than a real bone tumor, it is a cartilage bud that
"migrates" out from the growth plate. It is
quite common, giving 5 0-60% of all benign
lumps. In 1 0% of the cases it is multiple and
shows a familial accumulation, it is classified
among the bone dysplasias (Chapter l 3 . 1 .7.).
A solitary osteochondroma is usually di­
agnosed in the first decade, involving the
metaphyses of long tubular bones. Distal fem­
oral, proximal tibial and proximal humeral
metaphyses predominate. The osteochondr­
oma grows as long as the bones continue to
grow. Rapid expansion with fast progression
in adults is always a suspicious sign of malig­
nant transformation.
Symptoms. At the beginning, there are al­
most no symptoms, the osteochondroma is
discovered as an accidental finding. During its
expansion it may compress the surrounding
nerves and vessels, tendons snapping over the
a b
Fig. 21.16.
a: Ca uliflower-shaped osteochondroma in the proximal metaphysis of the fibula
b: 3 dimension reconstruction a i mage of this osteochondroma
osteochondroma may also be sources of com­
plaints .
X-ray signs. Thin pedunculated or
wide-based sessile forms are typical (Fig.
2 1 . 1 6, a, b). The peduncle bends in the direc­
tion of the diaphysis as a result ofthe dragging
force of the neighboring muscles, resulting in
the X-ray finding of a typical "hat-rack"
shape. The size varies from a few cm up to 1 0
cm.
Pathology. Macroscopically, the cortex of
the bone continues in the shaft of the
osteochondroma, and the end is widened as a
butt, this area is covered by the hyaline cap. In
the cartilage-bone border an ordinary
enchondral ossification zone is visible.
Treatment. The chiseling of osteo­
chondromas from their base ensures a perfect
outcome. There may be a rare recurrence if
parts of the cartilage or the covering connec­
tive tissue capsule are left behind.
In less than I % of the cases, malignant
transformation may occur in the substance of
hyaline cartilage cap. The frequency is much
 2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l syst e m
Fig. 21.17.
a: Lateral X-ray of the knee: round lytic destructions
(arrows) in the epiphysis - chondroblastoma.
b: MR image: the extent of the chondroblastoma
can be well estimated.
� 20 1
higher, (ca. 5 - 1 0%) in multiple osteo­
chondromas, in multiple cartilaginous exo­
stosis. These patients must be thoroughly fol­
lowed up, and control X-rays are to be done. A
sudden enlargement following bone matura­
tion is usually a sign of a malignant transfor­
mation, these should be excised immediately.
� Chondroblastoma
This is a rare benign tumor located in the
epiphysis, next to the joint.
Symptoms. It appears chiefly on the tibia,
femur and humerus of boys before the end of
growth. The early symptom is swelling of the
limb with a restricted range of motion of the
neighboring j oint. Later a joint collection may
follow with reactive synovitis.
X-ray signs. These are very specific : oval
or rounded lytic bone destruction in the epiph­
ysis, usually centrally with a sharp, occasion­
ally sclerotic border (Fig. 2 1 . 1 7. a, b).
Treatment. The most common procedure
is intralesional excochleation and autologous
cancellous bone grafting.
� Chondromyxoid bone fibroma
This rare benign tumor elicits hardly any
symptoms . It presents in the second decade in
the metaphyses of the long tubular bones. The
X-ray image shows an eccentric lytic lesion
with an uneven sclerotic margin, which may
occasionally lead to bone protrusion.
Treatment. Excochleation, autologous or
homologous cancellous bone grafting.
21.2.3.2. Malignant cartilage-forming
tumors
� Chondrosarcoma
This is a malignant bone tumor containing
hyaline cartilage cells in various stages of mat­
uration and a matrix.
There are many groups of chondro­
sarcomas, which differ in histological appear-
 202 � 2 1 . T u m o rs of t h e m u sc u l o s ke l eta l system
s u rvival among

1,0 1 - -1- - - - - - - - - - - - - - - - - - - - - - - - - - - - - - - -� __q,�_5..

0,9

0,8

0,7

0,6
5%
0,5
\. J 11 %
0,4

-':;'<;4\ I I
0,3
11 %
0,2
0,10
0,1

0,0
° 12 24 36 48 60

grade 1 chond rosa rco ma �» 11 1\

m o u nth 23 %

___ gra de 1 1 + 1 1 1 c h o n d rosarcoma

Fig. 21.18. ( � 12%

Survival among chondrosarcoma patients
1%

ance. It is important to be familiar with them,

because biological behavior of a given tissue
with the prognosis of the illness correlates L 10 %
closely (Fig. 2 1 . 1 8.).
The chondrosarcoma is the second most
5%
common malignant bone tumor after the
osteosarcoma. Males are affected slightly
more often than females. Onset occurs pri­
marily in the 3_6th decade. It may be present
in any bone, but it mainly appears on the prox­
imal part of the femur, pelvis, humerus, scap­
ula, less often the ribs and tibia, very rarely in 3%
the bones of the wrist and hind foot (Fig.
2 1 . 1 9.).
Symptoms. Well differentiated chondro­
sarcomas grow slowly. Patients often observe
limb swelling during many years.
The less-differentiated forms break Fig. 21.19.
through the cortex early, and invade the soft­ Incidence of primary chondrosarcoma
tissue, and metastasize in the lung (mesen­
chymal and highly-malignant chondro­
sarcomas). 2 1 .20.). Calcification often develops in the
X-ray signs. Intraosseal central chondro­ substance of the tumor, giving a fine spotty, in
sarcoma is a process which swells the bone other cases a massive radiodense X-ray im­
and causes lobular, lytic destruction (Fig. age.
 2 1 . Tu m o rs of t h e m u s c u l o s ke l eta l syst e m
Fig. 21.20.
a: Chondrosarcoma destroying the proximal
epi-metaphyis of the right humerus
b: Surgical specimen of this chond rosarcoma - blu­
ish-gray transparent cartilage tumor, shining like
mother of pearl
c: Low malignancy chondrosarcoma: histological pic­
ture
The soft-tissue element of chondro­
sarcoma is generally lobular, the calcified
spots clearly show the extent of the turnor
(Fig. 2 1 .3. a, b). Peripheral chondrosarcomas
do not initially involve the medullar cavity,
only in a later stage.
The well-differentiated chondrosarcomas
are hypovascular, the less-differentiated ones
are hypervascular.
Pathology. Classical chondrosarcoma is a
macroscopically grayish-white or blu­
ish-white, transparent, solid tumor, with a car­
tilaginous rigidity and a "mother-of-pearl"
appearance (Fig. 2 1 .20. b, and c)
Features of rare chondrosarcomas are
listed in Table 2 1 .4.
The treatment of chondrosarcomas.
Low malignancy chondrosarcomas (Grade I .
� 203
•
chondrosarcoma, light cell chondrosarcoma,
secondary chondrosarcomas) are radiother­
apy- and chemotherapy-resistant tumors.
They grow characteristically slowly, meta­
stasize very rarely in the lung, the 5 -years sur­
vival is over 90%. Their treatment is chiefly
surgical, depending on their location and ex­
tent. On radical or possibly wide resection, the
lump should be removed with an intact mus­
cle envelope. Rarely, in an advanced stage,
only amputation may be performed, however
limb saving is very often possible.
In cases of Grade H, IH chondrosarcomas,
mesenchymal and dedifferentiated chondro­
sarcomas, the lump (which is usually quite ex­
tensive when it is first observed) can be re­
moved only by amputation to provide proper
radicality. In these cases, surgical treatment is
204 � 2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system

Table 2 1.4.
Chondrosarcomas
Diagnosis Age Most common X-ray appearance Malignancy Therapy 5 year
(years) localization survival
rate

Central 20-70 pelvis, proximal lytic-sclerotic 60% Grade 1-2: 90%

chond rosarcoma femur, humerus, tu mor, blu rred Grade 1, surgical
tibia, ribs border, calcification 40% Grade 3: 30-40%
Grade 2-3 surgical,
chemotherapy?
Mesenchymal 15-40 pelvis, rib, skull, lytic-sclerotic tu mor, Grade 3 surgical 30-50%
chondrosarcoma femur calcification chemotherapy?
Dedifferentiated 35-75 proximal femur, sclerotic-lytic Grade 3 surgical 30%
chond rosarcoma humerus, pelvis destruction, blurred chemotherapy?
and scapula border, calcification
Clear-cell 20-70 femur, humerus lytic-sclerotic Grade 1-2 surgical 90%
chondrosarcoma epiphysis, pelvis, destruction, sharp
rib border, calcification
Juxtacortical 20-70 femur, humerus, Hardly mineralized Grade 1-3 surgical Grade 1:
(periosteal) tibia superficial bone tu- chemotherapy? 80-90%
chondrosarcoma mor, spicu la, Grade 3:
calcification 30%
Secondary, 20-55 Short tubular lytic destruction, Grade 1-2 surgical 75-95%
central bones, femur, blurred border,
chondrosarcoma humerus, tibia calcification
Secondary, 20-55 metaphysis of calcified tumor in Grade 1-2 surgical 80-95%
peripheral long tubular soft-tissues + solitary
chondrosarcoma bones, scapu la, / mu ltiplex exostosis
pelvis

be supplemented by irradiation of the regional
lymph glands. Unfortunately, in spite of this
the 5-years survival is quite low, between
30-35%.
2 1 .2.4. Bone marrow tumors
• Ewing-sarcoma / PNET
This is a malignant tumor, which presents at
a n early age (5-25 years). Ewing's sarcoma and
primitive neuroectodermal tumor (PNET) fam­
i ly include round cel l sarcomas, which exhibit
various extent of neuroectodermal differenti­
ation. In 85% of the cases they show special
chromosome translocations (t 1 1;22) and
(q24;q 12).
The Ewing' s sarcoma / PNET family are
the 3rd most common primary bone sarcomas.
The localized form involves a single bone, but
not infrequently it is disseminated, can be de­
tected in other bones by needle biopsy and
spreads to the lungs. This cases are to re­
garded as systemic.
Symptoms. The swelling, pain, which are
insignificant at the onset, progress rapidly, are
soon accompanied by fever and leukocytosis.
This condition can therefore be confused with
acute osteomyelitis.
The duration of the complaints is on aver­
age 3-6 months. The lump spreads rapidly
from the intramedullary space to subperiostal
area, and soon elevates the periosteum.
 2 1 . T u m o rs of t h e m u s c u l o s k e l e t a l syst e m � 205

X-ray signs. Ewing' s sarcoma / PNET ap­

pear in the diaphysis, dia- metaphysis of long
tubular bones. They most often involve the fe­
mur, tibia and humerus, pelvic occurrence is
also frequent. Originally there is an almost in­
visible fme spotty radiolucency on the
diaphysis (Fig. 2 1 . 2 1 . a) . Later, the radio­
lucent areas link up, creating a larger region of
spotty destruction. This is accompanied by a
special lamellar, onion peel-shaped periosteal
reaction. The Codman triangle is common at
the edges.
The extent of the tumor in either intra­
medullary or extraskeletal dimensions, is de­
tected by eT and MR tests.
Pathology. The tumor comprises a gray­
ish-white, soft substance which exhibits
bleedings, necroses. The substance of the in­
volved bone is necrotized, the cortex is soft­
ened or has disappeared. Microscopically, it
contains uniform tumor cells lined up closely
side by side with round or oval nucleus (Fig.
2 1 . 2 1 . b).
Treatment. Ewing' s sarcoma is a radio­
and chemotherapy-sensitive tumor. The algo­
rithm of the treatment following the biopsy
and diagnosis is as follows: 1 . preoperative
chemotherapy, 2. irradiation of the tumor (to­
tal dose is 40-60 Gy), 3 . radical surgical inter­
vention and 4. postoperative chemotherapy.
In axial locations (pelvis, spine, etc.) radio­
and chemotherapy without surgery is consid­
ered (survival is however less favourable).
Some decades ago the 5 years survival rate of
patients with Ewing-sarcoma was 5- 1 0% .
With combination of radiotherapy, surgical
and chemotherapy, this proportion increased
to 50-70%.
a: Ewing's sarcoma in the proximal metaphysis of
A new modality is the separation of stem
the fibula, typical onion-peel type periosteal reac­
cells after the removal of the tumor, then total tion
body irradiation and return of the stem cells . b: Ewing's sarcoma: histological picture
Unfortunately, i n cases oflung metastases and
local recurrences, the prognosis is still poor.
volved. In contrast with Ewing' s sarcoma a
� Primary bone lymphoma long history, and the relatively good condition
Lymphoma rarely occurs in the primary of the patient with no complaints are consid­
form in bone, it may be seen at any age, but ered typical, even in cases oflarge tumors. Of­
most frequently in middle age. Most often the ten a pathologic fracture is the first symptom.
pelvis, femur, tibia, humerus and ribs are in- The X-ray picture reveals extensive bone de-
206 � 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system

struction in the dia-metaphyses of long tubu­ around 3 0-50%. In cases of impending or

lar bones, often without a periosteal reaction.
Earlier, in cases of bone lymphomas the cho­
sen procedure was always amputation, but
nowadays limb saving is coming into the fore­
ground, following local irradiation, surgical
procedures and chemotherapy. Histologi­
cally, this bone lymphoma is similar to the
lymphomas occurring in other lymphatic or­
gans.
pathologic fractures, palliative osteosynthesis
is carried out (intramedullary nail, plate, etc.)
to ease the pain, and provide independence,
chance of ambulation for the patient.
2 1 .2.5. Giant cell tumor of bone.
(osteoclastoma, "brown tumor of
bones")

� Multiple myeloma
According to recent research this intermediate
Multiple myeloma (multiple plasmocyt­ tumor evolves from the medullar stam-cells,
oma, myeloma multiplex) is generally classi­ osteoblast precursor cel ls. Mainly occurs in the
fied among malignant hematological dis­ relatively young in the 2 nd _4th decade. The
eases. This tumor elicits focal destructions in epi-metaphyses of long tubular bones are
the bones, and the patients therefore often usually involved, most often the distal femo­
seek the help of an orthopedist, traumatologist ral, proximal tibial, proximal humeral, distal ra­
with their first symptoms. The treatment of dial epi(meta)physis, and less often occurs in
solitary forms and pathologic fractures of the sacrum, pelvis.
multiple forms requires surgical intervention.
Symptoms. Theses usually present after
the age of 40, often in the vertebrae, ribs,
skull, pelvis and sternum. The illness often
starts with a long period of no clinical symp­
toms, or vague back pain, which points to a
spinal manifestation. In severe cases, the ver­
tebrae may be compressed, paralysis, par­
esthesia may be present. The immune system
of the patients is usually impaired, banal in­
fections are frequent. The sedimentation rate
is usually very high (over 1 00 mm / hour), se­
rum electrophoresis proves monogamma­
pathy and the bone marrow gained by sternal
puncture contains a very large amount of
pathologic plasma cells, confirming the diag­
nosis. The pathologic protein also appears in
the urine (Bence Jones protein) .
X-ray signs. Typical rounded, lytic bone
destructions (as they were induced by a
punch) are visible with sharp margin in the
bones of skull, vertebrae, ribs, pelvis,
Treatment. In solitary forms irradiation
and resection-replacement of the bone seg­
ment involved is to be considered. In the most
common multiple myeloma, the decisive role
played by chemotherapy. The remission Fig. 21.22.
achieved by cytostatic therapy is usually tem­ Aggressive giant cell bone tumor, spreading from
porary, and the 5-years survival rate is low, the tibia to the fibula (a-p and lateral X-ray).
 2 1 . T u m o rs of t h e m u s c u l o s ke l et a l s y s t e m
Brownish-red giant cell bone tumor in the proximal epi-metaphysis of the tibia surg ical specimen
Symptoms. The tumor usually grows rap­
idly, the patient reports vague local pain,
swelling.
X-ray signs. Giant cell bone tumors cause
exclusively lytic bone destruction. The lesion
is usually rounded or oval, eccentric, and the
cortex is thinned, with no periosteal reaction
(Fig. 2 1 .22.). It is more or less separated from
the surrounding intact bone tissue, but has no
sclerotic margin. It frequently invades the
subchondral region of the joint surfaces, con­
sequently the surfaces may collapse in re­
sponse of load bearing.
Pathology. Macroscopically, reddish­
brown, soft, occasionally fragile tumor tissue
with haemorrhages is seen in the bone defect
(Fig. 2 1 .23.) . Microscopically, the lump con­
tains a connective tissue-type fusiform cell
population, and mononuclear cells, osteo­
clast-type giant cells.
Treatment. The giant cell bone tumor is a
classical semimalignant tumor: it grows ag­
gressively, breaks through the cortex, invades
the soft-tissues, joints, and often recurs after
inadequately radical treatment. On the other
hand it very rarely metastasizes to the lung.
Recent classifications put giant cell bone tu-
� 207
mor as intermediate in biological behavior,
between the benign and malignant tumors .
This essentially means that the histological
image and biological behavior are not closely
connected. The statistical data indicate, that
80% of these tumors is benign, 1 0% are pri­
marily malignant, 5% become malignant sec­
ondarily, and 3-5% give lung metastases
(mostly through their recurrences) together
with the repeatedly benign histological pic­
ture.
Treatment is surgical, excochleation ofthe
defect, which is then filled up with bone chips
or bone cement. After this intervention, recur­
rence is not rare. Resection can also be per­
formed with intact tissue margin (if it is not
necessary to sacrifice the joint surface).
2 1.2.6. Other rare bone tumors
Adamantinoma and chordoma (the latter
appears in the line of the embryonic dorsal
chord) are classified here. Certain typical
soft-tissue tumors also occur in bones, but
very rarely. Apart from a few exceptions
(adamantinoma, chordoma) clinical symp-
208 � 2 1 . T u m o rs of t h e m u sc u l o s ke l eta l s y s t e m

I Table 21.S.1
Other rare bone tumors
Diagnosis Age Most common X-ray appearance Malignancy Therapy 5 year
(years) localization survival
rate

Benign

Hemangioma 20-60 flat bones, skull, oval lytic latent, observation,

spine destruction, "tiger active su rgical
stripes" spiculum
formation
Desmoplastic 20-40 long tubular lytic lesion, cortical active, surgical
bone fibroma bones, destruction aggressive
metaphysis
Lipoma 20-60 tubular bones, lytic lesion, latent, surgical
cuboid bones +/-sclerotic border active
Malignant

Rbrosarcoma 30-50 femur, tibia lytic-sclerotic Grade 11-111 su rgical 30-70%

(primary, metaphysis destruction, blurred
secondary) border
Malignant 20-50 femur, humerus, lytic-sclerotic Grade 11-111 surgical 60-80%
fibrous tibia destructive, blurred chemotherapy
histiocytoma border
Liposarcoma 30-50 femur, pelvis, blu rred border lytic Grade 11-111 surgical 40-60%
tibia, humerus destruction
Adamantinoma 5-30 tibia (diaphysis) Cystous, Grade I su rgical 90%
"
" honeycomb-like ,
sharp bordered
lesion
Chordoma 20-70 sacrum, cervical blurred border, lytic Grade 1-11 su rgical 60-90%
spine, skull destruction,
calcification

toms and X-ray appearance are not typical,
they are pinpointed as an accidental finding
following an injury and the final diagnosis is
established only on the basis of the histology.
Table 2 1 -5. summarizes their relevant fea­
tures.
2 1 .3. Bone metastases
Oncology management is becoming an in­
creasingly more serious task in o rthopedic
and trauma surgery. The main reason is that
the increased average lifetime increases the
number of diseases, and in the number of
patients, who have solitary or mUltiple sec­
ondaries after the treatment of their primary
tumor, but who can survive on cytostatic
treatment for years.
The significance of the treatment of bone
metastases is indicated by the fact that they
are 80- 1 00 times more common than primary
bone turnors . In about 20-40% of the cases,
the secondary is discovered first, and the pri­
mary can not be found even with a compre­
hensive search.
 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l system
After the lung and liver (primary filters),
malignant tumors metastasize most often to
the skeleton. Various tumors have very differ­
ent "bone affinities" as concerns their
Fig. 21.24.
Common localizations of bone metastases
� 209
metastases . 65-85% of bone secondaries are
given by breast, lung, kidney and prostate
cancer. The bones, most frequently involved
in decreasing sequence : lumbar, dorsal, cervi­
cal spine, ribs, proximal femur, skull, pelvis,
sternum and humerus. Tumors are very rare in
the short tubular bones of the hand and foot
(Fig. 2 1 .24.).
Symptoms. The first symptom is usually a
deep, intermittent pain that is independent of
the movement and barely moderates in re­
sponse to painkillers . Such pain often presents
weeks or months before the X-ray changes are
detected. The involved area is tender, perhaps
swollen, warmer. The case history and labora­
tory tests must be thoroughly evaluated. In
1 0-30% of the cases the first episode is a
pathologic fracture. In the background there
may be a kidney or lung cancer, giving mostly
lytic metastases, or less frequently breast
(Fig. 2 1 .25.), or thyroid cancer. Osteoplastic
metastases of prostate cancer rarely break and
have good propensity to heal (Fig. 2 1 .26.).
Pathologic fractures occur most often in the
vertebrae and load-bearing long tubular bones
metastasis with pathologic fracture i n the prox­
imal metaphysis of the humerus
2 10 � 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l system
Fig. 2 1.26.
Osteoplastic metastases of prostate cancer in the
pelvis
(the femur, tibia), less frequently in the hu­
merus.
X-ray signs. In suspected cases, e.g. when
there is local bone pain after history of turnor,
an X-ray is taken of the area in question and
eT, MR (occasionally PET) scans are added
if necessary. A bone scan is also extremely
important to decide if the process is single or
multiple. The sensitivity exceeds that of the
X-rays, the pathologic area shows an in­
creased isotope uptake months before the
X-ray changes are detected.
Fig. 21.27.
ney cancer (3D er i mage)
Vertebral destruction caused by metastases of kid­
In spine, in the opposite of inflammation
processes the tumor involves a single verte­
bral body (Fig. 2 1 .27.), invading the inter­
vertebral space only in later stage.
In the long tubular bones, the lesion may
be central, though it is more often eccentric,
involving the cortex, with a round or oval ap­
pearance. A periosteal reaction is rare. At the
time of discovery metastases are usually mul­
tiple, only in 1 5-20% are solitary. There is im­
minent danger of fracture if the level of cortex
destruction exceeds 50% or 2,5 cm.
The laboratory parameters are less specific
and may remain between the normal limits,
especially for small solitary metastases.
Differential diagnosis. Primary malig­
nant bone tumors usually present at a younger
age, there is no history of tumor, and the tu­
mor is usually solitary and accompanied by a
periosteal reaction. Similar X-ray changes are
detected in cases of primary bone lymphomas,
giant cell tumors, aneurysmal bone cysts.
Treatment. Earlier a somewhat passive
approach was favored. Today considering the
possibilities provided by modem radio- and
chemotherapy, and the marked development
that have occurred in re constructive surgery
(minimally invasive interventions, tumor
endoprostheses) surgical treatment has came
to the forefront.
Aims of the surgical treatment:
� to prevent the imminent fracture
� radical removal of the lesion if the
conditions are suitable
� in cases of fracture, reconstruction of
motion, alleviation of the pain
� to ensure independence, mobility for the
patients
� to ensure proper care and quality of life
In cases with a vertebral involvement (Fig.
2 1 .27.) in order to solve the neurological
symptoms following a compression fracture,
decompression is carried out with plate stabi­
lization.
 2 1 . T u m o r s of t h e m u s c u l o s ke l et a l syste m � 211

A) Primary tumor is known and oncologically managed S) Metastasis of unknown origin

�
X-ray, bone scan, PET-a. M R Primary tumor su rvery
I I
+ + + +
Solitary M u ltiple/mu ltio rganic Successful U nsuccessful

.. .. .. ..
Staging O ncological management
Staging Biopsy
skeletal-extra ske letal of primary tumor

.. + +
I n bone (a. MR) Imminent fracture Staging metastasis Staging skeletal-extraskeletal
(solitary, multiple, i m minent fracture
.. imminent fracture)
extra skeletal fracture
chest, abdomen a. + +
1 ..
US surgical yes no

.. .. .. Pall iative surgery

or chemotherapy
Needle biopsy Biopsy Biopsy

.. .. ..
Assessment Stag ing extraskeletal
Assessment
of general condition of pt.

1 1
of prognostIC factors

Assessment
Surgery of general condition of patient
.
. ..
c hemoth erapyI
-....." 11".at.ve
--------surgic I
Palliative

non- surglca I treatment

a Planning surglcall
Radical
a
radiothera py
.
Fig. 21.28.
Treatment algorithm in cases of suspected metastasis (before pathologic fracture)

In long tubular bones, the following proce­
dures are employed depending on the local­
ization of the fracture : intramedullary nailing,
osteosynthesis fixed with a plate and curet­
tage of the defect, or insertion of a tumor
endoprosthesis with cement fixation. Intra­
medullary nailing is advantageous, for it is
stable weight-bearing, and even if the tumor
progresses, loosening of the implant is not
likely (Fig. 3.4. a, b).
Surgical intervention can be supplemented
by palliative radiotherapy and chemotherapy,
to achieve pain relief and temporary remis­
sion. Amputation is very rarely performed for
palliative purposes, only in cases of ulcerated
tumors and unbearable pain.
Surgery is not done if the expected sur­
vival is less than \ -3 months, if the general
state ofthe patient does not permit; or in cases
of multiple metastases or multiorgan meta­
stases, if no imminent fracture is to be pre­
vented. In the latter cases palliative chemo­
therapy, radioisotope treatment (in certain
thyroid, prostate, breast cancers), and hor­
mone therapy (in prostate cancers and in
metastases of estrogen positive breast cancer)
are preferred, and bisphosphonates are pre­
scribed in lytic and mixed-type bone meta­
stases.
Prognosis. The life expectancy is good if
the primary tumor is discovered and can be
controlled oncologically, if the metastasis is
solitary, appears years after the onset of the
primary tumor, grows slowly, does not invade
to the soft-tissues and no pathologic fracture
2 12 � 2 1 . T u m o rs of t h e m u s c u l o s k e l eta l syst e m

has occurred. I n these cases (unfortunately
only 1 0-20% of all patients) curative-type
radical tumor excision is warranted, with limb
savmg.
The most sensitive prognostic factor is the
origin ofthe primary tumor. In cases of breast,
prostate, thyroid and kidney cancers, the ex­
pected survival time is much longer than in
cases of lung cancers or bone metastases of
melanoma.
The life expectancy is poor when the pri­
mary tumor is unknown or unsuitable for sur­
gery, or when the primary is discovered at the
same time as the metastases, if the metastases
are multiorganic or multiple. In these cases
surgery is palliative and the aim is to restore
the function and ambulatory capability with
the least possible intervention, and in terminal
case to ensure the chances of painless care.
2 1.4. Tumor-like bone processes
The significance of tumor-like bone pro­
cesses (Table 2 1 .6.) is, that their X-ray ap­
pearance may mimic that of malignant bone
tumors, which gives rise to differential diag­
nostic problems, since they are much more
common than the real tumors . It is necessary
to be aware of the possibility of misdiagnosis
and of an unnecessarily radical intervention.

Table 2 1.6.'
Tumor-fike bone lesions
Diagnosis Age Most common Symptoms X-ray appearance Treatment
(years) localization

Juvenile 5-20 metaphysis of usually pure lytic lesion with a sharp depot-steroid,
bone cyst the humerus, accidental or a border thin cortex, swollen eXCDchleation,
femur, tibia pathologic bone sclerotization
fracture
Aneurysmal 5-30 meta-epiphysis pain, swelling, central or eccentric growth, excochleation +
bone cyst of the long pathologic blu rred margin, deformed cancellous graft,
tubular bones fracture bone sclerotization
Non 5-25 long tubular symptom less or subcortical, sclerotic margin, in case of imminent
ossifying bones mild pain or a ..lobular" cystic thinning fracture,
bone metaphysis pathologic excochleation,
fibroma fracture cancellous bone
graft, or observation
Fibrous 10-40 meta- mild pain, blurred margin, often multiple, in cases of imminent
dysplasia diaphysis of curvature of "opaque glass" lytic defect fracture
long tubular bones, excochleation +
bones, pelvis pathologic cancellous graft
fracture
Eosino- 5-25 vertebra, long pain, a sharp border, a lytic bone excochleation + a
phyle tubular bones, eosinophilia defect, lamellar periosteal cancellous graft
granuloma Meta- reaction
(solitaer) diaphysis of
flat bones
Myositis 5-30 long tubular initially: fever initially: oval lump showing su rgical excision in
ossificans bones, over leukocytosis, ossification at its periphery, mature stage, if it is a
meta- pain, later: a centrally radiolucency; source of complaints
diaphysis in solid lump, later: a lump with uniform (considerable
the muscles contractures bone density, divided from the contracture, pain)
I
,
bone by a radiolucent layer
 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
Fig. 21.29.
Non ossifying bone fibroma: multiple appearance in
femur and tibia.
Regarding their progress tumor-like bone
processes may be latent, like non-ossifying
fibroma (Fig. 2 1 .29.), and juvenile bone cysts
are ordinarily discovered accidentally, or at­
tention is drawn to them by a pathologic frac­
ture, due to the weakened cortex. In these a
cases plaster cast is applied, which sometimes
is enough for a bony consolidation of the le­
sion. If this fails, the non-ossifying fibroma is
curetted and the cavity is filled with bone
chips. A juvenile bone cyst (Fig. 2 1 .30.) is
treated with injection of the crystalloid steroid
to induce the bony restructuring, or similarly
cancellous bone plombage is performed. Ifthe
non-ossifying fibroma is small, observation is
satisfactory with regular X-ray checks .
Local aggressive growth is observed for
aneurysmal bone cyst (Fig. 2 1 .3 1 . a, b),
� 2 13
Juvenile bone cyst (with pathologic fractu re) in hu­
merus proximal metaphysis .
which are regarded as intraosseal circulatory
disturbances. This growth causes the bone
segment to expand, weakening the cortex,
causing risk of a pathological fracture. In
about 25% of the cases, this lesion appears in
association with other primary bone tumors
(giant cell tumor, osteoblastoma, chondro­
blastoma, osteosarcoma), causing problems
in differential diagnosis.
Eosinophile granuloma appears mainly in
the meta-diaphysis of long tubular bones as a
central lytic lesion showing a thick sclerotic
periosteal reaction. It may be solitary or mul­
tiple. In the former case, curettage and
spongiosa plasty are performed. The latter
case has a significantly worse, sometimes fa­
tal prognosis, chemotherapy can be at­
tempted. Fibrous dysplasia is classified to
bone systemic diseases (Chapter l 3 .), it can
mimic a bone tumor on the X-ray picture. It is
very inconsistent as regards its extent in the
bone : it varies from round small metaphyseal
lesion to a "shepherd' s crook" shaped femur,
2 14 � 2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system

a b

a. Multilobular aneurysmal bone cyst from the sciatic bone, impressing the medial side of the acetabulum
b. Aneurysmal bone cyst destroying the d istal end of the radius (cut surface of the surgical specimen)

a b

Fig. 2 1.32.
Myosit iSOSsificans i n the form of oval calcification in the anteromedial side of the femur (ap (a) and lateral (b)
X-rays)
 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l syste m � 215

when the entire long tubular bone i s affected. 2. Connective tissue (fibroblastic,
In cases of latent, non-progressing forms a myofibroblastic) tumors
regular check-up is sufficient, in progressive A) Benign
forms, curettage, cancellous bone plombage fibroma
is indicated. A good outcome was recently re­ fasciitis nod ularis
ported following bisphosphonate treatment, myositis ossificans
which increases the strength of the surround­ B) Intermed iate (locally aggressive)
ing bone. palmar and plantar fibromatosis
Myositis ossificans rarely occurs in a pro­ desmoid-type fibromatoses
q Malignant
gressive form in infants; this progressive form
develops rapidly and has a lethal outcome.
Adult fibrosarcoma
This condition occurs in the overwhelming
Myxofibrosarcoma
majority in young adults, metaplastic ossifica­
tion develops in the soft-tissues, muscles after 3. Fibrohistiocytic tumors
a trauma or even without any injury (Fig. A) Benign
2 1 .32.). It is often difficult to differentiate fibrous histiocytoma
from malignant soft-tissue tumors or from giant cel l tumor of tendon sheaths
bone tumors involving the soft-tissues. If the B) Malignant
diagnosis is certain and the lesion is malignant fibrous histiocytoma
symptomless, removal need not be urged, as
4. Smooth muscle tumors
recurrence is common. In case of symptoms
A) Benign
or a maj or loss of function excision is advised
leiomyoma
with a wide margin.
B) Malignant
leiomyosarcoma
s.
21.5. Soft-tissue tumors
Pericyte (perivascular) tumors
Classification the soft-tissue tumors . Ac­ glomus tumor
cording to the WHO definition published in 6. Striated muscle tumors
2002, soft-tissue tumors are extra skeletal A) Benign
lumps originating from non-epithelial tissues, rhabdomyoma
except those that develop from the reticulo­
B) Malignant
endothelial system, the glia tissue and special
rhabdomyosarcoma
connective tissues of the viscera and certain
organs. Soft-tissue tumors are discussed by 7. Vascular tumors
the WHO definition on a histogenetic basis. A) Benign
The basis of the classification is the tissue hemangioma
from which the lump develops and the tissue it B) intermediate
produces. Soft-tissue tumors are relatively hemangioendothelioma
q Malignant
Hemangioendothelioma (epitheloid)
Table 21.7. Angiosarcoma
Classification of soft-tissue tu mors
8. Soft-tissue tumors originating from
(simplified version)
cartilage and bone tissue
I. Fat tissue tumors A) Benign
A) Benign soft-tissue chondroma
lipoma B) Malignant
B) Malignant extraskeletal chondro-
liposarcoma and osteosarcoma
216 � 2 1 . T u m o rs o f t h e m u s c u l o s ke l eta l system

9. Tumors of questionable or unknown their location. Tumors lying near the superfi­
etiology cial fascia are detected when they measure
A) Benign 0.5-2 cm, if they are located deep in the mus­
intramuscular myxoma cles or next to the bone, detection is possible,
B) Intermediate when they exceed 5 cm in size.
ossificans fibromyxoid tumor Unlike to the bone tumors, for the stage
q Malignant classification of soft-tissue tumors the TNM
synovial sarcoma system is used (Table 2 1 .8.), which is useful
alveolar soft-tissue sarcoma
both clinically and prognostically.
epitheloid sarcoma
This takes into account the size of the pri­
mary tumor (Tx: the size ofthe primary tumor
clear- cell sarcoma of tendons and tendon
sheaths
can not be assessed, TO : no primary tumor can
extraskeletal Ewing/PNET
be detected, T l : the primary tumor measures
less than 5 cm, T2 : the diameter of primary tu­
malignant mesenchymoma
mor is more than 5 cm, T l a: it has a superfi­
cial localization, T l b: it has a deep localiza­
tion); the histological malignancy of the tu­
rare, but many types are known; the following mor (G I : low malignant, G2 : highly malig­
table (Table 2 1 .7.) lists only the more impor­ nant tumors); to the involvement of regional
tant ones. lymph glands (Nx: can not be judged, NO: no
Malignant soft-tissue tumors comprise metastasis in regional lymph glands, N I : me­
about 1 -3 % of all malignant lumps. 60-65% tastasis in regional lymph glands); and the dis­
of these tumors are localized in the limbs, tant metastases (MO: no distant metastases,
two-thirds of them in the lower limbs. De­ M I : metastases are present).
pending on the type of the tumor, local recur­ Benign soft-tissue tumors can also be di­
rence is common, 40-60%, distant secondar­ vided into latent, active and aggressive types
ies are detected in 40-60% of the cases, (Table 2 1 .9.). Latent and active tumors may
mainly in the lung. be marginally or pericapsularly excised, as
The symptomatology of the tumor is very concerns aggressive forms (e.g. desmoid) ex­
indistinct, their detection depends mainly on cision with a wide margin must be achieved.

Table 2 1.8.
TNM classification of soft-tissue tumors
Stage Histological Tumor size Lymph gland Distant metastases
malignancy involvement

lA stage Gl Tla NO, NX MO

Gl Tlb NO, NX MO
I B stage Gl T2a NO, NX MO
Gl T2b NO, Nx MO
IIA stage G2 Tla NO, NX MO
G2 Tlb NO, NX MO
11 B stage G2 T2a NO, NX MO
III stage G2 T2b NO, NX MO
IV stage Gl/G2 Tl/T2 Nl MO
Gl/G2 Tl/T2 NO/N l Ml
 2 1 . T u m o rs of t h e m u s c u l o s ke l eta l syste m
Table 21.9.
Recurrence rate of some common benign soft-tissue tu mors
Diagnosis
Fibroma
Palmar and plantar fibromatosis (Dupuytren)
Extraabdominal fibromatosis
Rbrous histiocytoma
Lipoma
Angiolipoma
Intramuscular lipoma
Hemangioma cavernosum
Hemangioma arteriovenosum
Hemangioma venosum
Giant cell tumor of tendon sheaths (synovioma)
Morton neuroma
Neurilemmoma
Neurofibroma, solitary
Myositis ossificans
Myxoma, intramuscu lar
Regarding the malignant soft-tissue tu­
mors that commonly occur in the limbs, the
time of onset, common localization and the
histological malignancy is presented in Table
21.10.
Therapy. The therapy depends o n the
stage, the localization of the tumor, on the age
of patient, etc. It is mandatory for these rare
tumors to be managed in centers with a
multidisciplinary approach. Optimal treat­
ment must be decided in consultations involv­
ing an oncologist surgeon, a pathologist, a ra­
diotherapist, a chemotherapist and a radiolo­
gist. It is optimal, if the orthopedist surgeon is
specialized in oncological surgery, since tu­
mor resection should often be followed by re­
construction, and tendon and muscle trans­
plantation. Vessel replacements, and use of a
vascularized musculocutaneous flap to cover
� 217
Stage Recurrence rate
Latent Active Aggressive Moderate High
+ + +
+ + +
+ +
+ +
+ + +
+ +
+ + +
+ +
+ +
+ + +
+ +
+ +
+ +
+ +
+ + +
+ + +
the defect is often needed, which may necessi­
tate to involve vascular- and plastic surgeons.
Specimens for histology are obtained causing
the least possible trauma, aspiration cytology,
percutaneous core biopsy may also be appro­
priate if an experienced pathologist and spe­
cial test methods (cytogenetics, etc.) are avail­
able.
In the surgical planning, the optimal inter­
vention selected should minimize the risk of
local recurrence, besides ensuring the best
quality of life. If the tumor involves many
muscle groups, it must be excised with a deep
margin extending in the direction of intact tis­
sues, some muscles may be cut in half so as to
ensure some function. The surgical procedure
is radical, if the entire muscle is cut out from
its origin to its attachment (myectomy), when
the tumor has not broken through the fascia.
In case the tumor has reached the periosteum,
218 � 2 1 . T u m o r s o f t h e m u s c u l o s ke l et a l system

Table 21.10.
Localization and histological malignancy of some malignant soft-tissue tu mors
Malignant Age Localization Grade

11.
Diagnosis decade
I. Ill.
Fibrosarcoma 3-6 Limb, trunk, head, neck + + +
Myxo-fibrosarcoma 3-6 Lower-upper limb + + +
I nfantile fibrosarcoma 1-2 Limb d istal + +
Malignant fibrous 4-6 Lower -, upper limb, trunk + + +
histiocytoma
Liposarcoma, 3-5 Thigh, mediastinum +
well-differentiated
Liposarcoma, myxoid 2-4 Limb, thigh +
Liposarcoma, pleiomorphic 4-6 Lower, upper limb, trunk +
Leiomyosarcoma 4-6 Retroperitoneum, limb + + +
Rhabdomyosarcoma, 1-2 Head-neck, genital + +
embryonal
Rhabdomyosarcoma, alveolar 2-4 Limb, perineal, para nasal + +
Angiosarcoma 3-8 Lower limb, upper arm, neck + + +
Synovial sarcoma 2-3 Lower limb, foot, popliteal, hand + +
Extra-skeletal myxoid 4-7 Thigh, upper arm + +
chondrosarcoma
Extra-skeletal osteosarcoma 5-7 Thigh, bottom, shoulder girdle +
Alveolar soft-tissue sarcoma 2-4 Lower limb, thigh extensor side + +
Epithelioid sarcoma 2-4 Forearm, hand, fingers + + +
Clear-cell sarcoma 2-4 Limb, foot, ankle + +

it must likewise be excised, since it represents nancy. Following marginal resection of

the "capsule" of the tumor. In case the tumor
has eroded the bone cortex, or invaded the
medullary cavity, the involved bone segment
must be excised with the lump. Conditions of
limb-saving operations are similar those listed
in connection with bone tumors (Chapter
2 l . 1).
deeply seated tumors with high malignancy,
neoadjuvant / adjuvant chemotherapy and / or
radiotherapy is recommended. If the histology
of the tumor was unclear at the time of the first
surgery, and the remaining tissues were con­
taminated by tumor, wide excision of the tu­
mor bed is suggested in a second session.

Adj uvant therapy. If a soft-tissue sar­ 2 1.5. 1. Some soft-tissue tumors of

coma is superficial, it measures under 5 cm,
and the wide margin excision succeeded with
2-3 cm-thick intact muscle envelope, the sur­
gical intervention is sufficient. Chemotherapy
is not recommended for tumors of low malig-
orthopedic significance
� Connective tissue tumors
Connective tissue tumors comprise the
largest and most colorful group of soft-tissue
 2 1 . T u m o r s of t h e m u s c u l o s ke l et a l system � 219

tumors. The most common i s the fibroma clude trauma, rapid abdominal wall contrac­
durum, which measures 0,5 - 1 cm and is usu­ tion after delivery, a surgical procedure and
ally located in the subcutis, sometimes pro­ scarring, hormonal factors may also play a
truding from the skin as a pedunculated lump. role. It may occur anywhere, it is observed (in
Fasciitis nodularis is a reactive fibroblastic the sequence of decreasing frequency) : in the
tissue mass rather than a tumor. shoulder girdle, upper arm, thigh, pelvis and
It grows fast, reaches its final size, not ex­ forearm. Treatment is surgical, wide-margin
ceeding 3 cm within a few weeks . It generally excision is advised, without sacrificing the
occurs in young adults . It may be found in any vessels and nerves. Local recurrence is fre­
region in the body, in half of the cases occurs quent.
in the upper limb, mainly on the volar side of Fibrosarcoma is a malignant soft-tissue tu­
the forearm, in the superficial fascia, subcutis, mor that originates in the connective tissue
rarely in muscles. Its rapid growth and (Fig. 2 1 .34.). It may occur at any age, usually
histological picture may give rise to the suspi­
cion of malignancy, and particularly diagno­
sis of fibrosarcoma. Surgically, excision is the
solution, but if the procedure is intralesional,
it may recur.
Superficial fibromatoses are plantar and
palmar fibromatoses. The former is also
called Dupuytren contracture (see chapter 27,
Fig. 27.12.). They are sometimes both pres­
ent. Total excision is advised.
One of the deep musculo-aponeurotic
fibromatoses is the extra-abdominal desmoid
(Fig. 2 1 .33.). Early recognition is essential to
differentiate it from fibrosarcoma. The etiol­
ogy is unknown, the predisposing factors in-

Fig. 21.33. Fig. 2 1.34.

Aggressive desmoid infiltrating the left gluteus mus­ Fibrosarcoma in the middle third of the left leg,
cle, and also the femur (MRI, frontal reconstruction) showing pathologic vessel structure
220 � 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l system
h
i n the 3 _ S t decade. Usually develops in
deeper regions, next to tendons, aponeuroses,
infiltrating the deep muscles. It is better de­
fined than the desmoid, it has a pseudo­
cap sule. Histologically in a typical case the
bundles of connective tissue cells and colla­
gen fibers show "fishbone" pattern. There is a
broad scale from the differentiated forms to
anaplastic tumors, the latter display abnormal
mitoses, and tumorous giant cells are also
present. The recommended treatment in well
differentiated forms is wide resection, in less
the differentiated forms it is radical resection,
perhaps supplemented with radiotherapy.
a

b
� Fibrohistiocytic tumors
Malignant fibro-myxosarcoma is the most
common form of malignant fibrohistiocytic
tumors (Fig. 2 1 .35. a, b). This lump is pre­
sumed to originate from primitive mesen­
chymal cells, which later undergo partially
histiocytic, partially fibroblast differentiation,
and are capable of phagocytosis and for colla­
gen production. It occurs in the limbs, less of­
ten in the retroperitoneum, in the middle aged
and in elderly.
It usually grows deep between the mus­
cles, often causing cortical erosion on the
neighboring bone. It expands rapidly, with
a: MK I mage of the middle third of the thigh: malig­
typical clinical symptoms of fever, leuko­
nant fibro-myxosarcoma in the substance of the
cytosis, neutrophilia or eosinophilia. Its may rectus femoris muscle
reach S-20 cm in size. The cut surface is gray b: The same tumor after radical surgery (myectomy)
to yellow, depending on the fat content, inter­
spersed with necrotic bleeding spots . Accord­
ingly, despite radical surgery, local recur­ between 0 , S -20 cm. It is well separated from
rence rate is 40-S0%. The S years survival rate the surrounding tissues. Its cut surface is ho­
is low, at around 3 0-40%. This tumor most of­ mogenous, butter-yellow, with a fatty shine.
ten metastasizes to the lung, or to the regional Lipomas often contain other tissues: angio­
lymph glands . The therapy is mainly surgical : lipoma, pleiomorphic lipoma, angiomyo­
radical excision, often amputation. Irradiation lipoma, fusiform cell lipoma can be differenti­
of the regional lymph glands or adjuvant ated. The lipoma is usually encapsulated. Sur­
cytostatic therapy is also employed. gery comprises marginal resection.
Liposarcomas are malignant tumors of the
� Fat tissue tumors fat tissue the (Fig. 2 1 .36. a, b). They are the
The most frequent mesenchymal tumor is second most common soft-tissue tumors after
the lipoma, which contains mature fat tissue malignant fibrous histiocytoma. They occur at
and has a capsule. It may be solitary or multi­ the 4-6th decade, in deep sites adj acent to the
ple, usually superficially in the subcutis, intermuscular fascias, aponeuroses. This tu­
rarely deep between the muscles. It measures mor most commonly occur in the thigh,
 2 1 . T u m o rs of t h e m u s c u l o s k e l e t a l syste m � 221

popliteal region, and retroperitoneum. It usu­

ally grows slowly, and because of its deep lo­
cation its discovery is late . In consequence of
the variable histological picture, many subdi­
visions are differentiated, the broad scale
ranging from less malignant forms to forms of
high malignancy. For the latter, the 5 -year
survival rate is around 20%, whereas adequate
treatment of the well differentiated forms
leads to cure in 80- 8 5 % of the cases. Surgical
procedure for the less malignant forms is wide
excision, the highly malignant forms require
amputation. The effectivity of chemo- and ra­
diotherapy is questionable.

� Tumors of muscle tissue

The malignant turnor of the smooth mus­
cle tissue is the leiomyosarcoma.
a
It is a relatively rare lump, occurs in
adults, sometimes in the skin in the form of b
small nodules, more often in the deeper re­
gions . Excision with a wide margin is recom­
mended. It rarely metastasizes, but local re­
currence is frequent, prognosis is fairly good.
The malignant turnor of the skeletal mus­
cle tissue is the rhabdomyosarcoma.
This is the most common soft-tissue sar­
coma in children. Many forms are known : e . g .
embryonic, botryoid, alveolar, pleiomorphic
rhabdomyosarcoma. The embryonic form is
already present at birth, while the pleio­
h
morphic type takes place in the 3 _ 5 t decade,
chiefly in the muscles of the limbs. The lump
is usually 2- 1 0 cm in diameter, and has no
capsule, the cut surface shows bleedings,
necroses. Earlier the prognosis was very poor
because of the local recurrences and lung
metastases . Recently combined radio-chemo­
therapy and surgical treatment increased the
survival in a great extent, the 5 years a: H uge I I nrIC::Irrn nn::l
symptomless survival rate now reaching 80%. b: Cut surface of the specimen: liposarcoma inter­
spersed with fatty, shining and necrotized spots
� Vascular tissue tumors
The hemangioma is a common localized Cavernous hemangioma, which may be
form of benign vascular turnors . larger, occurs in deep tissues, often in the
Capillary hemangioma occurs predomi­ muscles. It may exhibit local destructive
nantly in the skin and subcutis, in the form of growth, which may cause complaints . Exci­
typical purple - reddish nodules, it may be sion or percutaneous sclerotization is neces­
present at birth. sary.
222 � 2 1 . T u m o rs of t h e m u s c u l o s ke l et a l system

Arteriovenous and venous hemangiomas

are rare, occurring in deeper tissues. The com­
pression of the surrounding tissues, nerves
may induce severe pain.
Intramuscular hemangioma may infiltrate
the deeper muscles, the limb is swollen, the
skin is hot. It is often large, excision with a
wide margin may be difficult. If tumor tissue
is left behind, local recurrence may arise.
The angiosarcoma is a malignant vascular
tumor. Its histological structure is de­
differentiated, it is usually impossible to de­
cide if the pathologic growth contains
a
pericytes or endothelial cells. It takes place
primarily in the head, neck, less often in the b

limb muscles. It requires a radical surgical

procedure. The prognosis of the highly malig­
nant form is poor.

� Synovial tumors
In orthopedic practice it is perhaps most
important to be familiar with tumors situated
in the synovial lining embedding the tendon
sheaths, bursae, j oint capsules.
Many synonyms are known for the benign
varieties: benign synovioma, nodular teno­
synovitis, nodular pigmented villous syno­
vitis, giant cell tumor of tendon sheaths, etc .
Their histological pictures are similar or fully
identical. Their etiology is unclear, some ex­
pert consider them to be as inflammatory,
while others regards them as genuine tumors .
The � may occur at any age, most often in the
3_5 1 decade. They appear primarily in the Fig. 21.37.
small joints ofthe hand. The patients often no­ a.: Synovial sarcoma on the left upper arm next to
tice increasing swellings on their fingers . The the elbow
lump measures 0,5-5 cm. It is covered by cap­ b.: MR image of the same lump in sagittal plane
sule. It has a lobular structure, it is ocher yel­
low on the cut surface. The lump should be
excised with its capsule, if part of the tumor is
left behind, local recurrence is frequent. cut surface exhibits necrosis, bleeding and
A malignant variety of the synovial tumors calcification. Microscopically, it can be di­
is the synovial sarcoma (Fig. 2 1 .37. a, b). It vided into two maj or groups: biphasic and
usually occurs in the 2_3rd decade, in the lower monophasic forms, which can mutually trans­
limb, the foot, the popliteal area, the thigh, form into the other form during the recur­
rarely the hand. It is closely connected to the rences. The growth rate of the lump is vari­
tendons, tendon sheaths, bursae and joint cap­ able, the patients occasionally notice the lump
sules. Macroscopically the tumor contains for months, in other cases for years, but it may
5-20 cm cysts, and has a pseudocapsule. Its then suddenly start to grow and attain consid-
 2 1 . T u m o r s of t h e m u s c u l o s ke l eta l syste m
erable size. This tumor is classified a highly
malignant sarcoma, it metastasizes early, of­
ten to the lung and to surrounding lymph
glands. The histological picture and the prog­
nosis are not closely related, the 5 years sur­
vival rate is rather low, at 25-60%. Radical
surgical intervention has recently been sup­
plemented with local radiotherapy.
� Peripheral nerve tumors
Traumatic neuroma is a tumor-like lesion,
occurring after the transsection of peripheral
nerves. In these cases a small painful nodule
may develop at the end of the proximal stump.
Similarly the Morton neuroma is not a real
tumor, but rather a fibrotizing process of the
distal nerve. It is situated most often in the
sole between the 3 rd and 4th , less often be­
tween the 2nd and 3 rd metatarsal heads . On ex­
posure, the division of the plantar nerve is
fusiform, thickened. It causes painful attacks,
which can be provoked by direct pressure.
The involved nerve segment should be ex­
cised.
Neurilemmoma (benign schwannoma) is a
real benign tumor of the peripheral nerves. It
often develops along the peripheral nerves,
grows slowly. Pain, neurological signs are ob­
served only for maj or lumps. Marginal exci­
sion is advised.
� 223
Solitary neurofibroma is also benign. It is
more common, than its multiple form, the
Recklinghausen 's neurofibromatosis. The lat­
ter often produces a malignant transforma­
tion. Solitary neurofibroma may occur at any
location in the body, usually causing no
symptoms.
Malignant schwannoma is a malignant tu­
mor of the peripheral nerves. About half of the
cases develops from Recklinghausen 's neuro­
fibromatosis. It is most common in the 2_5 th
decade. It is often located in deep tissues,
along maj or nerve trunks, closely adhering to
them. The clinical symptoms are : referring
pain, paresthesia, weakness. The cut surface
of the tumor is grayish-white, with scattered
bleedings and necroses. The histological pic­
ture is very variable. The prognosis of the sol­
itary forms is rather good, but in cases based
on neurofibromatosis the 5 -years survival rate
is less than 3 0-40% . The treatment is radical
excision or rather amputation.
Cartilage and bone producing malignant
tumors also occur in soft-tissues, chondro­
and osteosarcomas of soft-tissues. The diag­
nosis is suspected by X-ray findings and con­
firmed by histological tests. The histological
picture is the same as that for the forms occur­
ring in the bones. The treatment is radical sur­
gery.
 Zo lta n Cse r n a t o n y
22. Reflex dystrophy (Sudeck's syndrome)
Referred to in the literature as Sudeck's syn­
drome, Sudeck's reflex dystrophy, algo­
dystrophy, reflex sympathetic dystrophy,
acute post-traumatic osteoporosis or acute
focal bone atrophy, this is a regional vasomo­
tor and trophic syndrome that generally de­
velops in the limbs, causing painfu l and se­
vere functional disorders, affecting the skin,
the subcutaneous tissue, and the articular and
periarticu lar structures.
Etiopathology. The pathological basis of
this disease is dystrophy, which generally en­
sues in areas rich in nerve terminals .
The essence of the pathogenesis o f
Sudeck 's syndrome is the vicious circle of the
metabolic and trophic processes, associated
with circulatory failure induced by pain, and
with severe locomotor function disorders
(Fig. 22. 1 .).
Besides the classic example of trauma,
there may be numerous factors provoking
Sudeck ' s syndrome, though around 25% of
the cases no cause can be demonstrated (Ta­
ble 1 .) .
Evo king factor
Pain
Focal
Artriolar
bone
atrophy
spasm
Ti ssue
hypoxia
Ti ssue
acidosis
activity
Pain
Fig. 22.1.
Vicious circle of Sudeck's dystrophy
Table 22.1.
Possible causes of Sudeck's syndrome
- Pregnancy and post partum condition (hip)
- Bone fracture, frostbite, bu rns, or some other
trauma (in 8.8% of all i nju ries and in 27% a mo ng
women older than 60 years)
- Suppuration of bones, joints or soft parts
- Thrombosis
- Certain rheumatic conditions (psoriatic
arthropathy, dermatopolymyositis, lupus
erythematosus, etc.)
- Distortion of the cervical and lumbar spine.
- Central or peripheral d iseases of the nervous
system (cerebral thrombosis, epilepsy, radiculitis,
herpes zoster, polyneuropathy, hemiplegia,
cerebral tu mor, i njuries of the brain or the spinal
cord)
- Cardiopulmonary conditions (myocardial i nfarct,
pulmonary synd rome, etc.)
- Endocrine and metabolic diseases
- Lyme disease
- Psychic disorders
Iatrogenic provoking factors:
Improper immobilization
Impro perly adapted physiotherapy
Vegetative hypersensitiveness and certain psychic
disorders may be predisposing factors.
Clinical picture. The course of the clini­
cal picture is usually divided into three stages,
defined by the concomitant symptoms. Radio­
logical alterations generally appear some
weeks later, may initially be absent.
� Stage 1
This lasts from some weeks to three
months. The first sign of the illness is gener­
ally the pain, which can practically converge
226 � 2 2 . Reflex d y st r o p hy ( S u d eck's sy n d r o m e )
with that, caused b y the inducing condition,
but is disproportionately great.
Local inflammatory symptoms (edema­
tous swelling and hyperemia) are observed;
the skin is warm and dry, and often becomes
hairy. The patient has no fever. Pressure, heat,
movements, and emotional stress can increase
the pain.
� Stage 2
In some cases, the disease begins directly
with stage 2. It lasts for three to six months,
during which vasoconstriction predominates,
possibly leading to dystrophic symptoms. The
skin is cold, moist and livid. Contractures can
develop. In the X-ray picture (Fig. 22.2.) it
can be observed that the focal atrophy of the
bones at the certain area turns into diffuse os­
teoporosis at the end of the stage, the condi­
tion of the cortical bone remaining unaltered.
� Stage 3
This final stage, may last for two or more
years from the onset. Severe pain may also be
experienced in this stage. More or less perma­
nent tissue changes can occur: the skin of the
Fig. 22.2.
Anteroposterior picture of the hands: typical focal striped atrophy in the short tubular bones of the carpus
and the hand.
limb is shiny, silk-like, thin and pale, and the
subcutaneous tissues are atrophic.
Clinically, the limitation of articular mo­
tion is the outstanding feature. Radiolo­
gically, the symptoms of osteoporosis are ac­
companied by fibrous bones, due to the thick­
ening and parallel positioning of the
trabeculae.
Treatment. Apart from the specific ther­
apy of the basic disease, the treatment of the
developed algodystrophy depends on the
stage.
� Stage 1
The goal is to ease the pain and the symp­
toms of inflammation. The limb must be put
in a resting position, or if necessary in a plas­
ter cast in a neutral position, immobilized,
cooled, and treated with antiphlogistics.
Non-steroid anti-inflammatory drugs can
be administered as additional medication, to­
gether with calcitonin, and alpha- and
beta-blocking drugs.
In more severe cases, steroid can be given
systemically for 1 -2 weeks.
 2 2 . Reflex dyst ro p hy ( S u d e c k's s y n d r o m e)
A further possibility is sympathetic block­
ade, with paravertebral or ganglion stellatum
lidocaine infiltration, depending on the local­
ization.
Physiotherapy is significant, but it should
not induce pain.
If the lower limbs are affected, partial
weight-bearing with crutches for some weeks,
and before getting up, the putting-on of flexi­
ble stockings is suggested.
Underwater exercises and alternating
bathing, edema draining massage can also be
applied.
� Stage 2
There is only a need for a resting position
if the patient still suffers from pain.
Physiotherapy is still necessary by the
same principles. Careful warming of the cold
limb is suggested.
� 227
� Stage 3
In this stage the developed contractures
are relieved conservatively, with active and
passive orthoses. If there is no other way, then
an operation is required.
Prevention of the disease is much easier
than its treatment.
Since Sudeck 's syndrome can evolve in
the event of short-term immobilization, strict
control of the patient and careful physiother­
apy are necessary.
The therapy is mostly effective in stage I ,
and at the beginning of the stage 2 , but from
the end of stage 2, there is a need for
long-term rehabilitation, because of the prob­
lems caused by permanent deformities.
 Arpad Bel lyei

23. Aseptic bone necrosis

23. 1. Childhood bone necrosis

ijuvenile osteochondrosis, juvenile
osteochondritis, juvenile
osteochondronecrosis)

Bone necrosis generally refers to disorders

which start in child hood. These disorders,
which have a similar histological appearance,
affect the epi- metaphysis, the apophysis,
maybe even the accessory bones d u ring
growth. These illnesses can occur at any site
where enchondral ossification is taking p lace.

Currently more than 50 types of aseptic

bone necrosis are known and depending on
the localization and grade they can cause dif­
ferent complaints; however, regardless of the
localization, all of these disorders are
histologically the same. The more common
ones are presented in Fig. 23- 1 . Initially bone
and marrow cells necrotize in the affected
area. This is followed by a revascularization
process. The well-vascularized connective
tissue gradually invade between the bone
trabeculae, it dissolves the necrotized bone
trabeculae and new bone formation com­
mences. The process is a slow one, and the de­
velopment of a normal structure takes months
Fig. 23.1.
or even years . Resorption of the dead bone
Frequent localizations of osteochondrosis
segments and the creation of new bone are 1: Sternal epiphysis of clavicu le
usually a parallel processes (creeping substi­ 2: Proximal epiphysis of humerus
tution) . Due to the breakdown of the balance 3: Vertebra plana (Calve)
between decaying and building, the bone in 4: M. Scheuermann's disease
question may undergo distortion if exposed to 5: Os lunatum
even a normal load. 6: Femoral head (M. Perthes)
7: Patella
Because of the histological alterations the
8: Apex patellae
term ' aseptic osteochondrosis ' appears opti­ 9: Tuberositas tibiae (Schlatter-Osgood sy.)
mal since the cartilaginous area also takes part 10. Apophysitis calcanei
later in the process. The word "osteo­ 11: Os nacicu lare (K6hler I.)
chondritis" used in the English medical litera- 12: II-IV. metatarsal head (K6hler 11.)
230 � 2 3 . A s e p t i c bo n e n e c r o s i s
ture does not correspond with the pathological
events since no inflammation occurs. The ex­
pression osteochondrosis not only implies the
absence of any basic inflammatory event, but
also indicates the occurrence of alterations
leading to degenerative disorders .
The attribute "juvenile" expresses the fact
that this type of disease appears in childhood,
during development. The forms occurring in
adulthood, such as os lunatum malacia and id­
iopathic necrosis of the hip and knee joints,
are completely different in appearance and
course from juvenile osteochondrosis. In the
case of Blount 's disease (tibia vara epi­
physarea), there a failure of ossification of the
tibia in the epiphysis as well as in the
metaphysis.
Etiology. The cause of aseptic bone necro­
sis is not clear. According to Weis and
Lindemann theories claiming that all such ill­
nesses have a mutual, definite cause are un­
founded. The many views can be condensed
into 3 hypotheses.
� The mechanical theory. Many believe that
the primary cause is a mechanical­
dynamic overload, involving excessive
pressure or traction, while others claim
that the explanation lies within mutations
caused by repeated trauma and micro­
trauma. Even though the partial role of
mechanical factors cannot be ruled out, in
themselves they do not provide an
adequate answer.
� The vascular theory postulates that the
illness is caused by a primary vein-supply
disorder (thromboembolic mechanism) or
external compression of the nutritional
supply artery. The cause of the local
circulatory disorder is still unknown. It is
important to emphasize that the artery
supply in the region of the epiphysis and
the apophysis remains unstable till they
connect to the meta-diaphyseal artery
system during the closing of the
developing cartilage.
� Constitutional theory. Many suspect that
hereditary ossification disorders, or a
hormone dysfunction are to blame. The
most accepted theory is that of
Idelberger 's, who discusses genetically
determined hypoplasia and hypo­
vascularization of the skeletal system. It is
important to note that it is not osteo­
chondrosis itself that is inherited, but only
the disposition.
There are also different opinions regarding
the categorization of sterile necrosis. Accord­
ing to Harbin and Zollinger consider that
forms emerging during the first decade com­
prise primary necrosis, which should be dis­
tinguished from the secondary forms develop­
ing during the second decade. Hirsch 's defini­
tion includes local and generalized forms.
Gofflabels deformities created by pressure
(e.g. Perthes 's, Scheuermann 's, Kijhler 's 1.
diseases) as real, while deformities generated
by traction force as unreal necrosis (cal­
caneus, patella, trochanter, and sciatic bone
apophysis).
This is not a perfect distinction and ignores
the fact that the maj ority of osteochondroses
only have a mild effect and do not always lead
to any consequences.
Frequency. In childhood and adolescence
sterile necrosis and associated pain syn­
dromes occur quite frequently, and have been
increasingly observed in the orthopedic prac­
tice recently. This may be explained by the
following factors :
� Acceleration: the continuous increase in
body height. This is accompanied by an
increase in the power of the arms, the
forces at the origin and attachment of the
various muscles and ligaments therefore
also increasing.
� The spreading of mass sport, which leads
to higher pressure on the developing and
cartilaginous ossification centers.
� The demands imposed by professional
sport and the lowering of the age limits in
competitive sport. Sport frequently
becomes an existential factor even in
childhood, and year round pain syndromes

occurring at this stage cause serious
problems for child, parent and doctor
alike. The other extreme is when osteo­
chondritis and related pain syndromes
with no medical consequences lead to the
child being excused permanently from all
forms of physical education (PE), even
though this could be avoided by the use of
brief activities coupled with non-steroid
medication.
� Osteochondritis in childhood and adol­
escence is responsible for most of the
cases of polypragmasia in orthopedic
practice. Polypragmasia not only leads to
the unfounded exemption from PE class,
but also the incorrect mental treatment of
chronic pain syndromes, with the parents
tending to go from one doctor to another.
The most frequent forms of osteo­
chondrosis observed today are the following :
- distal apex patellae syndrome,
- proximal patella (main bone nucleus)
syndrome,
- Schlatter-Osgood 's disease,
- Osteochondritis spina iliaca anterior-
inferior,
- Osteochondritis spina iliaca posterior-
superior,
- Scheuermann ' s disease,
- Osteochondritis dissecans gen.,
- Perthes 's disease,
- Kohler ' I-I!. disease,
- Calcaneal apophysitis (Schinz 's
disease) .
X-ray signs. In these diseases the X-ray
appearance can be divided into 3 stages:
1. The sclerotic stage. The affected bone
nucleus becomes sclerotic, smaller than
normal and shrunk, leading to the decaying
of the ossifying center.
2. The fragmentation stage. Soft foci appear
within the sclerotic bone nucleus and the
shape of the fragmentation of the bone
nucleus is visible. The radiolucent zones
show the invasion of vascular shoots and
the destruction of dead bone trabeculae.
2 3 . A s e p t i c bo n e n e c r o s i s � 23 1
3. The remodeling stage. In cases of com plete
recovery the contou r of the original bone
nucleus is restored into the original
trabecular structure. I n some cases
however due to the mechanical forces it
deforms, flattens, and certain bone
segments sometimes separate.
The treatment. The treatment of sterile
necrosis depends on localization, extent and
age. The English and German orthopedic
schools remain divided on this issue. The for­
mer considers sterile necrosis to be a
self-healing illness, as an unstable boundary
state, and provisional or symptomatic treat­
ment is recommended only when the condi­
tion actually causes an ailment. In contrast the
German orthopedic school define it rather as
an illness which should be treated as soon as it
has been diagnosed.
23.2. Adu lt aseptic bone necrosis
Adult bone necrosis resem bles childhood
bone necrosis o n ly in the histological picture.
The a ppearance and progression differ com­
pletely since the ability of decayed bone seg­
ments to repair comes to a close d u ring
adu lthood.
The joints most affected are the hip, knee
and shoulder. The most common types are
femoral head necrosis in the hip joint, medial
femur condyles necrosis in the knee j oint
(Ahlbeck 's disease) and necrosis of the head
of the humerus in the shoulders. A significant
difference in localization is that necrosis does
not occur in areas where the apophysis was
situated earlier, but within the area of the
epiphysis that bears all the static pressure, ir­
respective of the earlier epiphysis boundaries.
Clinical symptoms. In adults the disease
is considered irreversible since it destroys not
only the relevant bone tissue, but also the en­
tire hyaline cartilage surrounding it. Another
vital difference is in the symptoms : in some
232 � 23. Aseptic bone necrosis

cases there are n o spectacular clinical symp­
toms or ailment zero or silent stage), then, as
the disease progresses, the permanent syno­
vitis causes significant pain and immobility,
which is initially temporary and gradually be­
comes permanent.
Aggressive synovitis is caused by a mas­
sive surge of necrotic micro fragments into the
j oint which in turn causes reactive synovitis.
Early X-ray pictures usually do not indicate
anything, whereas an MR test can already
show the size of the deformity in stage zero.
Diagnosing in stage zero is vital as in most
cases this is a bilateral process including vari­
ous phases. After the diagnosis of active and
painful necrosis on one side, it is imperative to
check the opposite side, as minimal preven­
tion treatment is possible only if the diagnosis
is made in stage zero.
Differential diagnosis. Adult transitory
osteoporosis (osteopenia) has very similar
clinical symptoms; but its clinical progression
and appearance are different. It occurs most
frequently in the hip joint, causing pain on
motion and synovitis. The native radiological
picture is negative, while the MR images
show a sterile necrosis-like state on the femo­
ral head. The only difference is that the pro­
cess affects not only the head, but the whole of
the metaphysis. With this type of deformity,
recovery is usually spontaneous and requires
only observation.
� Chemical intoxication, causing enzymatic
disorders in the liver,
� Long-lasting steroid treatment for
systemic diseases (rheumatoid arthritis,
non-differentiated collagenosis, organ
transplants). Osteoporosis due to chronic
steroid treatment causes micro fractures,
then necrosis at the site most affected by
mechanical strain (the femoral head).
� Necrosis following radiation treatment
(radiotherapy) : A vascular disorder due to
lasting radiotherapy and damage to the
cells responsible for the bone metabolism
could be the cause.
� Caisson disease this afflicts people work­
ing at constant high pressure, especially if
they leave this too quickly, or in a
defective sluice chamber). The viscosity
of the blood changes and micro­
embolization disrupts the blood supply.

Etiology and types. Sterile necrosis oc­

curring during adulthood generally does not
indicate any cause or source of the illness in
which case it is classified as idiopathic.
However, various systemic diseases and
causes are known, including iatrogenic disor­
ders.

� Alcoholism or long-term alcohol abuse

features in the history data in most cases of
adulthood femoral head necrosis, there is a
latent or manifest liver enzyme disorder
that causes metabolic changes and local
osteoporosis.
Fractured neck of femur treated earlier with a
cannulated screw. One year later, necrosis of the
head is visible, with a wedge-shaped crack.
 23. A s e p t i c bone n e c r o s i s � 233

� Post-traumatic bone necrosis, which i s

usually suffered b y older patients after a
fracture of the femur neck. The reasons for
this lie in the sensitive arteries supplying
the femoral head (Fig. 23.2.).
� Iatrogenic, bone necrosis develops after
surgery. A typical example is the meta­
tarsal head necrosis following osteotomies
for hallux valgus.

Besides the epiphysis, bone necrosis may

occur in the meta-diaphysis during adulthood.
Its causes are generally unknown. It appears
in X-ray films as irregular, dot-like calci­
fications (Fig. 23.3.), and sometimes difficult
to differentiate from real bone tumors, like
enchondroma, central chondroma of long tu­
bular bones, chondrosarcoma.

Fig. 23.3.
An old lady, with extensive bone necrosis in the
meta- and diaphysis of both femur
 Ta mas I l les, J6zsef La katos, Pet e r Pa l Va rga
24. Diseases of the spine
24. 1. Biomechanics, functional
anatomy, functional units
of the spine
Bony transformation of the cartilaginous
spine field starts in embryonic weeks 9 and 1 0
with the appearance of the ossifying centers.
Each vertebra has three primary ossifying
centers : one in the vertebral body, and one on
each side of the vertebral arch (Fig. 24. 1 .) .
The primary ossifying centers of the arches
develop at the origin of the transverse pro­
cesses, and the ossification then starts in every
direction (forward to the posterolateral part of
the vertebral body, backward to the lamina of
the arch and to the spinous process, laterally
to the transverse processes, and up and down
in the directions of the superior and inferior
articular processes.
The ossification of the vertebral bodies
and arches is completed at the age of 3 years
Fig. 24. 1.
Primary ossifying centers of vertebrae
for the cervical segment, and at the age of 6
years for the lumbar part.
Further development of the vertebral bod­
ies originates from the secondary ossifying
center, which appears at the edge of the upper
and lower endplates at the age of 8 years .
The spine must be stable and firm, and at
the same time flexible and elastic. It can fulfil
this double, antagonistic requirement thanks
to its columnar functional units.
The basic functional unit of the spine is the
elementary moving segment (Fig. 24.2.).
The basic moving segment contains two
neighbouring vertebrae and the connective
structures, naturally together with the muscles
Fig. 24.2.
Elementary movement segment and its compo­
nents.
a: Ligamentum longitudinale anterius, b: inter­
vertebral disc, c: ligamentum longitudinale
posterius, d: facet joints, e: ligamentum
interspinosum
236 � 2 4 . D i s e a s e s of t h e s p i n e
and ligaments attached t o the corresponding
transverse and spinous processes.
As concerns the function of the basic mov­
ing segment, there is a special connection be­
tween the two vertebrae. Because of their
shape, the vertebrae can be regarded as levers,
with the support point located in the facet
j oints. The force sustained by the vertebral
body is mitigated directly and passively by the
intervertebral disc (Fig. 24.3.). Through the
levers created by the vertebrae, the tension of
the interspinous ligaments and muscles both
indirectly and actively decreases forces that
affect the basic moving segment. In this way,
the compression forces in each basic moving
segment are eliminated both passively and ac­
tively.
The elasticity of the spine, ensured by the
basic moving segments, is essential to elimi­
nate the rough quivering produced during
walking. The physiologic curves of the spine
also contribute to this elimination.
Fig. 24.3.
Tasks of the elementary movement segment. The
vertebrae are the levers; they act both directly and
passively to decrease the forces acting on the verte­
bral body; the tension of the interspinous ligaments
acts both indirectly and actively.
In the sagittal plane, the normal spine ex­
hibits a series of curves : cervical lordosis, tho­
racic kyphosis, lumbar lordosis and sacral
kyphosis, with average angles of 300(± 1 0°),
40° (± 1 0°), 45°(± 5°), respectively for tho­
racic kyphosis, lumbar lordosis and lordosis
for the lumbosacral transition.
The sagittal curves of the spine are conse­
quences of the erect posture and are typical of
the human race. The normal spine is a curve in
a single plane which is balanced at rest and
can be described by mathematic formulas.
One extremely important function of the
spine is to ensure the stability and balance of
the body.
The movements ofthe spine are the overall
resultants of the movements of the basic seg­
ments (see Chapter 2).
24.2. Examination of the spine
The successful treatment of diseases af­
fecting the spine often demands long and per­
sistent cooperation between patient and doc­
tor. The diseases of the spine are experienced
as neck, back and lumbar pain, the restriction
of movements, and deformities.
Since the spine is the central axis of the
body, its diseases may be accompanied by pe­
ripheral, neurological and vegetative symp­
toms . Consequently, in cases of spinal com­
plaints, an assessment of the spine itself is not
sufficient: the body as a whole, and the indi­
vidual limbs and other organs should also be
surveyed.
The first step in the assessment is a de­
tailed history. The accent must be put on the
pain that accompanies the disease of the spine
(since the deformities and movement restric­
tions are easier to specify).
Pain: The time of onset of spinal pain is
not always the same as the time of onset of the
disease.
In degenerative diseases the pain is some­
times manifested only years after the disease
has started. Chronic pain may create reflexes,
which result in pain that persists even after the
disease has ceased; such reflexes frequently
 2 4. D i se a s e s of t h e s p i n e � 237

are observed in chronic spinal pain syn­ tilt o f the shoulders can b e described i n terms
dromes. During the evaluation of the com­ of the difference from the horizontal, while
plaints, attention must be paid to the age, the the horizontal position of the pelvis can be de­
constitution, the occupation and factors influ­ fined by the direction of the iliac spines and
encing the psychological state. the cristae. If the pelvis is tilted, the next step
The type of the pain is important. Pain re­ is measurement of the sizes of the lower
lated to movement is usually a sign of a de­ limbs, to discover functional scoliosis result­
generative or benign disease. Pain at rest or ing from lower limb discrepancy.
during the night may relate to a tumor or an in­ Part of the physical assessment comprises
flammatory origin. the determination of the type and localization
The location and irradiation of the pain can of any spinal deformity. It is particularly es­
be significant features in spinal diseases. Pa­ sential to describe the sagittal curves exactly;
tients often complain of peripheral pain with­ this it may be of substantial help in demon­
out being aware of its significance, and do not strating the etiology of a possible lateral
associate it with a possible spinal disease. The curve.
doctor however must be fully familiar with Determination of a compensated or de­
the segmental structure of the spine and the compensated state of the upper body is a fur­
spinal cord. ther important element in the assessment (Fig.
24.4.). If a plumb bob hung from the spinous
process of vertebra C7 is situated in the mid-
24.2. 1. Assessment of the cervical
spine
Deformities of the cervical spine are rela­
tively rare. An antalgic posture, torticollis and
scoliosis can be detected on inspection.
During the assessment of pain a search is
made for compression-induced pain. Pressure
from above on the head, and tapping on the
spinous processes may generate pain in the
segments involved. The paravertebral muscu­
lature may also be painful and tender. Re­
ferred pain and occasionally radicular signs
may be detected on the upper limb. In
cervicobrachialgia, the upper edge of the tra­
pezium muscles, the lateral area of the shoul­
der joint and the lateral epicondyle of the hu­
merus can be especially tender. A detailed
neurological assessment of the upper limb and
a precise examination of the fine functions of )
the hand may be necessary.

Assessment of the dorsal spine. As con­

cerns the dorsal spine, the early recognition of
deformities is most important.
The first step in the physical assessment is
L
to inspect the standing patient from every di­ Fig. 24.4.
rection. Any asymmetry revealed by such an Assessment of the decompensation of the spine
inspection must be accurately recorded. The with a plumb bob.
238 � 2 4 . D i s e a s e s of t h e s p i n e
Fig. 24.5.
Assessment of rib hump.
dIe of the buttocks, the spine is compensated;
if there is any deviation, it is decompensated.
The most important part of the physical as­
sessment is the hyperflexion test, the aim of
which is to determine whether a deformity is
Fig. 24.6.
Bending test. With the patient bending lateral ly, the mobility of the curves can be assessed.
accompanied by structural changes. A charac­
teristic feature of structural changes is the
presence of vertebral rotation.
This can be assessed most easily with the
patient in a standing position, with a 90° hip
flexion and the upper trunk bent forward (Fig
24.5.). Hyperflexion resulting in a rib hump
on the convex side proves vertebral rotation
and hence structural changes in the spine.
Spinal mobility is an important character­
istic as concerns structural changes in the
spine. During bilateral flexion of the spine
(bending test), the ability of the spine to
achieve correction can be examined, while ax­
ial distraction can characterize the rigidity or
mobility of the spine (Fig. 24.6).
If a deformity with tenderness of the
spinous processes and paravertebral muscles
has been discovered, a search must be made
for possible pain, even though deformities do
not typically cause pain.
If the shoulders are pushed down, the pa­
tient may experience pain in the dorsal seg­
ment. If a patient standing on tiptoe transfers
 2 4. D i s e a s e s of t h e s p i n e � 239

the body weight to heel, that may also elicit and particularly a n assessment o f the
pam. abdominal or cremaster reflexes, may
The cause of pain elicited above and be­ disclose causes of neurological origin
tween the spinous processes may be chronic (syringomyelia or Chiari malformation),
muscle tension, or contact with the spinous
processes (Baastrup 's phenomenon, inter­
spinous arthrosis).
Pain may be provoked in the paramedian
line and in the facet j oints by lateral move­
ment of the spinous processes.
Tenderness is frequent even more laterally
due to the lasting, reversible tone increase of
the paravertebral muscles.
Myogelosis involves well-outlined, mo­
bile nodules in the musculature. The most
common localizations are the shoulder, the
neck and the lumbosacral area. Its cause is
muscle fiber hyaline degeneration and conse­
quent connective tissue deposition.
Movement of the dorsal spine is difficult
to judge separately. When the trunk is bent
forward, the distance between vertebrae C7
and D2 increases by 4-6 cm in normal cases 3 4
(Giinz 's sign). It is important toe assess of the
mobility of the dorsal kyphosis, which is per­
formed as follows : the forward-bending pa­
tient is requested to hollow the back and at the
same time to lift the upper limbs and head.
Normally, the dorsal kyphosis is then elimi­
nated.
Assessment of the dorsal spine includes a
consideration of the costovertebral joints,
a 5
which are best described by the expansion of
the chest. The expansion between full expira­ b

tion and inspiration, measured in the line of

the nipples, is normally 7-8 cm or more.
The general assessment comprises the de­
termination of various features :

• The time and circumstances of the onset of

the given deformity, and of any other
deformities in the family.
• The general appearance of the patient
(weight, height, and facial asymmetry),
the condition of the joints, muscles and
skin, and hyperpigmented spots on the Fig. 24.7.
skin (cafe au lait) . Risser's sign. Depending on the development of the
• The neurological status. A reflex iliac crest apophyses, stages 0-5 are differentiated
assessment (patella and Achilles reflexes), (al. This X-ray shows stage 3 (bl
240 � 2 4 . D i s e a s e s of t h e s p i n e
where the first appearance o f the spinal
cord manifestations may be a scoliosis
considered to be idiopathic.
� The time of the first menstruation.
� The current stage of sexual development
and the characteristics of secondary
gender features, according to Tanner 's
signs. In girls, this means observation of
the development of the breasts, and the
appearance of pubic and axillary hair; in
boys, besides the facial, pubic and axillary
hair, it involves determination of the size
of the testicles.
� Establishment of the phase of ossification,
related to Risser 's sign (Fig. 24.7.), is
based on observation of the ossifying
centers of the iliac crest, and the presence
or absence of the ossifying centers of the
vertebrae. The ossifying center of the iliac
crest appears in the anterior-superior iliac
spine, progresses to the posterior, and then
gradually unites with the iliac crest.
Fig. 24.8.
Schober's sign. On measurement of the forward flexion of the lumbar and dorsal spine, the marks drawn on
the skin move apart.
Risser ' s stages 1 -4 denotes the proportions
(in percentage) of the full length of the
crest, while in Risser 's stage 5 the
ossifying center is already fused to the
iliac crest.
� The biological age revealed by Tanner 's
signs and the bone age indicate the general
level of development of the patient,
accurate estimation of which is essential
for the prognosis and for the decision
concerning the treatment plan (see later).
24.2.2. Assessment of the lumbar
spine
The lumbar spine is the most mobile seg­
ment of the spine, but its range of motion is
difficult to describe in degrees. The extent of
forward bending of the lumbar spine is deter­
mined by Schober 's sign (Fig. 24.8.), via
measurement of the distance between two
�.

marks on the skin. The caudal skin mark is
drawn at the S I level, with the cranial mark 1 0
cm cranially, and the expanding distance is
measured. During forward bending, this dis­
tance usually increases to 1 5 cm; in the vent of
restricted movement it is less. Lateral bending
is 20°, while rotation is 1 0° . It should be noted
that the extent of rotation movement is great­
est at C l -C2 (80°) and gradually decreases
caudally. Below L l , the level of rotation be­
tween two vertebrae is only 1 -2°. This is quite
important for an understanding of degenera­
tive problems, where the first clinical sign is
an increase in the rotation, i.e. rotational insta­
bility.
Passive displacement can be provoked in
the sacroiliac joint; if this elicits pain, it points
to a disturbance of the j oint. Bilateral com­
pression of the iliac crest may provoke pain in
these cases (Fig. 24.9.). If there is sacroiliac
joint involvement, hyperextension of the hips
in the prone position may also be painful (sa­
croiliac Mennel ' s sign) .
The lumbosacral Mennel ' s sign is utilized
to examine the lumbar facet joints . The lum­
bar spine of the prone patient is fixed on the
examining couch, the lower limbs and thighs
are then lifted, and the pelvis is pulled dor­
sally. In a positive case, the patient feels pain
in the lumbar spine, which is a sign of irrita­
tion in one of the lumbar segments, most
probably in the lumbosacral segment.
During the assessment of local pain, ten­
derness and pain are searched for in median
Fig. 24.9.
Compression assessment of the sacroiliac joint.
2 4 . D i s e a s e s of t h e s p i n e � 24 1
and paramedian lines and along the sciatic
nerve (Valleix ' s points).
Referring pain and radicular signs. One
of the most important radicular signs is
Lascgue ' s sign (Fig. 24.5 1 ) . With the patient
lying supine, the lower limb is gradually
flexed at the hip, with the knee extended.
Normally, such flexion is possible to an extent
of 90%. If the patient feels sharp pain in the
flexion internal 3 0-80°, Lascgue ' s sign is pos­
itive; the pain is due to the tension and patho­
logic compression of the sciatic nerve. When
Bragard' s sign is examined, the hip is flexed
until the onset of pain and the foot is then
dorsiflexed: the pain increases. While
Lasegue 's sign is positive for any root in the
sciatic nerve, the femoral sign may be positive
in cases of root compression of the higher
lumbar spine segments. If the knee of a patient
in the prone position is flexed, severe pain is
experienced at a certain degree of flexion,
blocking further flexion. The knee flexion can
be extended only after the pelvis is lifted or af­
ter a significant flexion of the hip.
During examinations of the radicular
signs, motor, sensor and reflex changes are
searched for that correspond to the individual
roots . The most common symptoms are re­
lated to roots L4, L5 and S I (Fig. 24.48.,
24.49., 24.50.).
The assessment of the lumbar spine in­
cludes an assessment of the lower limbs, as it
is a common dilemma to decide whether com-
242 � 2 4 . D i s e a s e s of t h e s p i n e
plaints are o f spinal i n origin o r whether some
hip disorder plays a role in their appearance.
24.2.3. Radiological assessment
Basic examinations. The X-ray assess­
ment of spinal disorders demands antero­
posterior and lateral exposures ofthe involved
segment. Much more practical, however, is a
total spine film (30 . 90 cm) including the pel­
vis and the skull, taken with the patient in a
standing position. Standing is necessary, be­
cause the curves resulting from loading of the
spine are easier to consider. If there are tho­
racic spine complaints, the end-points of the
possible curvature are located on the antero-
Fig. 24.10.
Measuring the degree of scoliosis with (obb's
method.
posterior X-ray film. The upper end of the
structural curve is the vertebra, at which the
upper endplate displays the greatest tilt in the
direction of the concavity, and the lower end
of the structural curve is the vertebra, at which
the lower endplate has the greatest tilt in the
direction of the concavity. The supplementary
angle to the perpendicular lines drawn to the
endplates of the end vertebrae shows the ex­
tent of the structural curves (Fig. 24.1 0.). The
tip of the curve, decided from the X-ray film,
is normally the vertebra located farthest from
the axis ofthe body, which also has the largest
rotation. The extent of the rotation is usually
estimated from the contour of the vertebra,
and from the projection of the pedicles and
laminae that deviate from symmetry. Two in­
ternationally approved methods (those of
Nasch-Moe and Perdriolle; Fig. 24.1 1.) are
used to measure the rotation of the vertebrae,
from which the neutral vertebrae of the curves
can be determined. These are located next to
the extremities of the curves without rotation.
From a therapeutic aspect every vertebra with
a similarly directed rotation is considered a
component of the curve.
Lateral films are taken for detection of the
borders of the sagittal curves and their possi­
ble changes.
[0 il] o
++
�w
[Q-HH
Fig. 24. 11.
Measurement of vertebral rotation with the method
of Nasch-Moe and Perd riolle.

To summarize: In the course of the normal
X-ray assessment, information is obtained on
the presence of scoliotic curves, and on the
presence or absence of possible concomitant
changes (congenital abnormalities, traumas,
tumors, dystrophic bone diseases, etc.).
Additional examinations
Bending films: Information is usually
acquired on the mobility of the curve and
on the exact borders of the main and
compensatory curves. This information is
needed primarily for the planning of
therapeutic interventions (Fig. 24. 1 2.).
� Dittmar 's views: These special, oblique
X-ray views are mainly used to visualize
the foramen and the interarticular part of
the vertebral arch (see: spondylolysis). In
the event of deformities, these views are
used to determine the anteroposterior and
lateral aspects of the markedly rotated
vertebrae. This may be of great help
particularly for the recognition of possible
a b
Fig. 24.12.
Bending X-ray. The mobility of the spinal curve can be estimated.
a: Normal posture, b: Bending to the rig ht, c: Bending to the left
2 4. D i s e a s e s of t h e s p i n e � 243
congenital abnormalities located at the
level of the punctum maximum of the
curves.
� eT examinations: These are needed in
special cases for the assessment of bony
structures . They help to reveal congenital
abnormalities. The 3D reconstruction
picture accurately determines the
localization of wedge vertebrae. In
degenerative diseases, eT scans are
performed to detect prolapsed discs, spinal
stenosis, or possible bone tumors.
� MRl examinations: These are informative
if there is a suspicion of pathological
processes involving the soft tissues and the
spinal cord. In cases of neurofibromatosis,
MRI examinations of the entire spine are
mandatory to exclude spinal cord mani­
festations and a paravertebral soft-tissue
mass, and also when congenital vertebral
abnormalities are present, to exclude
concomitant congenital spinal cord
abnormalities (syringomyelia, diastemato-
c
244 � 2 4 . D i s e a s e s of the s p i n e

a b

Fig. 24.13.
Myelography. A definite stoppage of the contrast flow is visible. a: Anterioposterior view, b: Oblique view

myelia, tethered cord, and Arnold-Chiari metastasis, or inflammatory disease arises.

malformation), and in degenerative spinal
disorders to evaluate the state of the
intervertebral discs.
Scintigraphy (bone scan) : This is most
frequently performed when the suspicion
oftumoral changes (e.g. osteoid osteoma),
Since scoliosis is rarely painful, in cases of
painful scoliosis the bone scan is indicated
to discover the cause of the pain.
Myelography: X-ray pictures taken
following the inj ection of contrast material
into the epidural space can reveal ex-

a b
I-..- �
Fig. 24. 14.
Discography demonstrating a healthy (a) and a diseased (b) discogram.
 24. D i se a s e s of t h e s p i n e � 245

pansive processes (herniated discs, spinal Deformities in 3 dimensions :

cord tumor). They can be highly infor­
mative when combined with a CT scan � Scoliosis
(Fig. 24. 13.). - Functional (nonstructural) scoliosis
Discography: X-ray pictures taken - Postural scoliosis
following the inj ection of a known amount - Scoliosis related to limb length in-
of contrast medium into the disc furnish equality
information on the state of the disc and the - Hysteriform scoliosis
pain-inducing effect of the inj ected - Scoliosis related to root irritation
medium (Fig. 24. 1 4.). - Scoliosis related to disc hernia
- Scoliosis related to tumor
- Scoliosis related to inflammation
24.3. Classification of deformities - Appendicitis
- Structural scoliosis
of the spine - Scoliosis of unknown origin (idio-
pathic) scoliosis
Deformities in one plane, localized to the
- Scoliosis of known origin
sagittal plane
- Congenital scoliosis
�
- Neuromuscular scoliosis
Hyperkyphosis
- Neurofibromatosis
- Functional (nonstructural)
- Mesenchymal diseases
hyperkyphosis
- Trauma
- Postural hyperkyphosis (negligent
- Extraspinal contractures
posture)
- Osteochondrodystrophies
- round back (dorsum rotundum)
- Metabolic diseases
- saddle back (dorsum
- Tumors
kypholordoticum)
- Structural hyperkyphosis
- Scheuermann 's disease
- Calve 's disease 24.3. 1. Characteristics of
- Neuromuscular diseases deformities in one plane
- Congenital hyperkyphosis
- Trauma During the demonstration of deformities,
- Metabolic disease it is essential to differentiate the functional
- Osteoporosis and structural curves, since absolutely differ­
- Tumors ent treatment strategies must be employed for
- Iatrogenic hyperkyphosis the two curve types.
- Postoperative Functional curves are extremely mobile,
- Post-irradiation and well correctable. They never become
� Hyperlordosis rigid, and they are not accompanied by struc­
- Functional (nonstructural) tural changes of the vertebrae.
hyperlordosis Sagittal deformities are curves that usually
- Postural lordosis originate from increased or decreased physio­
- flat back (dorsum planum) logic curves. Physiologic sagittal curves de­
- Hyperlordosis related to hip flexion velop as the individual learns to sit, stand and
contracture walk, in parallel with the development of the
- Structural lordosis trunk muscles.
The spine of a neonate is characterized by
the intrauterine position, with kyphosis of the
246 � 2 4 . D i s e a s e s of t h e s p i n e
entire spine. I n the course o f individual devel­
opment, the first curve to appear is cervical
lordosis. This is observed when the child turns
onto the tummy and lifts up the head. In the
other segments of the spine at this stage there
is a ventrally concave curve, kyphosis of the
entire spine. The kyphotic spine first straight­
ens when the individual begins to stand and
walk (at 1 3 months), and lumbar lordosis then
develops. From the age of 3 years, mild
lordosis is visible, which attains the final form
at the age of 8- 1 0 years .
Any external factor which disturbs this
spontaneous process, depend primarily on the
level of development of the muscles, will im­
pair the further development of the child.
The most common error is to force a child
to sit and walk too early before the appropriate
development of the necessary muscles. In
many children, overfast vertical development
is imposed, instead of ensuring a spontaneous,
horizontal motion which provides a more
healthy static development course. If the child
a b
Fig. 24. 15.
Correct and improper postures.
a: Normal postu re, b: Round back, c: Kypholordotic
(saddle back), d: Flat back
has enough room following turning onto the
tummy, the demand for motion will be ful­
filled by moving horizontally (creeping or
crawling) without sitting up. Standing and
walking will start only when the musculature
has properly developed. The advantage of
horizontal as compared to vertical motion de­
velopment is that each static developmental
stage takes place with the properly developed
musculature without external assistance.
Thus, the child sits rarely or not at all, avoid­
ing the posture which imposes the highest
passive load on the spine.
The normal sagittal curves become auto­
matic and characteristic qualities of the indi­
vidual at the age of 8- 1 0 years.
24.3. 1 . 1 . Fu nctional hyperkyphosis
The most typical example is a negligent or im­
proper posture. Its development is related to
the diminished load-bearing capability of the
dorsal and trunk muscles, but psychological
factors may also contribute.
The following types can be distinguished
(Fig. 24. 1 5.):
� Round back (dorsum rotundum) : The
enlarged dorsal kyphosis is enhanced,
while the lumbar lordosis is physiological.
The hyperkyphosis usually involves the
upper lumbar section. It is a consequence
of inequality between the development of
the muscles and the gravitational load, but
a lack of the willpower, needed for the
proper posture also plays a role in its onset.
� Saddle back (dorsum kypholordoticum) :
Both the dorsal kyphosis and the lumbar
lordosis are larger than normal. The most
common form occurs at the age of 6- 1 0
years, i f the muscle development i s not
proportionate with the growth. The
d
increase in lumbar lordosis is most
probably is compensatory.
A negligent posture may be balanced by
active gymnastics and muscle training exer-
 24. D i s e a s e s of t h e s p i n e � 247

cises, whereby its progression may be
stopped. An effort must be made to discover
the origin, since a negligent posture is fre­
quently a symptom of a general weakness of
the muscular system. A negligent posture is
often accompanied by generalized j oint lax­
ity, drooping shoulders, and a protruding ab­
domen. Such children are often easily fa­
tigued and reticent, which suggests a psycho­
logical cause.
Treatment. The etiology must be discov­
ered and the cause must be eliminated, as an
irreversible postural deformity can develop in
a neglected case.
24.3. 1.2. Functional hyperlordosis
An increased lumbar lordosis is generally
compensatory. It serves the aim of balancing
the spine in the sagittal plane when the dorsal
kyphosis is enhanced. Normalization and
straightening in a lying position are typical of
compensatory hyperlordosis.
the dorsal kyphosis disappears and relative or
absolute dorsal lordosis develops. The lumbar
lordosis is also greatly diminished, the spine
becomes expressively straight, and as a result
the back becomes flat.
The Delmas 's formula indicates that, if the
number of spinal curves diminishes, the sta­
bility also decreases, and consequently the
spine becomes insufficient and unstable; this
IS a common source of low back pain in
adults.
24.3. 1.3. Struct u ral kyp hosis
The most frequently observed structural
changes o f t h e p hysiologic curves in one
plane are kyp hotic changes. There are two
well-differentiated forms: reg u lar and angular
ky p hosis (Fig. 24.16.).
Regular hyperkyphosis : The most typi­
cal example of thoracic hyperkyphosis devel­
ops as a result of Scheuermann 's disease (Fig.
24.1 7.). The extent of the kyphosis increases

Besides compensating dorsal hyper­

kyphosis, hyperlordosis may somewhat cor­
rect an ill-positioned pelvis.
In consequence of the flexion contracture
of the hip the pelvis tilts forward, and the sa­
cral slope is greatly increased, reaching even
90°. To compensate this, lumbar hyperlor­
dosis develops, in order to maintain the nor­
mal, balanced bearing.
In parallel with the weight gain of the em­
bryo in pregnancy, the sacral slope increases,
and hence the gravity line of the body runs in
front of the hip j oint. A precondition of
straight standing is the compensatory increase
of the lumbar lordosis, which may explain the
frequent low back pain during pregnancy. Af­
ter delivery, the hyperlordosis declines, and
accordingly this low back pain alleviates. Ex­
tremely marked lumbar hyperlordosis devel­
ops in cases of paralysis of the abdominal and
long dorsal muscles.
Flat back (dorsum planum) : This is an­
other characteristic sagittal curve abnormal­ Fig. 24. 16.
ity. The dorsal segment is almost flat, since Regular and angular kyphosis.
248 � 24. D i s e a s e s of t h e s p i n e

along the full length o f the thoracic spine, and

the punctum maximum is located distally. In a
parallel process, the cervical and lumbar
lordosis also increases considerably, so as to
compensate the increase in the thoracic curve.
After the end of growth, progression is not
likely.

� Scheuermann 's disease (osteo­

chondrosis j uvenilis dorsi, kyphosis
dorsal j uvenilis)

This d isease develops because of damage to

the secondary ossifying centers of the in­
volved vertebrae; it is mai nly localized to the
dorsal section, and involves many vertebrae.
In a typical case, the anterior third of the af­
fected dorsal vertebra l body loses its height,
and consequently an increase in the regular
dorsal ky p hosis fol lows. The intervertebral
discs become thinner, and display degenera­
tive signs. Typica l p henomena are the rigid
dorsal hyperkyphosis and in advanced cases,
back pain. The treatment is chiefly conserva­ Fig 24.17.
tive; surgery is rarely performed (Fig. 24. 17.). Kyphosis dorsalis juvenilis.

Etiopathology. This condition was first
described as juvenile dorsal kyphosis by
Scheuermann in 1 920, referring to the aseptic
necrosis of the secondary ossifying centers of
the vertebral bodies. The ossifying distur­
bance involves chiefly the front half of the
epiphyses, and the vertebral bodies assume a
wedge shape. The epiphysis areas with weak­
ened resistance may crack, and nucleus
pulposus substance may protrude through
these fissures to the cancellous mass of the
vertebral bodies (Schmorl 's node) . The X-ray
changes point to the absence of normal
growth rather than a destructive process. In
the period of active growth, the mechanical
overload due to the physiological kyphosis
further hinders the development of the verte­
brae, leading to a further diminution of height.
The compression of the anterior part of the
vertebrae causes the dorsal kyphosis to in­
crease, enhancing the mechanical load on the
ventral edge of the vertebrae. This vicious cir­
cle results in Scheuermann' s hyperkyphosi s.
Dominant inheritance factors also play a
role in this ossification disturbance, since the
likelihood of multiple occurrence in the fam­
ily is 50%.
Incidence : Various sources report the in­
cidence in puberty to be 0. 5 - 1 1 %. The gender
distribution also varies. According to clinical
and X -ray surveys, the Hungarian incidence is
1 1 %, and there is no difference between the
two genders.
Localization. This condition is most com­
mon in the dorsal vertebrae, and it involves
more than one. Less frequently, it is seen in
the dorsolumbar section, where it likewise in­
volves the epiphyses of a few vertebrae. Even
more rare is a lumbar localization, when it in­
volves only the epiphysis or end-plate of a sin­
gle vertebra, resulting in a large Schmorl ' s
hernia.
Apophyseal osteochondrosis should be
classified as a separate pathological entity.
This also involves lumbar vertebrae, and frag­
mentation and the disturbed growth of the
 2 4. D i s e a s e s of t h e s p i n e � 249

apophyseal bone centers forming the anterior type of pain usually ceases at the end of
edge of the vertebrae can be observed. The growth, unless there is a severe residual defor­
etiology is unclear, as for the epiphyseal type. mity.
Clinical symptoms: The disease starts at After the end of growth, however, if a se­
around the age of 1 0 years ; the explanation is vere residual deformity is present, the pain
the onset of secondary bone centers activated caused by the degenerative changes will be
from the age of 8 years. The most important the leading clinical symptom, paradoxically
clinical symptoms are hyperkyphosis and localized in the area of the compensatory
pain. curves.
Hyperkyphosis : The chief clinical symp­ In lumbar Scheuermann 's disease the most
tom is the fixed, absolutely rigid and in­ important clinical symptom is the accentu­
creased dorsal kyphosis, which also causes se­ ated, severe pain localized to the involved
rious cosmetic problems . In Scheuermann ' s vertebrae. Since the deformity in this location
disease, the kyphosis i s decreased to only a is difficult to discover (initially only a de­
very small extent or not at all . creased lordosis is seen, and manifest
Apart from the rigid, fixed dorsal kyphosis can be detected only in advanced
kyphosis, another important symptom may be cases), the source of the pain is often confused
distalization of the punctum maximum of the with inflammatory diseases (vertebral
kyphosis. A conspicuous sign is the hyper­ osteomyelitis or spondylitis tuberculosa).
pigmentation of the skin over this segment, X-ray signs: Besides the physical findings
resulting from the increased dorsal kyphosis the diagnosis of Scheuermann 's disease is
in response to the pressure against the based on the X-ray examination. The typical
back-rest of a chair or bench . vertebral changes are the following (Fig.
Another clinical sign is the increase in the 24. 1 8.):
lumbar lordosis. This is compensatory and
therefore especially mobile; it may be accom­
panied by spinal pain because of the stretched
ligaments of the lordotic spine .
In about one-third of the cases hyper­
kyphosis is accompanied by moderate, usu­
ally functional or mildly structural dorso­
lumbar scoliosis, which is never progressive.
In general there are no neurological signs and
radicular pain.
Pain: Besides the cosmetic problem con­
cerning the spine, the pain is an important
clinical sign; its appearance and intensity vary
with the age, the stage of the disease, and the
localization and severity of the kyphosis. In
the early phase, pain is usually not experi­
enced. The patient seeks out the doctor be­
cause of the visible deformity.
At around puberty, the frequency of pain
may increase, but in only 1 0-20% of the cases.
The muscular pain in the interscapular region
can be characteristic, and as can the tender­
ness of the involved vertebrae, which in­
creases in response to physical activity and Fig. 24.18.
the pain if these vertebrae are tapped on. This X- ray signs of Scheuermann's d isease.
250 � 2 4 . D i s e a s e s of t h e s p i n e
- Trapeze-shaped vertebrae: One o f the
most significant X-ray findings is the
wedge formation of at least three ver­
tebrae. The criterion of the wedge shape
is that the end-plates converge to the
front by more than 5 ° . Trapeze-shaped
vertebrae lead to the development of
regular hyperkyphosis in excess of 50°.
The intervertebral spaces diminish,
particularly anteriorly, due to the hyper­
kyphosis and the trapeze-shaped
vertebrae.
- Schmorl 's hernias: These changes are
considered to be a diagnostic criterion
for Scheuermann' s disease. Small hypo­
dense, pearl-like indentations are visible
under the end-plate fissures, often
surrounded by a sclerotic margin.
- End-plate irregularity: The secondary
ossification centers are fragmented, and
the end-plates therefore display a zigzag
irregularity.
Two rare, atypical radiological signs are
observed in the lumbar spine :
- Epiphyseal form: A huge Schmorl ' s
hernia is visible in a single vertebra,
usually in the anterior third of the body.
This differs from the common
Schmorl 's hernias, for it may attain a
considerable size; it is connected widely
to the intervertebral space, and the edges
are markedly sclerotic.
- After the end of growth, in the late phase
of Scheuermann ' s disease, besides the
radiological features outlined above,
degenerative changes may appear in the
involved and in the compensatory
curves, with the most typical osteo­
phytes .
Clinical sequence. Three stages are dis­
tinguished:
The first stage starts between the ages of
1 0 and 1 2 years, with a negligent posture and
a mildly increased dorsal kyphosis, usually
with no complaints. In this period, the in­
creased dorsal kyphosis is mobile, and can be
eliminated through active muscle power. The
child is taken to the doctor because of the bad
posture.
The second stage starts at the age of 1 2 and
lasts until the end of growth. The spinal seg­
ment becomes rigid. The progression may ac­
celerate in teenagers, and the deformity and
the complaints intensify. 1 0-20% of the pa­
tients seek the doctor' s help for these com­
plaints . Apart from the visible deformity, the
interscapular pain and vertebral tenderness
are the leading signs. The symptoms may be­
come stronger on physical activity, but they
usually cease at the end of growth.
The third or late stage, after the end of
growth accompanies the patient throughout
life. However, large proportions of the pa­
tients remain symptom-free. In the event of a
severe residual deformity, the early
spondylosis progresses. Pain will be strong in
the compensatory curves, mainly in the
cervicothoracal junction and in the form of
headache. In the lower lumbar spine, the com­
pensatory curves may lead to the appearance
of lumbago at a relatively young age.
Treatment. Although three stages are dif­
ferentiated in Scheuermann' s disease, the his­
tory is not well known. Both conservative and
surgical treatment are based on empirical fac­
tors. An adolescent with mild hyperkyphosis
(50°) without rapid progress does not gener­
ally need treatment, but check-ups should be
regular, with continuous training of the active
elements of the spine (exercises and swim­
ming). These patients have a good chance for
a symptom-free life following the end of
growth.
The basic treatment of Scheuermann 's dis­
ease is conservative. The principles and indi­
cations of therapy are as follows :
- Rapidly increasing dorsal kyphosis.
- Vertebral changes, and progressive
wedging.
- Severe dorsal kyphosis and pain that
does not respond to medication or
conservative measures.
- Breathing disturbances due to vertebral
deformities.

With timely therapy, the overwhelming
majority of the patients respond well to corset
treatment. Indications are :
- A curve less than 70°
- Risser ' s sign less than 3 .
- More than 40% o f the curvature can be
corrected passively.
- Minimal vertebral wedging is seen.
The most important precondition of effec­
tive corset treatment is spinal flexibility, the
chances of the success of corset treatment be­
ing particularly good when treatment is
started in the first stage. In this benign condi­
tion, surgery is rarely justified. An indication
for surgery is a curve exceeding 80°. If the de­
formity is accompanied by severe pain, which
hinders the everyday activities and is not al\e­
viated by conservative means, surgery may be
considered with dorsal kyphosis of 60-80°.
The strategy of the surgery is different in
the second and third stages. In the second
(
r 'i -
'iH
'
-
L
..J
.,
a b
Fig. 24. 19.
A 16-year-old boy with Scheuermann's disease. Preoperative hyperkyphosis 74° (a). Following surgical correc­
tion 44° (b). No lateral cu rve (c); the correction is in only 2 dimensions. (d)
24. D i s e a s e s of t h e s p i n e � 25 1
stage, operative correction may be performed
from a posterior approach; in the third stage,
after the end of growth, the surgery is carried
out in two steps: anterior release and posterior
correction (Fig. 24.1 9.).
Angular hyperkyphosis: This is a strictly
sagittal deformity, where the changes involve
only a few (sometimes only a single) levels,
and consequently the harmonic sagittal align­
ment of the spine is badly distorted with an an­
gular deformity. This deformity has two prin­
cipal consequences: the risk of instability, and
the possibility of marked secondary deformi­
ties, depending on the extent and location of
the primary deformity, to maintain the com­
pensation of the spine. These two complica­
tions are closely related, the increase in defor­
mity may enhance the instability.
A number of diseases may result in angu­
lar kyphosis, e.g. Calve 's disease, neuro­
muscular conditions, congenital vertebral ab­
normalities, trauma, metabolic diseases, such
c d
252 � 2 4 . D i se a s e s of t h e s p i n e
a s osteoporosis, certain tumors, iatrogenic
conditions, and after surgery or irradiation.
The risk of instability associated with an­
gular kyphosis is evidently dependent on the
etiology and also on the level and/or the state
and spatial location of the connected func­
tional units. The most severe instability may
arise following trauma. Similarly, congenital
anomalies, such as a dislocated spine or poste­
rior hemivertebra lead to kyphotic deformities
and hence to potential (occasionally immedi­
ate) instability.
The outcome may be similar in infectious
diseases of the spine, where one or more discs
and vertebral bodies may be destroyed, lead­
ing to stable angular kyphosis.
The most important elements of the treat­
ment strategy are the restoration of the bal­
ance, the normal geometry and the bio­
mechanics of the spine, and the ensurance of
stability. These aims are achieved either by a
conservative approach, or by surgical means,
depending on the etiology.
� Calve 's deformity or vertebra plana
(vertebra plana, osteochondrosis
vertebrae)
This is a condition localized to a single verte­
bra and caused by damage to the primary
(central) ossifying center. In a typical case, the
ossifying center of a dorsal vertebra is flat­
tened and the anterior two-thirds of the ver­
tebra becomes paper-thin.
During recovery, the height of the verte­
bral body is partially restored, but it never
reaches its original height. The discs adj acent
to this condition remain intact.
The etiopathology is uncertain. Calve
classified this affection as a sterile necrosis of
the ossifying centers of the developing organ­
ism, on the basis of the radiological signs and
the benign outcome. Histological studies,
however, demonstrated that the reason for
vertebra plana was eosinophilic granuloma. It
is not excluded, that there are two different
etiologies which give a similar clinical pic-
..
ture.
Clinical symptoms. Typically, dorsal
pain sets in, which increases in response to ac­
tivity. Insignificant angular kyphosis is ob­
served at the painful segment, and the spinous
processes are tender. Since the segmental
nerves are irritated, mild neurological signs
may arise in the corresponding dermatome.
The movements of the spine may be restricted
in every direction.
X-ray signs. In the lateral view, the bony
center of the vertebra involved is hyperdense,
sclerotic and flattened (Fig. 24.20.). The ante­
rior part of the ossifying center becomes pa­
per-thin (silver dollar vertebra). Both below
and above, the intervertebral spaces exhibit
compensatory widening.
Fig. 24.20.
Calve's deformity. The D6 vertebral body is paper­
thin (on the left). After 2 years, almost total restitu­
tion has been achieved (on the rig ht).
Differential diagnosis. Eosinophilic
granuloma must be excluded. Sternal punc­
ture usually facilitates the diagnosis. It must
be differentiated from tuberculotic vertebral
diseases, the latter involving the neighbouring
discs. In Calve ' s disease the ESR (erythrocyte
sedimentation rate) and the result of other lab­
oratory tests are normal.
Therapy. This condition usually heals
spontaneously, and is not progressive; it does
not cause instability at the spine. In the acute,
painful stage, some days of bed rest, a reclin­
ing corset or a temporary reduction of activity
is needed.

24.3.2. Spinal deformities 3
dimension: Scoliosis
24.3.2. 1. Fu nctional (nonstruct u ra l)
scoliosis
Functional curves are quite mobile, well- cor­
rectable cu rves; they never become rigid.
On lateral bending, they are fully and sym­
metrically corrected, so they can never be
identified from bending X- ray views. No
structural changes are present in the verte­
brae; according ly, no rotation or related rib
hump is detected in the hyperflexion view.
Etiopathology. This entity is a lateral spi­
nal curvature of unknown origin. There is
generally a simple postural deformity in the
background. The most frequent reason is the
weak back musculature, the lateral bending of
the spine being related to the usually poor
muscular state. An important factor is the im­
paired balance between the active and passive
elements of the spine. It sets in when all-day
studies and an inactive made of life mean that
the musculature does not develop appropri­
ately, but weakens instead, not following the
development of the passive elements of the
spine. This deformity may also be the first in­
dication of psychic problems, and it may also
turn attention to root compression (a juvenile
disc prolapse). It is almost always present
when there is a limb length discrepancy.
Incidence. It occurs mainly in girls, to­
gether with the first signs of puberty, in about
1 5% of the population.
Localization. In 90% of the cases a
'
wide-arched, left convex thoracic or
thoracolumbar, very mobile curve is seen.
Clinical symptoms. There is a lateral
curve, and a badly negligent posture, and the
shoulders are drooped forward. Abdominal
breathing and other signs of joint laxity are
frequent. Flatfoot is also usually present. The
children are asthenic, and their activity is di­
minished.
2 4. D i s e a s e s of t h e s p i n e � 253
The lateral curve is quite mobile, and well
correctable with active and passive muscle
power. When the individual is lying or bend­
ing forward, the curve disappears and no rib
hump or any structural change is detected.
X-ray signs. Since the deformity disap­
pears in a lying position, it can be detected
only in a standing X-ray, as a wide arched
bend showing no structural signs.
Therapy. If the functional scoliosis is due
to a limb length discrepancy, the curve is cor­
rected by equalization. Functional scoliosis is
caused by appendicitis, or disc herniation: if
the etiologic factor is eliminated, the bend
ceases. Regular swimming and physical exer­
cises are recommended to strengthen the back
and belly muscles.
24.3.2.2. Struct u ra l sco liosis
Scoliosis is the best known of the general­
ized 3D deformities of the spine. Structural
scoliosis is such a deformity, characterized by
frontal bending, an anteroposterior, almost
exclusively lordotic deformity and horizontal
vertebral rotation around the gravitational
axis.
24.3.2.2. 7. Idiopathic scoliosis
This is the best-known structural deformity.
Idiopathic structural scoliosis is a 3D deformity
of the spine, characterized by frontal bending,
an a nteroposterior, almost exclusively lordotic
deviation and vertebral rotation in the hori­
zontal plane around the gravitational axis. The
presence of vertebral rotation is a diagnostic
criterion of structural bends. A number of
subtypes are differentiated within idiopathic
structural scoliosis as regards the onset, local­
ization and prognosis. Some of them appear
early, and progress rapid ly, causing severe
spinal and chest deformities, whereas others
progress more slowly.
Etiopathology. The cause of idiopathic
structural scoliosis is unknown, as are the fac-
254 � 2 4 . D i s e a s e s of t h e s p i n e
tors responsible for the differences i n onset,
location and progress ofthe various bendings.
Many factors are suspected as being primary
causes of idiopathic structural scoliosis. The
more important ones are summarized below:
Genetic origins: There are certain facts,
such as the similar appearance in twins, and a
familial accumulation, which turn the atten­
tion to genetic factors . However in spite of the
indisputable presence of genetic factors, the
exact course of inheritance is not clear. It is
certain that scoliosis inheritance is not con­
nected with chromosome X. The presence of a
dominant gene is suspected, but polygenic in­
heritance is not excluded. The Scoliosis Re­
search Society has declared scoliosis to be a
genetic malformation. It must be emphasized
that idiopathic structural scoliosis is an iso­
lated developmental disorder. An isolated
manifestation is not typical of monogenic
(dominant, recessive and gender-related) in­
herited disorders, but rather of polygenic in­
heritance (see hip dislocation and clubfoot) .
Thus, instead of a multiple disorder, we are
faced with the consequences of the distur­
bance of a given developmental process.
It is also a fact that there are no maj or dif­
ferences in geographic incidence, which sug­
gests that environmental factors do not play a
significant role in the onset of idiopathic
structural scoliosis.
Growth. The connection between growth
and idiopathic structural scoliosis has been
known since the start of the 1 9th century.
Growth is a complex process that depends on
genetic, hormonal and environmental (health
and feeding) factors. Not much is known of
the complex regulation of the process. An
analysis of the connection between growth
and idiopathic structural scoliosis reveals
some striking connections :
- Growth is needed for the development
of structural scoliosis.
- The progression of an existing spinal
deformity accelerates in the period of ra­
pid growth.
- Small, mild curves attain a steady state
following the end of growth.
A discussion of growth necessitates men­
tion of the widely investigated hormonal fac­
tors. The extensive female predominance in
idiopathic structural scoliosis points to the
role of the estrogen metabolism, as does the
observation that, in severe, progressive cases,
hypogonadism is detected in girls (late devel­
opment of secondary gender characteristics,
late menarche) . However, no disturbance of
the secretion of sex hormones has been identi­
fied. The suspected disturbance of the estro­
gen metabolism does not explain either the
scoliosis occurring in boys or the progressive
scoliosis appearing at a younger age.
Connective tissue causes: The compo­
nents of the viscoelastic connective tissue in­
clude collagen and proteoglycans. The me­
chanical properties of the connective tissues
are determined by the distributions of these
components and the orientation of the fibrils.
Qualitative and quantitative changes in both
the collagen and the proteoglycans may be
recognized in idiopathic structural scoliosis,
and these connective tissue defects of genetic
origin are considered by many experts to be
the primary reason for idiopathic structural
scoliosis. It has not yet been determined
whether the changes perceived are causative
factors or secondary changes due to the effect
of the developed curvatures.
Neuromuscular factors : It is generally ac­
cepted that postural reflexes are the most im­
portant factors in the stability of the axial skel­
eton. A clear understanding of the muscular
function, and especially that of the para­
vertebral muscles, is essential for familiarity
with the development of the idiopathic struc­
tural scoliosis. Any neurological or muscular
disturbance in a growing subject is a factor
predisposing to scoliosis.
Accordingly, any subclinical neuro­
muscular dysfunction could be a primary
etiologic factor in idiopathic structural
scoliosis. Maschida presumed a central neuro­
muscular anomaly or asymmetry in the
proprioceptive reflexes as a causative factor.
Biomechanical factors: Many bio­
mechanical factors may be considered to be
the primary signal in the development of idio-

pathic structural scoliosis. The most impor­
tant is a decrease of physiologic curves, the
cessation of dorsal kyphosis, which is the ba­
sic, primary etiologic factor of idiopathic
structural scoliosis. From the Delmas formula
it is clear that, in parallel with a decrease in the
sagittal curves, the stability of the spine de­
creases, and if the load increases (an overload
triggered by fast growth), the response will be
lateral bending, i.e. scoliosis.
To summarize: Idiopathic structural
scoliosis is a genetically determined disease.
Both biomechanical factors and a genetic pre­
disposition play roles in its development. If
these two factors are present, hormonal and/or
connective tissue changes in the period of
growth may lead to the development of struc­
tural scoliosis.
Incidence. It is most common in child­
hood, before puberty. Curves exceeding 200
are observed in 0.5% of the population. It is
8- 1 0 times more common in girls than in
boys.
Depending on the time of onset of the
curve, idiopathic structural scoliosis can be
subdivided into groups, this traditional classi-
Fig. 24.21.
Mehta's costotransversal angle. The greater the dif­
ference between the two angles, the more likely
the progression of the scoliosis.
24. D i s e a s e s of t h e s p i n e � 255
fication possibly hinting a t the likelyhood o f
worsenmg.
Infantile scoliosis develops before the age
of 3 . The curves with the best prognosis here
are those without curvature extending to the
spine overall, while those with the worst prog­
nosis are the very short ones involving only
1 -2 segments.
The former of these two curves may be re­
garded as a manifestation of intrauterine com­
pression syndrome (the siebener, number 7
syndrome) and may be observed together with
the other symptoms of the intrauterine com­
pression syndrome : torticollis, skull asymme­
try, hip dysplasia, pelvic asymmetry, foot de­
formity, scoliosis and thoracolumbar kypho­
sis. These symptoms disappear by the age of
6, without treatment.
The second, much less common type is
malignant scoliosis. Huge curves may de­
velop by the end of growth, exceeding 1 000,
with associated cardiorespiratory, and often
neurological problems.
It is difficult to differentiate the two
groups by means of simple observation. Mea­
surement of the costovertebral angulation
(Fig. 24.2 1 . a) may help identify the progres­
sive variant. Malignant, untreated curves may
end up in severe neurological symptoms,
which practically never evolve in idiopathic
scoliosis. Thorough clinical tests may dis­
close the etiologic factor in a large proportion
of the curves.
Juvenile scoliosis develops between the
ages of3 and 1 0 years . These curves follow an
evolution scheme closely connected with the
onset of the curve. They dramatically worsen
in the period of puberty, and therefore a regu­
lar check-up is essential, every 3-6 months.
This is particularly important for double
curves, where the two curves compensate
each other and for a long time do not seem to
elicit maj or morphologic changes.
Adolescent scoliosis occurs after puberty.
It has a better prognosis, and the picture is not
so severe, as in the previously discussed
groups . However, it should be noted that ado­
lescent scoliosis undergoes a mild worsening
until ossification is complete.
256 � 2 4 . D i s e a s e s of t h e s p i n e

Localization. Various manifestations are not striking, because of the compensated

listed, the sequence reflecting the incidence state, and it can therefore be undetected for a
(Fig. 24.22.). long time. At its discovery, a large curve may
The thoracic curve is the most common already persist. The observation of sagittal
type, with the worst prognosis. It is situated curves is particularly important. The detection
between Th6 and 1 2, causing a severe chest of kyphosis in the spinal segment between the
deformity and breathing problems. It is gener­ dorsal and lumbar curves is an indication of a
ally right convex, and often accompanied by particularly bad prognosis as regards the
marked decompensation of the upper trunk. curve progression. Otherwise, this type has a
Combined scoliosis has a right convex moderate prognosis as regards the breathing
dorsal curve and a left convex lumbar curve, function and its progression.
which usually compensate each other. This is A lumbar curve is usually left convex with
the apex located at L2- 3 . It has a good progno­
sis in childhood. It is never accompanied by a
�( ( );;')'JJ
1)Jj/Al
breathing function impairment, though if it is
extensive, a marked pelvic tilt may occur.
=
From the age of 40, in the presence of even
o Cl
o Cl mild curves, degenerative changes may occur,
o o
o causing severe pain and disability.
o �
<6,-- The thoracolumbar curve with apex at
vIJ � �o
0 8 Th l l - 1 2 or L l -2 is generally right convex,
1::)
<::5
[5
A;) , and has a somewhat better prognosis, though
o
c2)' �' � a third of them exhibit marked progression.
� 8
o
c?' �gu Breathing function impairments are not typi­

~ �
cal.

A Clinical symptoms. In childhood the de­

a b
formity is the only symptom. The first note­
worthy sign is the flattening of the physiologi­
cal curves, leading to a flat back. The lateral

(( V)\ /n)')
L =�rV spinal curvature is notable only if it is exces­
sive. The height of the shoulders, the scapu­
lae, the trunk-arm triangle and the position of
o
[5 = the iliac crests are asymmetrical. On the con­
o
� vex side of the curve, the shoulder and scapula
'9 are higher; the scapula is elevated. The

§
13 ,,-
/ [] )"- "-j trunk-arm triangle is thinner and shorter, and
the iliac crest is less prominent (Fig. 24.23.).
�
�� �S
Ib The upper trunk is often decompensated.
Structural scoliosis is always accompa­

�
\ nied by a rib hump, which can easily be de­

�� tected and measured in the forward bending

position. A smaller rib hump is visible in
front, on the concave side, often causing dif­
c d
ference in appearance and size of the breasts,
Fig. 24.22.
evoking a marked psychological problem for
Main localizations of idiopathic structural scoliosis.
girls in puberty (Fig. 24.24.).
a: Right convex dorsal, b: Right convex dorsolumbal
The movements of the spine are apprecia­
c: Left convex lumbal, d: Right convex dorsal and
left convex lumbal scoliosis bly restricted; the change is proportional to
 24. D i s e a s e s of t h e s p i n e � 257

the extent of the curves. It can be assessed by

bending the spine laterally (bending test), and
by the axial traction of the head.
In childhood, the deformity is not related
to any pain. If pain sets in at an early age,
some cause other than scoliosis must be
searched for. At an older age, the frequency of
pain increases due to the secondary degenera­
tive changes. This is typical in lumbar curves.
Changes in pain intensity following pu­
berty are also related to hormonal changes.
During pregnancy and after menopause a pain
increase is to be expected.
One of the most important clinical signs is
an impairment of the breathing function. This
is significant for severe curves, when rest
dyspnea is often observed, due to two factors :
the chest rotation caused by the spinal defor­
mity, with the consequent lung compression
and restricted breathing motions, and the seri­
ous increase in pressure in the pulmonary cir­ Fig. 24.24.
culation, leading to a cardiac insufficiency. Appearance of vertebral rotation-, torsion and rib
hump in scoliosis.

Because of these two factors, in severe cases,

when the curves exceed 60°, the life expec­
tancy may be even 30% less than in the nor­
mal population. Most of these patients die
from heart failure.

X-ray signs: For the documentation of

scoliosis, bidirectional standing films (3 0x90
cm) are needed, including the pelvis and the
head. In the standing position the curves of the
loaded spine are easier to determine. The ex­
tent of the structural curve is measured on
anteroposterior films by Cobb ' s method. The
rotation is determined with the technique of
Nasch-Moe or Perdriolle. Assessment of the
shape of the vertebrae allows conclusions on
the structural changes due to deformities,
which are proportional to the extent of the
curve. The vertebrae are higher on the convex
side due to the growth asymmetry, and lower
on the concave side, where degenerative
Fig. 24.23. changes follow because of the increased pres­
Chief characteristics of scoliosis. sure. On the lateral films, the borders of the
258 � 2 4 . D i s e a s e s of t h e s p i n e

normal sagittal curves and possible changes
are detected.
To summarize: In the course of the normal
X-ray assessment, information is acquired on
the appearance of the scoliotic curve and pos­
sible associated changes (congenital anomaly,
trauma, tumor, bone dystrophy, etc.).
Therapy. Every step in the physical as­
sessment has the aim of the most precise diag­
nosis possible related to expected progression
of the curve.
Many experts have assessed the natural
history of scoliosis. It has been clearly estab­
lished that the possibility of the development
of scoliosis is already present at birth. The de­
formity emerges only in childhood, and its se­
verity is individually variable. Some curves
never worsen, whereas others undergo a dra­
matic progression at puberty.
The third group is, where the worsening is
linear, but the progress speeds up in puberty.
The cause of this dramatic progression at
puberty is simple. The peak in the growth of
the upper trunk and the spine at that time (Fig.
24.25.). Due to the rapid growth of the spine,
the worsening may reach 7-8° in a month in
this period. The rate of growth o f spine can be
observed from the appearance of the ossifica­
tion center of the iliac crest (Risser 's sign).
The end of ossification corresponds to the end
of the growth of the spine, and the end of the
rapid progression of the scoliotic curves.
It is essential to discover these curves
early, and to start the treatment as soon as pos­
sible, ignoring the arbitrarily outlined treat­
ment threshold (35-40°), in the hope of a
better outcome.
Idiopathic structural scoliosis can be
treated by conservative and surgical means,
the indications of which differ:
�
Conservative treatment:
Various corsets and plaster fixations are
used. The indications are as follows:
- Curves in the interval 1 0-40°
- Risser' s stages 0-3
- If surgery is contraindicated up to
skeletal maturity.
The various forms of conservative treat­
ment were introduced in the leading scoliosis
centers around 1 950, and both the techniques
and the results of corset production gradually
improved.

extent of curve .---. growth/year . o o -tO

Cob b O cm
Risser 5
20
� .

1
first periods
75 f- 15
�
Q onset of puberty q
. � 0
�
, ,

50 � ' 10

q
, '
'

�
,

0
,

0
,

25 t< 5

0
,

, If
year
I I
0 5 10 15
Fig. 24.25.
Connection of the progression of scoliosis with the growth and sexual development.

Corset treatment is currently recom­
mended in three groups of patients:
- Infantile scoliosis: In cases of malig­
nant, progressive curves, corset treat­
ment is the only therapeutic modality as
a supplement of plaster corsets until the
age when surgery can be performed.
- Juvenile and adolescent scoliosis:
Corset treatment may stop and/or
decrease the progression, leaving a
curve that can be tolerated well in
adulthood.
- Adult scoliosis: Their role is exclusively
functional.
The aims of corset treatment:
- To stop or decrease the worsening of the
curve, to decrease or eliminate the
increasing asymmetry of the individual
vertebrae.
� To enhance the compensation of the entire
spine, to transform a decompensated
single curve into two combined, well­
compensated curves, which are better
tolerated in the long run.
Fig. 24.26.
Milwaukee corset.
2 4. D i s e a s e s of t h e s p i n e � 259
� T o act o n the entire tru nk so a s to achieve
both cosmetic (rib hump, and shoulder
equalization) and functional (breathing
function) improvement.
Derotation is strived for with by posterior
pads in the horizontal plane. Derotation is
simple correction of the rib hump, which also
influences the sagittal and horizontal curves.
Basic types of corsets . The Milwaukee
corset was devised by Blount and Schmitt in
1 946 (Fig. 24.26.) for the postoperative fixa­
tion of scoliosis, and particularly that due to
poliomyelitis . It was introduced around 1 95 0
and was increasingly used for idiopathic
scoliosis too . The Milwaukee corset is the
prototype of active corsets . The rigid
head-holder creates a continuous stimulus to
make autocorrective movements; if the pa-
Fig 24.2�.
Boston corset.
Fig. 24.28.
Cheneau corset.
260 � 2 4 . D i s e a s e s of t h e s p i n e
tient relaxes the trunk muscles, the head
bumps against the head-holder.
The Milwaukee corset is used mainly to
treat infantile and juvenile scoliosis under
40°, since it does not affect the normal devel­
opment of the chest and does not harm the
breathing function. It is recommended in ado­
lescent scoliosis if the apex is above vertebra
Th6.
The Boston corset was created by John
Hall in 1 97 1 . It is posterior-opened, with one
layer, and is symmetrical in the sagittal plane;
it decreases lordosis (Fig. 24.27.). This type
of corset is used in thoracolumbar and lumbar
curves where the apex is below vertebra
Th l O .
The Cheneau corset (the official name i s
CTM = Cheneau, Toulouse and Munster,
where it was first introduced) is suitable for
the treatment of any curve under 40° (Fig.
24.28.).
The Charleston corset is used at night; it
overcorrects the spine into a convex direction.
It is indicated in highly mobile curves under
25°.
Similarly, the Spine Cor dynamic corset i s
recommended i n highly mobile curves under
20° and it uses elastic straps for correction. In­
tensive exercises are necessary.
Corsets are used for 23 hours daily, and re­
moved only at the time of washing. Exercises
are a necessity.
The aim of loosening exercises is to de­
crease the rigidity of the curve and of the rib
hump and to create dorsal kyphosis :
- Breathing exercises : These increase the
vital capacity and the mobility of the
ribs, and promote the symmetric
development of the chest.
- Overall upper trunk correction: The
patient must be aware of postural ab­
normalities and practice auto-correction
in order to create and actively protect the
physiologic curves.
- Elimination of the disadvantages of the
corset. General muscle training, kypho­
sis and breathing exercises.
- Psychological support for the child.
The value of physiotherapy is outstanding
in conservative treatment; no other method
can replace it. An alternative is regular swim­
ming, since the forces of gravity are then less­
ened and the muscles of the trunk and belly
are trained symmetrically. Exemption from
school exercises is not justified.
� Indication and stages of surgical
treatment
The first scoliosis operation was carried
out by Jules Guerin in 1 839 . He suspected
that the condition was of muscular origin and
performed percutaneous myotomy of the
paravertebral muscles.
The first fusion was done by Russel Hibbs
in 1 9 1 4 . His procedure and its modifications
were the accepted therapeutic methods for
about 5 0 years (Fig. 24.29.).
After loosening therapy for 1 year, in situ
spondylodesis followed without further cor­
rection. To protect the result, external fixation
was used until bony consolidation was
achieved ( 1 year). The outcome of this
2-year-Iong procedure was the prevention of
progression. No correction was accom­
plished.
The first real breakthrough was due to
Paul Harrington, who reported his procedure
for Heine-Medin scoliosis in 1 962. His opera­
tion (Fig. 24.30.) has become the gold stan­
dard for the treatment of spinal deformities
and especialJy scoliosis. He set the basic for
all operative corrections with his instrumenta­
tion. After 1 -2 months of active preparation
with traction, the curves are corrected surgi­
cally with distraction hooks inserted into the
end-vertebrae on the convex side. The correc­
tion may be enhanced by using compression
on the concave side. The postoperative treat­
ment is aimed at protecting the correction
achieved in the operation: 6-8 weeks of bed
rest in a plaster bed and a corset for 1 year.
The average correction achieved is 40-45%.
The greatest problem was the neurological
complications (6-8%) originating from the
distraction of the spine; moreover this device
permitted only a 2D correction, and thus flat
back frequently occurred.
 24. D i s e a s e s of t h e s p i n e � 26 1

Fig. 24.29.
Bony fusion without an implant in Barta's mod ification principle of the operation in drawing (a). The consid­
erable cu rve (b) could be red uced by prolonged traction (c). Fusion was then achieved done in situ without
an implant and with a bone graft (d). I n spite of the fusion and the lengthy conservative treatment, signifi­
cant scoliosis may still be observed 10 years after the operation (e).

Yves Cotrel and Jean Dubousset, moved a
great step forward by introducing a multi­
segmental corrective system in 1 98 3 , which
furnished completely new possibilities for the
treatment of spinal deformities. The basic fea-
ture in the Cotrel-Dubousset 3D corrective
technique is the correction of rotation, not
only in the horizontal, but also in the frontal
and sagittal planes (Fig 24.3 1 .) . This can be
achieved in a single procedure.

b c d

Fig. 24.30.
The essence and the result of the Harrington operation (a). Preoperative (b) and
postoperative a-p (c) and lateral (d) radiographs.
262 � 2 4 . D i se a s e s of t h e s p i n e

G G

�J
�\
Fig. 24.31.
Three-dimensional (3D) correction of scoliosis. Frontal, sagittal and horizontal correction of scoliosis in a single
proced ure. Through use of the lever arm principle, the sagittal correction can be increased.

Fig. 24.32.
Correction of scoliosis with a double cu rve in a 14-year-old girl. 54° dorsal right convex and 52° lumbar left
convex dou bled cu rved (a) scoliosis corrected in the dorsal segment to 10°, and in the lumbar segment to 4°
(b). The preoperative irregular sagitta l curves (c) are seen to be corrected postoperatively (d).

During the operation, the strategic points
of the curves are determined and fixed with
staples. A corrective rod, bent do as to con­
form to the expected postoperative curves, is
then inserted beside the spine and rotated by
90°, so that its curves turn into the sagittal
plane and the scoliosis is corrected. Using the
principles of the lever, the thoracic distraction
further corrects the dorsal kyphosis, while the
lumbar distraction further corrects the lor­
dosis. Derotation decreases the rib hump by
changing the position of the costotransversal
joints and ribs, indirectly proving the 3D cor­
rection.
The Cotrel-Dubousse 3D correction nor­
mally does not require preoperative loosening
treatment. In the event of a normal bone struc­
ture, the rods inserted on both sides of the
spine provide such a stabile fixation, that
postoperative external fixation is unneces­
sary, the patients are out of bed on the second
day and can resume school after 2 weeks (Fig.
24.32.) .
Indications of operation:
- A Curve exceeding lumbar 30°; in other
localizations, 40°
- More than 1 0° progression yearly.
- Risser's stages 4-5 .
Ifthe indication is well based, but the bony
age has not reached Risser 's stages 3 -4, ven­
tral corpodesis is performed. This is also the
recommended method at any age for curves
exceeding 90°.
In consequence of the extensive 3D cor­
rection during surgery, paralysis of the lower
limbs may occur because of cord or root in­
jury. There are two ways to avoid this during
the operation: to wake up the patient tempo­
rarily at the time of correction in order to
check on the movement of the lower limbs, or
to check the perioperative evoked electric po­
tential. In cases of motor abnormalities, the
correction must be decreased. The re­
transfusion of peri- and postoperative maj or
blood loss by autotransfusion (Cell-saver,
Solcotrans) may decrease the complications .
Antibiotic is necessary to prevent infection.
24. D i s e a s e s of t h e s p i n e � 263
24.3.2.2.2. Scoliosis of kno wn etiology
Here only those forms of scoliosis are in­
cluded which may be important for a general
knowledge of medicine. Spinal deformities
are frequent symptoms of various congenital
syndromes, and teratogenic and inherited dis­
eases (Marfan' s syndrome, general joint lax­
ity, neurofibromatosis, infantile cerebral pa­
resis, myelodysplasia and certain mucopoly­
saccharidoses). About 70 multiple develop­
mental anomalies are known where scoliosis
may be one of the signs.
� Congenital scoliosis
Congenital scoliosis can occur as a conse­
quence of growth asymmetry caused by a
congenital developmental anomaly. The word
congenital is slightly inappropriate, since at
the birth only the vertebral developmental
anoma ly is definitely present; scoliosis is not
a lways seen.
Incidence. The exact incidence is not
known, but it is presumed to be relatively rare.
The occurrence of known wedge vertebrae in
the dorsal spine is 0,05 %. , but it is probable
that the true occurrence is around 1 %.
Etiopathology. The cause of this spinal
deformity is known: growth asymmetry con­
sequent to a vertebral developmental error.
The reason for the vertebral developmental
shortcoming is unknown; it takes place be­
tween weeks 3 and 6 of pregnancy as a result
of some unknown noxa affecting the embryo.
The development of the central nervous sys­
tem, urogenital organs and heart also starts in
this period, and these systems too may be in­
volved.
The main types of vertebral developmen­
tal anomalies are the disturbances of segmen­
tation and formation. These two disturbances
are sometimes combined (Fig. 24.33.). The
disturbances of segmentation may be uni- or
bilateral. Defective unilateral segmentation
results in a unilateral block vertebra. On the
side of the bony union the vertebra does not
264 � 2 4 . D i s e a s e s of the s p i n e

a b c d e g
Fig. 24.33.
Types of wedge vertebrae.
Segmentation disturbance: a: Unilateral block vertebra. b: Bilateral block vertebra.
Formation distu rbance: c: Total wedge impression. d: Incarcerated wedge vertebra. e: Partially formed wedge
vertebra. f: Slight wedge vertebra. g: Combined segmentation and formation disturbance.

grow, whereas on the contralateral side the
growth is normal . Depending on the number
of segments involved, maj or curves may
evolve, finally reaching 50-60°. Bilateral seg­
mentation disturbances result in a complete
block vertebra, leading to a symmetric loss of
growth, but no curve.
Formation disturbances produce wedge
vertebrae. These may be fully developed
wedge vertebra (normal disc is present below
and over the wedge vertebra), incarcerated
wedge vertebrae (an atrophic disc is present
both below and above the wedge vertebra), or
partially developed and insignificant wedge
vertebrae.
The growth potential below and above the
fully developed wedge vertebra is normal, so
the expected growth on the convex side is
double relative to the concave side. This may
result in a severe curve (50-70°) by the end of
growth.
Incarcerated wedge vertebrae do not pro­
duce such severe curves, since the growth po­
tential of the atrophic discs is less.
The partially developed wedge vertebrae
do not give rise to a maj or curve worsening,
since a single normal disc is located below or
above with a normal growth potential. The in­
significant wedge vertebrae are not divided
from the normal vertebrae by discs.
The worst prognosis is expected in cases
of combined developmental anomalies, if a
unilateral loss of segmentation is associated
with contralateral wedge vertebrae even in
multiple segments. This may lead to a 1 00°
curve.
Clinical symptoms. Any segment may be
involved. The most common sites are in the
lumbar and cervical parts . Depending on the
number and location of the segments, huge
curves may develop, or the state may be fully
compensated. In the thoracic segment rib
synostosis may accompany the spinal
changes, but this does not influence the clini­
cal outcome.
Therapy. The treatment of congenital
anomalies is surgical. Conservative therapy is
only justified only in exceptional cases and in
order to gain time.
In cases of unilateral block vertebra,
contralateral fusion is to be performed at the
time of the diagnosis because of the expected
rapid progression. Cases of complete block
vertebrae need only observation.
It is most important to decide whether
fully-developed or incarcerated wedge verte­
brae are present. Fully-developed wedge ver­
tebrae demand surgical excision because of
the expected rapid progression (Fig. 24.34.).
In cases of incarcerated wedge vertebrae, sur-
 24. D i s e a s e s of t h e s p i n e � 265

gical intervention must be considered, de­
pending on the growth potential. The worst
prognosis is to be expected in cases of com­
bined developmental anomalies. Each devel­
opmental anomaly most be evaluated individ­
ually.
� Neuromuscular scoliosis
Neuromuscu lar deformities occur, when the
balance between the active and passive stabi­
lizing elements of the spine is disturbed.
The occurrence and progression of the de­
formities are similar in this group, despite the
diverse etiology. Neurological or central and
muscular or peripheral subgroups are to be
differentiated. The neurological subgroup,
which involves damage to the central nervous
system, is subdivided into conditions associ­
ated with upper or lower motor neuron dam­
age. The following abnormalities can result
from upper motor neuron damage : ICP,
syringomyelia, myelomeningocele and spinal
cord tumors. Lower motor neuron damage
causes are present in (Heine-Medin 's) polio­
myelitis and spinal muscular atrophy.

a b

c d e f
Fig. 24.34.
Wedge vertebra in an 8-year-old girl. ThlO wedge vertebra (a) and 3D CT reconstruction (b). After removal of
the wedge vertebra (c) segment fusion (d). The spine in saggittal plane before (e) and after (f) the correction.
266 � 2 4 . D i s e a s e s of t h e s p i n e
The muscular o r peripheral subgroup com­
prises the deformities due to arthrogryposis or
muscle dystrophies (e.g. progressive muscle
dystrophy) . These patients are often unable to
walk and are wheelchair-bound.
Both groups are characterized by
C-shaped thoracolumbar deformities affect­
ing the sacrum, which start at an early age and
progresses rapidly, ending up involving the
entire spine. Their progression does not cease
at the end of growth, which is a marked differ­
ence from idiopathic scoliosis.
Deformities of central origin are large­
arched, very rigid curves. The curves related
to peripheral factors are also large-arched, but
remain very mobile for a prolonged period.
"Collapsing spine" frequently develops,
which is difficult to manage despite the mo­
bile spine.
The management of neuromuscular defor­
mities is far from simple. The aim of conser­
vative treatment is to ensure normal sitting,
a b
Fig. 24.35.
Neu rofibromatosis in a 13-year-old girl. 108° dystrophic scoliosis (a) and 72° kyphosis (c), corrected surgically
to 35° (b) and 44° (d), respectively.
and additionally to control the curve, in an at­
tempt to delay the surgical intervention,
which is often unavoidable.
The most important aim of surgery is to
form a balanced, stable spine which will re­
tain its stability in spite of the missing active
elements. If the basic condition creates an in­
creased muscle tone, a corset is needed post­
operatively for I year. In paralytic cases, a
postoperative corset must also be considered,
which may help the patient regain balance.
� Neurofibromatosis
Neurofibromatosis is an autosomal domi­
nant progressive disease involving the ecto-,
meso- and endodermic tissues. Its incidence
of 1 in 3 000 is the same in the two genders
with a constantly high penetrance, but with
different expressivities. Genetically, there are
at least 4 types. 90% of the cases are classified
into the classical (van Recklinghausen 's)
group. Typically, subcutaneous neurofibro-
c d

mas, pigmented skin lesions (cafe au lait
spots) and pigmented iris haematomas
(Lisch 's nodules) are detected.
Besides the neurocutaneous manifesta­
tions in neurofibromatosis, skeletal changes
may occur in 1 0-50% of the cases, producing
mainly spinal deformities. The deformities
vary. On the basis of the bone dystrophy, they
are divided into two groups, differing in prog­
nosis and therapy. The nondystrophic curves
are very similar to those in idiopathic sco­
liosis, so their management is identical. The
dystrophic curves are short, involving 4-6 ver­
tebrae, and are extremely severe. Apart from
dystrophic bone changes (hypoplastic and/or
missing pedicles, tiny spinous, transverse pro­
cesses and excavated bodies), they are accom­
panied by severe kyphosis. The extensive
early lesions progress irresistibly, leading to
cardiorespiratory and neurological symp­
toms, despite the wide spinal canal. The treat­
ment of these deformities can be effective
only, ifthe early aggressive surgical treatment
is commenced in multiple steps, with ventral
corpodesis and posterior corrective spondylo­
desis (Fig. 24.35.). A corset is contraindicated
in this condition.
� Mesenchymal diseases /
Mar/an 's syndrome
Marfan 's syndrome is an autosomal domi­
nant disease with differences in expressivity
and high variability. It is caused by qualitative
and quantitative changes in fibrillin, a glyco­
protein component of the microfibrillary fi­
bers. While the connective tissue is involved
in the entire body, the main clinical signs are
present in the eye, and cardiovascular and
skeletal systems (Chapter 1 3).
The incidence of spinal deformities is high
(40-80). Scoliosis is most often observed, but
other changes (spondylolysis, spondylo­
listhesis, spina bifida and facet subluxation)
are also possible.
Mar/an 's scoliosis differs from the idio­
pathic form. The gender distribution is equal.
Infantile and j uvenile forms are frequent.
Rapidly progressing double curves most often
appear (right convex dorsal and left convex
2 4. D i s e a s e s of t h e s p i n e � 267
lumbar), with severe rigid deformities. The
physiological curves are frequently inverted,
causing severe breathing failure.
Deformities in Mar/an 's syndrome should
be treated with early combined surgery: ven­
tral release and corpodesis and posterior cor­
rective spondylodesis. Conservative treat­
ment is justified only if surgery is contraindi­
cated.
� Metabolic diseases / Rickets scoliosis
Rickets scoliosis is a spinal deformity in
rickets and osteomalacia due to a disturbance
of the vitamin D supply, absorption or metab­
olism (see Chapter 1 4) . The developing bones
lose their solid structure, and various other
spinal skeletal deformities may arise: caput
quadratum, craniotabes, Harrison groove,
'rickets rosary' rib changes, sword-sheath
tibia, etc. The spinal deformities first appear
as lower dorsal and lumbar kyphosis (sitting
hump). Scoliosis follows. The earlier the rick­
ets starts, the more severe, short and rigid the
curve produced. The trunk becomes shorter
due to compression of the vertebral bodies.
In severe cases, the disproportion between
the trunk and the limbs is noticeable. Com­
pression of the anterior or lateral elements of
the vertebrae damages the growth plates and
the ossifying centers , so the curve therefore
progresses.
Rickets due to a vitamin D deficiency is
currently rare; it can be cured by means of vi­
tamin D treatment before the deformities de­
velop.
24.4. Congenital abnormalities
24.4. 1. Spondylolysis,
spondylolisthesis
I n spondylolysis the thinnest part of the ver­
tebral arch, the interarticular part, exhibits a
uni- or bilateral division. In the event of bilat­
eral interarticular lysis, the involved vertebra is
divided into two parts. The frontal part com­
prises the vertebral body, the adjacent parts
of the arch, the transverse processes and the
268 � 2 4 . D i s e a s e s of t h e s p i n e
Fig. 24.36.
Spondylolysis i n pars interarticularis.
superior articular processes. The posterior
part contains the posterior a rticular processes,
the spinous p rocesses and the corresponding
part of the arch (Fig. 24.36.).
Spondylolisthesis may occur, when a vertebra l
body (together with th e spinal segment
above it) slips forward on the vertebra below.
The most common cause of spondylolisthesis
is bilateral spondylolysis. Rarely, the slip may
take place backward direction; this is cal led
retrolisthesis.
Incidence. The incidence of spondylolysis
and spondylolisthesis varies in different geo­
graphic areas. The incidence in Europe is 6%,
among Eskimos it is 26-27%, and in certain
isolated ethnic groups it may even reach 40%.
The incidence also varies with the age. In Eu­
ropean newboms, spondylolysis is not de­
tected, whereas the incidence at the age of 6 is
5%, and by the end of growth it attains the fi­
naI 6%.
Etiopathology. Many theories have been
put forward to explain the development of
spondylolysis and spondylolisthesis. In the
oldest theory, the tension exerted on the
interarticular parts because of a developmen­
tal anomaly obstructs the normal ossification
from the double ossifying centers of the verte-
bral arches, creating interarticular lysis. This
theory is supported by only a few data.
Due to the erect posture, the interarticular
part is exposed to an increased load and is
overloaded, and this is further aggravated by
possible hyperlordosis. This may explain why
Scheuermann' s disease is accompanied by
spondylolysis and spondylolisthesis in 50%
of the cases. Again due to the erect posture,
the interarticular part may be secondarily
overloaded and, because of the repeated
microtraumas, a fatigue fracture may occur,
causing spondylolysis and spondylolisthesis.
Repeated microtraumas occur in individu­
als who repeat extreme movements that affect
their lumbar spine (extreme flexion - exten­
sion) : competitive gymnasts, kayak - canoe
competitors and athletes.
The disease was classified be Wiltse into 5
types (Fig. 24.37.) :
� Dysplastic type: A multiple develop­
mental anomaly of the lumbosacral seg­
ment is present as a predisposing factor.
The spondylolysis develops as a con­
sequence of the insufficient development
of the proximal part of sacrum (cupola
formed, rounded, hypoplastic base of the
sacrum), and of the inferior articular
processes of vertebra L5, promoted by the
enhanced pelvic incidence accompanied
by the increased sacral tilt (sacrum
acutum) . Besides the dysplastic inferior
articular processes of vertebra L5, occult
spina bifida is often detected. This type is
more common in girls and is detected
around the age of 6.
� Isthmic type: This is the most common
type of spondylolisthesis and can be
further divided into 3 subtypes. The lytic
subtype is a fatigue fracture of the inter­
articular parts. Elongation of the inter­
articular part without lysis may also occur.
The third subtype involves an acute
fracture of the interarticular part. The
isthmic type is more common in males,
and has a worse prognosis in females with
a greater likelihood of progressing. The
isthmic type often causes lumbar in-

stability. The earlier the deformity occurs,
the more severe the clinical symptoms are.
� Degenerative type: This deformity is
usually a limited slip, observed as a
consequence of chronic segmental in­
stability due to disc degeneration (spon­
dylosis) and facet joint changes (spondyl­
arthrosis). Retrolisthesis is also frequent.
It is fairly common in women over 50 .
� Traumatic type: Spondylolisthesis i s
caused b y a vertebral fracture a t some
other site than the interarticular part. It is
rare.
1 2
3 4
5 6 7
Fig. 24.37.
Types of spondylolisthesis.
1: dysplastic, 2: degenerative, 3: trau matic, 4: pat ho­
logic, 5: pars interarticu lar lysis, 6: elongated, but
intact pars interarticular, 7: trau matic pars inter­
articular fracture.
24. D i s e a s e s of t h e s p i n e � 269
� Pathologic type: This is due to weakening
of the interarticular part in localized (tu­
mor) and/or generalized (osteoporosis or
inflammation) bone diseases.
Localization. Spondylolysis and spon­
dylolisthesis may occur at any age and affect
any spinal segment. Most often (80%), L5
slips on the sacrum. L4 slips occur in 1 5% of
the cases. Slips at L3 and L2 have a frequency
less than 1 %. In the other segments, the rate of
occurrence of spondylolisthesis is around 5%.
Clinical symptoms. Spondylolysis and
spondylolisthesis often present without symp­
toms . The symptoms mainly occur in child­
hood and adolescence because of the changes
in the dynamics of the lumbar spine, resulting
in segmental instability, and present as acute
or chronic lumbago. The most typical symp­
tom is low back pain after long standing or af­
ter extensive flexion - extension of the lumbar
spine; this pain increasing in response to
physical activity. The pain is not radicular
pain, but often radiates to the buttock or the
thigh. The paravertebral muscles, the hip ex­
tensors and the hamstrings are very tight, re­
sulting in a special gait with short steps, with
externally rotated legs, as a consequence of
the limited hip flexion. Neurological signs,
radicular pain or signs, and reflex changes de­
velop only in cases of excessive slip or disc
herniation. Pseudoradicular pain may occur
regularly.
Clinically, increased lumbar lordosis is
found at the location of slip step formation.
The spinous processes of the involved verte­
brae and the paravertebral area may be tender.
In one-third of the symptomatic cases, minor
lumbar scoliosis may be present.
The symptoms may worsen with age, but
the radiological signs do not demonstrate
close correlation.
The patients do not tolerate load-bearing
well : a sudden, chiefly rotational motion may
produce heavy pain. This may be related to
the segmental instability.
X-ray signs. The anteroposterior and lat­
eral views are not always sufficient. The
2 70 � 2 4 . D i s e a s e s of t h e s p i n e

Fig. 24.39.
Dittmar's view of spondylolysis.

Fig. 24.38. slipped vertebra is located in the pelvis in

Severe spondylolisthesis: the picture resembles a re­ front of the vertebra below it, this is called
verse Napoleon hat.
spondyloptosis (Fig. 24.40.).
Treatment. Spondylolysis and non­
anteroposterior film shows the inverted Napo­ progressive insignificant spondylolisthesis
leon ' s hat projection of the vertebrae only if are often symptom less and do not require
the slip is severe (Fig. 24.38.). Other develop­ treatment. Spondylolysis cases discovered in
mental anomalies are often seen, e.g. spina
bifida. The lateral view unequivocally reveals
the slip (spondylolisthesis) and tearing of the
interarticular part is often seen. The increase
in the axis of the sacral slope induces hyper­
lordosis, and a hypoplastic, rounded sacrum
base is frequent.
The region of the interarticular part and the
tear is observed in oblique, Dittmar ' s views
(Fig. 24.39.). The borders of the tear may be
irregular, with a relatively narrow gap indica­
tive of a fresh lesion. When the defect has per­
sisted for a long time, the gap is wider, and the
edges are rounded and sclerotic, proving the
presence of pseudoarthrosis.
To establish the extent of slip,
Mayerding 's scale is used, in which the sacral
plateau is divided into 4 equal parts. The dis­
placement in grade I is 0-25%, in grade 11
26-50%, in grade III 5 1 -75% and in grade IV Fig. 24.40.
76- 1 00%. In the most serious cases, the Spondyloptosis, lateral view.

a b
Fig. 24.41.
Isthmic reconstruction (Jakab's screw).
a: Preoperative X-ray; the lysis is clearly visible. b: Postoperatively after the insertion of screws. c: Screws i n
oblique view.
children must be followed up clinically and,
depending on the individual findings, X-ray
images are taken to check on the progression
of the slip.
The complaints present as instability-in­
duced lumbago. The treatment is mainly con­
servative. Nonsteroidal anti-inflammatory
drugs and muscle relaxants are prescribed
and, following a temporary reduction in phys­
ical activity, back and belly muscle exercises
are encouraged. Physiotherapy may also be
employed. Physical education at school is al­
lowed, but overloading and heavy spinal
flexion - extension movements should be
avoided.
The treatment is naturally dependent on
the extent of the spondylolisthesis. In cases in
which there is only a minor slip, conservative
methods predominate with the use of an
antilordotic corset. This device decreases the
lordosis and possibly the angle of the sacral
slope.
If the complaints are not alleviated by con­
servative methods, the slip progresses; if the
slip is extensive (grades Ill-IV), surgical treat­
ment must be contemplated. The progression
24. D i s e a s e s of t h e s p i n e � 271
c
of spondylolisthesis is likely in early adoles­
cence, in women, in dysplastic slips, in grade
Ill-IV cases and when there is a high sacral
slope (sacrum acutum) .
The essence of surgical treatment is to
eliminate the instability induced by the
spondylolysis and spondylolisthesis. Reduc­
tion of the slip is not attempted.
In mild slips, isthmic reconstruction is em­
ployed: direct compressive arch synthesis is
achieved with a malleolar screw introduced
through the lysis of the interarticular part, fol­
lowing resection of the pseudoarthrosis (Fig.
24.4 1 .) .
Instability may also be cured by in situ fu­
sion ( Wiltse 's method) without internal fixa­
tion. Fusion between the sacrum and the
arches and transverse processes of vertebrae
L4-5 is performed with bone grafts taken from
the iliac crest. The patient is mobilized in a
corset 3 weeks after the operation. Bony fu­
sion is expected in 3-6 months.
Fusion can also be effected with implants.
The vertebrae involved are fixed with trans­
pedicular screws and rods; stable fixation en­
hances bony fusion (Fig. 24.42.).
272 � 2 4 . D i s e a s e s of t h e s p i n e

a b

Fig. 24.42.
Spondylolisthesis, segment fixation.
a: Preoperative X-ray. b: Postoperative X- ray.

On use of the implant, reduction of the

spondylolisthesis is possible, but this is not
the primary aim of the operation. When the
spondylolisthesis is to be reduced, the poste­
rior arch of the reduced vertebrae must be re­
moved in order to avoid nerve compression.
In cases of posterior lumbar interbody fu­
sion, discectomy is carried out from a poste­
rior approach, retracting the cauda equina,
and interbody fusion is accomplished with a
bone block(s) inserted in place of the disc.

24.4.2. Sacralization, lumbarization

The lumbosacral region, called the "rest­
less point" of the spine, is the most frequent
site of developmental abnormalities.
In sacralization, the transverse processes
of vertebra L5 are longer and wider, like but­
terfly wings, and the vertebra is partially or
completely fused with the sacrum. Bilateral, Fig. 24.43.
symmetric fusion is usually symptomless. Sacralization. The enlarged transverse L5 process
However, due to the uneven load-bearing uni- touches the base of the sacru m on the right.

lateral change, more often leads to spondy­
losis in the upper levels and complaints.
In partial sacralization, the common cause
of the pain is the neoarthrosis created between
the enlarged and extended transverse process
and the sacrum lateral mass (Fig. 24.43.).
Lumbarization is present when the first sa­
cral segment is not ossified with the other
parts of the sacrum but remains partially or
fully separated; its appearance is similar to
that of the vertebra L5 (Fig. 24.44.). The con­
dition may be symptomatic because of the
intersacral neoarthrosis or the improper load­
ing that induced lower lumbar spondylosis.
Since this defect may be symptomless for de­
cades, it is not generally agreed whether the
symptoms are caused by the lumbarization or
by other factors .
Fig. 24.44.
Lumbarization
24 . D i s e a s e s of t h e s p i n e � 273
Clinical symptoms. In both abnormali­
ties, the complaints usually appear gradually
as lumbago in middle age. The pain is boosted
by physical activity. No neurological signs are
detected. Typical lumbago may be present.
The paramedian points are usually tender,
mostly unilaterally. In sacralization, local ten­
derness is felt uni- or bilaterally over the at­
tachment of the transverse process and iliac
crest (5-6 cm laterally from the midline. This
pain increases during bending to the involved
side and on rotation of the trunk (Baastrup ' s
sign).
Therapy. In most cases, conservative
treatment is needed, with temporary rest,
nonsteroidal anti-inflammatory drugs, muscle
relaxants and pain killers. The complaints are
improved by physiotherapy too. As soon as
the pain has subsided, regular exercises are
recommended. Temporary use of a lumbar
corset may alleviate the complaints. In the
complaints are stubborn and conservative
treatment fails, surgery must be considered. In
sacralization causing neoarthrosis, an en­
larged transverse process may be removed. In
other cases posterolateral fusion (spondylo­
des is) is recommended, with or without inter­
nal fixation.
24.4.3. Occult spina bifida
The relatively common developmental
anomaly is generally discovered accidentally.
It usually appears on vertebra L5 or S I . On
S I , this anomaly has no clinical effect, since it
can not disturb the stability of the firm sacral
bone. Occult spina bifida on L5 is generally
presumed to exert any clinical effect either,
but some claim that any variation or weaken­
ing of the static ally overloaded vertebra L5
may lead to complaints. Conservative care is
justified. If this fails, the cause of the back
pain may be different (see myelodysplasia).
2 74 � 2 4 . D i s e a s e s of t h e s p i n e
24.5. Other diseases
24.5. 1. Baastrup syndrome
(interspinous arthrosis)
The spinous processes are in contact with
each other, mostly in the lumbar section, and
the development of neoarthros leads to
arthrosis. The pain sets in on rotation and rec­
lination of the spine. The secondary form may
occur in spondylosis (see lumbar spondylo­
sis). It reacts well to conservative treatment;
the tip of the spinous processes may be
trimmed in some cases.
24.5.2. Sacrum acutu m
Opinions concerning to this entity differ.
Sacrum acutum is present if the slope axis of
the sacrum greatly increases, and it becomes
almost horizontal. The cause is presumably
the chronic and increased tension of the liga­
ments and of the disc at the lumbosacral level.
This deformity may accompany other defor­
mities (see spondylolisthesis dysplastic
form). The treatment is conservative. In the
pain-free period, exercises are advocated with
a view to decreasing the lumbar lordosis.
24.5.3. Coccygodynia
This is a condition involving coccygeal
pain, which may originate in various ways:
� While very thin individuals are sitting, the
prominent coccyx may chronically irritate
the skin and periosteum.
� A fall onto the buttocks may result in local
contusion and periosteal irritation, or
ventral dislocation may take place if a liga­
ment is tom in the sacrococcygeal j oint.
Clinical symptoms. Pain felt in the sacro­
coccygeal j oint, increases during sitting.
There is often some direct trauma in the his­
tory. Defecation sometimes is extremely pain­
ful. Lying and walking do not cause is symp­
toms .
Examination reveals tenderness and pain
in the sacrococcygeal joint. At times, the pain
increases on passive movement ofthe coccyx.
The X-ray picture sometimes indicates a
fracture or ventral displacement of the coc­
cyx. Most often, however, the X-ray result is
negative. Its main use is to exclude other pa­
thologies (tumor or infection) . Rectal exami­
nation is advised in every case.
Treatment. The complaints frequently
cease spontaneously or following conserva­
tive treatment. Individuals are often recom­
mended to use a ring cushion. Local physio­
therapy, nonsteroidal anti-inflammatory
drugs, and a lidocaine - local steroid injection
may succeed. In stubborn cases, surgical re­
moval of the coccyx may be the choice.
24.6. Degenerative disorders
of the spine
The importance of degenerative disorders
of the spine in medical practice is due to two
important and typical facts : 1 . Either acute or
chronic spinal pain may lead to a disability,
and impair the quality of life. 2. The risk of
damage to the spinal chord and radices, be­
cause of their special anatomical characteris­
tics in spinal diseases. The common occur­
rence of pain and neural signs urges both pa­
tient and doctor to seek the effective and suc­
cessful treatment of these conditions.
As far as the epidemiological significance
is concerned, in the USA in 2002 spinal con­
ditions were listed as the third most common
among the conditions that require health care.
As regards health care expenses, they have
been in a lending position for years.
Modem spinal surgery combines orthope­
dic and neurosurgical techniques, but also
makes use of the instrumentation of general,
thoracic, vascular and plastic surgery. It is
generally available in well-organized opera­
tive centers . The characteristic features in­
clude the extensive use of implants (metal or
plastic devices), advanced medical devices
(special motors, computer-guided operative

tools, endoscopes, lasers, neurostimulators,
etc.) and products of the biotechnological in­
dustry (produced from natural or artificial
sources and tissue culture).
24.6. 1. Degenerative spinal
disorders
The spine sustains structural changes in
the course of aging. These changes progress at
a segmental level, and the sum of the changes
of the individual segments leads to the typical
degenerative deformation of certain segments
and ultimately of the entire spine.
24.6. 1 . 1 . Degenerative p rocess
of the disc
The lumbar disc in healthy young adults,
with its well-hydrated (physically "perfect
gel") nucleus pulposus, the densely colla­
gen-fibered annulus fibrosus and the well-de­
fined cartilage-covered end-plates, fulfils its
purpose from every aspect, providing an elas­
tic and firm junction between the vertebrae,
which react properly to the axial load (mainly
Fig. 24.45.
Degenerative process of the movement segment.
a: Osteophyte formation (arthrosis). b: Osteophyte
24. D i s e a s e s of t h e s p i n e � 275
gravitation). At the end of the third decade,
fine, concentric (sometimes radial) fissures
appear in the lamellar system of the annulus,
indicating that the nucleus pulposus can no
longer distribute the circular pressure prop­
erly. The "perfect gel" of the nucleus pulpous
is gradually dehydrated, and during the fourth
decade the water loss is significant. As the age
progresses the nucleus - annulus border be­
comes ill-defined, and the inner rings of the
annulus loosen because of the numerous ra­
dial fissures. If the fissures between the
lamellae reach the outer rings, fibrous tissue
proliferation starts in the center of the disc.
This scar tissue fills the interlamellar space
and the entire nucleus pulposus, and nerve ter­
minals are introduced.
The loosening of the lamellar system of
the annulus fibrosus elicits changes in the me­
chanical properties of the disc. During flexion
- extension and rotation, the tension of the
lamellar system decreases, its motion-limiting
role also decreasing. The dehydrated nucleus
pulposus and the annular system with its im­
paired elasticity are decisive factors contrib­
uting to a loss in height of intervertebral
space. This loss has two consequences: 1 . the
relative tension of the longitudinal ligaments
diminishes, and 2. the j oints of the segment
exhibit first hypermobility, and then sub­
luxation (Fig. 24.45.).
24.6. 1.2. Disc prolapse, hernia
If the elements of the disc are detached from
their original position and invade the spinal
canal, the resu lt is a herniated disc. Depending
on the relation with the spinal anatomy, cen­
tral, mediolateral, foraminal and
extraforaminal hernias may be defined (Fig.
24.46.).
If a piece tears off the disc completely, the
process is called sequestration. If the pieces of
the destroyed lamellar system do not enter the
canal, but press against the posterior longitu­
dinal ligament and narrow the canal, it is
called protrusion.
2 76 � 2 4 . D i s e a s e s of t h e s p i n e

tures of the segment, and by the movement

limiting role of the uncinate processes.
Spinal cord compression may result in an
irreversible loss of function within a few
hours. Fortunately, this complication is rare.
From a practical medical aspect, lumbar
hernias play a prominent role. Their occur­
rence in the individual lumbar segments var­
ies considerably. The most common sites af­
fected are the lowermost two segments above
the sacrum: herniation of discs L4-5 accounts
for 60-0% of all lumbar hernias. Herniation
a above L3 is very rare.

24.6. 1.3. Degenerative spine instability

Due to the structural changes caused by

degenerative processes, the movement of a
segment may exceed the physiological limits.
This is called instability. Segmental instabil­
ity is usually chronic (disc degeneration), but
may appear in acute form (herniated disc or,
b
vertebral fracture).
Fig. 24.46.
Segmental instability activates the resta­
Herniated disc types by anatomical localization.
bilizing mechanisms of the body. As a result
a: Central hernia, b: Mediolateral hernia
of these processes, canal stenosis, and irrita­
tion or compression of neural components
may arise, causing clinical symptoms.
A herniated disc (either protruded or se­ Types of lumbar spinal stenosis (by
questrated) is regarded as a cause of compres­ Arnoldi)
SlOn.
This condition is accompanied by pain, 1. Congenital developmental anomalies
movement restriction and occasional neuro­ 1.1. Idiopathic
logical signs. The sequestrum first elicits in­ 1.2. Known etiology (achondroplasia,
flammation of the perineurium (edematous etc.)
thickening), and may then may disturb the 2. Acquired types
neural function. The sensory quality may 2. 1. Degenerative
change, the motor function of the muscles 2.2. Combined degenerative and
may diminish, and the reflexes may change. congenital
At the same time, the short interspinal mus­
2.3. Spondylolysis
cles may suffer a reflex (lasting) spasm, seem­
2.4. Iatrogenic
ingly to protect the segment from movement.
2.5. Post-traumatic
Herniated discs in the neck and back may
2.6. Others (metabolic, tumor, etc.)
cause symptoms by direct compression of the
spinal cord. Herniated discs in the dorsal seg­
ment are rare (this segment has limited mobil­ � Degenerative spinal stenosis
ity) . The modest symptoms in the cervical By the time of skeletal maturity, the
segment are explained by the special struc- sagittal diameter of the bony canal has at-

tained its final size (Fig. 24.47. a). This is the
distance from the posterior wall of the verte­
bra to the lamina. This distance is permanent
and never changes. This is the anatomic diam­
eter. The functional diameter is the distance
between the posterior wall of the
intervertebral disc and the ligament flavum
system (Fig. 24.47. a). This length naturally
changes during movement: it increases in
flexion (kyphosis) and decreases in extension
(lordosis). The degenerative processes pro­
duce excessive tissue mass (protrusion, liga­
ment thickening, etc.) at the height of the disc,
resulting in a decrease in the functional diam­
eter (Fig. 24.47 b).
The degeneration of intervertebral discs
progresses without any clinical symptoms,
until the spatial demands of the intraspinal
neurogenic elements begin to inhibit the phys­
iologic function.
a
b eral parts of the lumbar spine, including the
Fig. 24.47.
a: Normal physiological diameters of the spinal canal
(1. anatomical diameter, 2. functional diameter )
b: Fu nctional diameter of the spinal canal (3) spinal
stenosis.
2 4. D i s e a s e s of t h e s p i n e � 277
If a functional block vertebra is created at
the end of the process in such a way that both
the dural sac in the center of the spinal canal
and the nerve root laterally can fulfil their
tasks, then the process ends without symp­
toms . However, in the course of degeneration,
disproportion can occur in any phase between
the spatial demands of the neurogenic ele­
ments and the space narrowing induced by the
process.
According to the modem approach, a her­
niated disc is considered to be a special form
of degenerative spinal stenosis, where the nar­
rowing of the spinal canal occurs relatively
rapidly, and the symptoms are therefore ac­
centuated.
24.6. 1.4. Clinical exam ination of
degenerative spinal d isorders
Pain of spinal origin.
In the examination of a spinal patient, the
first step is the thorough recording of the his­
tory. One of the most important points is to
verify the circumstances of the onset of the
pam.
Besides its onset and its duration, spinal
(and low back) pain is characterized by its lo­
calization and radiation. Low back pain is
called local if it is present in the middle or lat­
lumbosacral area and the sacroiliac j oints. The
radiating pain starts in this area and involves
one or both lower limbs.
In the assessment of local pain, tenderness
and pain are sought over the spinous pro­
cesses in the median or paramedian line and
over the path of the sciatic nerve. Valleix' s
points are located between the buttock and the
greater trochanter, where the nerve leaves the
pelvis; in the middle third of the thigh posteri­
orly; and behind the fibula head correspond­
ing to the peroneal nerve. Tenderness at these
points is an indication of root irritation, but
these are not genuine radicular signs. The sa­
croiliac j oints, the sites of origin of the gluteal
muscles, may also be tender.
278 � 2 4 . D i se a s e s of t h e s p i n e
The pain syndromes involving the neck,
shoulder girdle and one or two upper limbs are
referred to as cervicobrachialgia.
Lumbago is a Latin word meaning low
back pain. If it is accompanied by radiating
pain to one or both lower limbs, it is called
lumboischialgia.
One of the most frequent reasons why the
patient consults the doctor is low back pain.
Three types may be distinguished on the basis
of the etiology:
- Vertebrogenic
- Extravertebral, somatic
- Extravertebral, psychological low back
pam.
Vertebrogenic low back pain is most often
a symptom of a degenerative process. It inten­
sifies in response to physical loading (e.g. a
standing posture), and is alleviated at rest (an
unloaded state). It is important if the patient is
disturbed by pain while sleeping. Pain in­
creasing at night excludes a degenerative ori­
gin, and inflammation or tumor is possible.
This is particularly the case when the pain is
accompanied by general symptoms (fever,
malaise, loss of weight, etc.), and conven­
tional pain-killing methods are not effective.
If local pain is accompanied by radiating
pain, an accurate assessment can provide im­
portant information. If the radiation corre­
sponds to any dermatome, typical radicular
pain is present, and symptoms of ·nerve in­
flammation are found. (Such patients have a
typical sign, functional scoliosis, the so called
"antalgic posture".) Radiculitis is caused most
often by some form of spinal stenosis, and if
radicular pain is accompanied by sensory or
motor loss signs, there is usualJy a herniated
disc in the background.
Extravertebral, somatic low back pain is
usually caused by some abdominal problem,
e.g. a nephrolith, pyelitis, a renal tumor, or in­
flammation of the gall bladder, colon or ova­
ries. Among women, low back pain is very
frequent during the periods. Aneurysm of the
abdominal aorta may also cause long-lasting,
excruciating low back pain.
Extravertebral, psychological low back
pain is one of the most characteristic manifes­
tations of psychosomatic conditions. These
patients may have already experienced real
somatic low back pain, and later the recollec­
tion of their suffering is their way out of a psy­
chological conflict.
Inspection. The appearance of an un­
dressed patient may be highly informative. If
the patient is able to stand, the antalgic (pro­
tecting) posture, may be striking as may be an
expressive paravertebral muscle spasm if the
patient is recumbent. In lumbar stenosis, the
lordosis is smoothed, fixed lumbar kyphosis is
sometimes seen. Degenerative spondylo­
listhesis sometimes produces a step sign. In­
activity atrophy may be marked in the lower
limbs.
The gait of the patient should be observed
with special care. The cause of a limp
(claudication) may be a blood supply loss (ar­
teriosclerosis), or a lower limb joint distur­
bance (e.g. hip osteoarthrosis). A typical sign
of lumbar spinal stenosis is the spinal
("neurogenic, intermittent") limp. The motor
power of the lower limbs undergoes a diffuse
deterioration after a certain walking distance.
The patient first starts limping, and is then
forced to stop in order to avoid falling. Fol­
lowing a short period of leaning forward or
sitting, the muscle power returns and the pa­
tient can carry on walking.
Neurological state. Following the inspec­
tion and the observation of the gait of the pa­
tient, and the recording of the cervical and
lumbar (active) ranges of movement, the neu­
rological state of the trunk and the limbs is as­
sessed. This means a basic assessment of the
sensor, motor and reflex functions.
Symptoms of monoradicular lumbar defi­
cits with low back pain point to nerve com­
pression (Table 24. 1 . and Figs. 24.48., 24.49.
and 24.50.) .
The presence of neuritis is demonstrated
by stretching provocation of the nerve. In the
case of L4 neuritis (the main bundle of the
femoral nerve), forceful bending of the knee
 24. D i s e a s e s of t h e s p i n e � 279

Table 24. 1.
Symptoms of mono radicular lu mbar
Level o f root Frequency Pain Reflex deficit Para-, Motor change
involvement hypesthesia (deficit, paresis)

5 1 root (disc 37-43% posterior thigh, Achilles 5 1 dermatome flexor hallucis

v. toe
prolapse L5-S1) lateral leg, (lateral leg) longus, triceps
su rae, tiptoe is
d ifficult
L5 root (disc 53-59% anterolateral none, oce. weaker anteromedial m. extensor
prolapse L4-L5) leg, big toe or missing tibialis leg hallucis longus,
posterior jerk m. extensor
digitorum, walking
on heels is d ifficult
or impossible
L4 root 1-2% anterior thigh, patella reflex is anterior thigh, m. quadriceps
(disc prolapse medial leg, weak or missing medial leg atrophy, extension
L3-L4 or L4-L5) femoral nerve of knee is weak,
sign knee instability
Central protrusion 0,5- 1% radicular pain reflex deficit both paresthesia in d istu rbance of
of higher lumbar radiating both lower limbs area of rectum, urinary and
discs - cauda lower limbs private parts defecation
equine syndrome

of the patient in a prone position provokes
pain in the inguinal region (this is the femoral
test).
Radiculitis of roots L5 and S I , the main
bundles of the sciatic nerves is called sciatica.
The stretching test of the sciatic nerve relates
to the classical Lasegue sign (straight leg rais­
ing; Fig. 24.51 .). With the patient in the su­
pine position, the hip is gradually flexed with
the knee extended. This flexion is normally
possible to 90°. The tension of the sciatic
nerve increases in direct proportion to the ex­
tent of flexion. In cases of inflammation, rais­
ing of the straight leg through an angle of
1 5-20° may already produce pain, which be­
comes more severe on further flexion. In
Bragard' s test, the straight leg raising test is
performed until the pain limit, and the foot is
then passively dorsiflexed to increase the ten­
sion of the nerve, producing even worse pain.
An increase in abdominal press are (coughing
or sneezing) also increases the pain.
Complaints related to defecation or urina­
tion should be evaluated with special care.
Overflow incontinence, urine retention or
complaints in connection with the tension of
the full bladder may refer to cauda equina
compression (many or all descending roots
are damaged).
The cauda equina syndrome is character­
ized by:
- severe radicular pain
- flaccid paralysis, with the loss of all
sensation in a riding-breeches distribu­
tion
- total stool and urine incontinence
- impotence
Additional examinations of degenerative
spinal disorders, and differential diagnosis:
Neuroradiologic examinations furnish in­
formation on the structural state, the localiza­
tion of defects, and space-reducing processes.
CT, MRI tests, myelography, discography
and bone scan are the other choices (Fig.
24.52.).
Neurophysiologic studies are not carried
out as a routine. EMG is indicated as a differ­
ential diagnostic tool (especially to differenti­
ate peripheral neuropathies).
280 � 2 4 . D i s e a s e s of t h e s p i n e

i j{J
8
Q�.y

Fig. 24.48.
Fig. 24.50.
S 1 root signs.
�
L4 root symptoms. Motor change: m. peroneus.
Motor change: m. tibialis anterior. Reflex change: Achilles reflex.
Reflex change: patellar reflex. Sensory change: Outer edge of foot.
Sensory change: inner edge of foot.

The psychologist and/or psychiatrist has

an important role to play in the assessment of

� therapy-resistant chronic pain syndromes. Be­

fore spinal operations, the patients routinely
perform a series of tests compiled by the psy­
chologist.

Fig. 24.49. "-

LS root symptoms.
Motor change: m. extensor hallucis longus.
Reflex change: medial ankle reflex. Fig. 24.51.
Sensory change: mid part of foot. Assessment of Lasegue's and Bragard's signs.

Fig. 24.52.
Herniated disc in segment L5 51 on MRI.
-
Even without treatment in the overwhelm­
ing majority of acute back pain cases there is
an alleviation within 3 weeks.
24.6. 1.5. Conservative treatment
of a herniated disc and degenerative
spinal stenosis
When the treatment strategy is being de­
cided, the history must be carefully consid­
ered.
During the process of herniation, the water
content of the hernia decreases and the hernia
shrinks. The tom-out sequestrum loses half of
its original volume in about 6 weeks, and the
space-reducing effect fundamentally de­
creases. This is why the hernia-elicited symp­
toms regress spontaneously even within a few
days, and the pain ceases totally in 3-6 weeks
without treatment. Sensory changes have a
similar likelihood of being resolved, but mo­
tor changes (paralysis) may signal the risk of
permanent harm. When the patient exhibits
no, or only mild motor effects the basic strat­
egy is nonoperative, conservative treatment.
This conservative treatment is intended to
resolve the neuritis. The patients are given
steroids via an infusion or an epidural cannule
or paravertebrally, or nonsteroidal anti-in­
flammatory drugs are administered.
Symptomatic pain reduction may also be
used (local cooling and pain killers). Muscle
relaxants too are popular, though it is not cer­
tain, that they are needed in every case.
24. D i s e a s e s of t h e s p i n e � 28 1
In the first few days after an acute onset, in
bed rest is advised; after 3 -4 days, a gradual
vertical posture and the resulting load-bearing
is encouraged. In optimal cases, a consider­
able improvement starts around day 3 - 5 , and
from around day 1 0 physiotherapist-guided
exercises may commence. The total recovery
time depends on the original symptoms and
the extent of the neurological deficit. Within
6-8 weeks most patients (with no motor in­
volvement) regain a painless state.
In degenerative spinal conditions, the
complaints and pain may recur.
24.6. 1.6. S u rgica l treatment
of degenerative spinal d isorders
Surgical treatment must be taken into con­
sideration if the complaints do not lessen in
response to conservative therapy, or even
worsen. If the first assessment reveals exten­
sive paresis accompanied by neuritis and pain,
surgery may be decided as the first choice.
The cauda equina syndrome is an absolute in­
dication for surgery.
The aims of the operation in degenerative
spinal disorders are to solve the compression,
to eliminate the pain, to support the natural
restabilizing processes in cases displaying
various stages and forms of instability, and ul­
timately to create block vertebra with fusion.
Percutaneous minimally invasive oper­
ations. In these procedures, via a relatively
small exposure, the skin is penetrated with a
trocar. A working channel (a 2-4 mm wide,
1 50-300 mm long tube) is advanced to the
disc or to the epidural space. To remove the
content of the hernia, mechanical devices can
be employed, but a laser beam may also be
used to vaporize certain parts of the hernia
(Fig. 24.53.).
Decompression. In open surgery, the spi­
nal canal is exposed, the nerves are decom­
pressed, and the sequestrum fragments are re­
moved. This type of operation is indicated
when there is no rough instability in the given
282 � 2 4 . D i s e a s e s of t h e s p i n e

Fig. 24.53.
Percutaneous approach for removal of herniated
d isc.

segment, and the decompression itself is ex­

pected to resolve the pain and the radicular
symptoms.
a
Decompression, fusion and stabiliza­
b
tion. In cases of advanced degenerative
changes, following decompression in the un­
stable segment, it may be necessary to create
an artificial block vertebra (fusion), which
constitutes the ultimate solution (Fig. 24.54.
a, b).
The fusion procedures (creation of a block
vertebra with a bone implant) are highly ac­
cepted procedures for the surgical treatment
of degenerative spinal stenosis. The following
indications are approved by a broad interna­
tional professional consensus:
- In cases of mono segmental invol­
vement, fusion is the choice, if de­
compression leads to definitive segment
instability
- In cases of degenerative spondylo­
listhesis, especially in segment L4-5 , Fig. 24.54.
where the incidence of degenerative a and b: Transpedicu lar lumbar intervertebral fusion.
spondylolisthesis is 5 times higher than
in all the other segments.
- In cases of polysegmental decomp­ to protect the movement of the segment. In se­
ression, the possibilities for recreation of lected cases, the damaged disc is replaced by a
the spinal balance (physiologic lordosis) prosthesis in either the cervical or the lumbar
must be carefully planned. In these segment (Fig. 24.55.).
cases, implants are necessary to ensure
the stabilization of the vertebrae in the
physiologic shape of the spine, by 24.7. Spinal changes
creating the appropriate bony fusion. in osteoporosis
Disc prosthesis. In modern spinal surgery, Aging presents a special sequence of prob­
innovative techniques have been introduced lems as concerns spinal disorders .

Fig. 24.55.
a: Outline of cervica l disc implant. b and c: Biplanar X-ray after insertion of a cervical disc implant.
Morphologic characteristics of the ag­
ing spine. The morphologic appearances de­
scribed in the previous sections in which the
degenerative changes were discussed, be­
come much more marked as aging progresses,
and emerge in all spinal segments. Block ver­
tebra formation occurs mainly in the lower
segments of the cervical and lumbar spine, as
a result of the bony union of collar-shaped os­
teophytes localized at the end-plates of the
vertebral body. In the intervertebral j oints,
ankylosis develops, and the intervertebral
space is filled with a fibrotic mass.
The end-plates of the vertebral body
(mainly the subchondral layer) become scle­
rotic, and the loosening trabecular system is
observed below it, in the cancellous bone.
These changes may lead to segmental de­
formities (segmental scoliosis, degenerative
spondylolisthesis, retrolisthesis, etc.). Degen­
erative changes in many segments give rise to
the characteristic (senile) deformation. Physi­
cal examination reveals retroflexion and ret­
roversion with decreased lumbar lordosis, and
occasionally lumbar kyphosis and an increase
in the dorsal kyphosis (see Chapter 1 4 . 1 ).
2 4. D i s e a s e s of t h e s p i n e � 283
b c
These processes cause the height to decrease
and the typical (stooping) posture of the el­
derly develops.
Treatment tactics of spinal complaints
of elderly. If there is acute spinal pain (but no
neurological deficit), the patient is relieved
from weight-bearing, and pain killers and
anti-inflammatory drugs are prescribed. The
background of the acute spinal complaints of
the elderly involves a pathologic fracture in
most cases. Accordingly if the pain is not alle­
viated within a few hours, an urgent diagnos­
tic scan is required in order acquire get infor­
mation on the structural integrity of the spine.
It must not to be forgotten that in this age
group, the pathologic fracture of the vertebra,
and the morphologic diagnosis may therefore
be urgent.
Operation is contemplated only after the
failure of long-lasting conservative treatment
or when neurological signs appear. In a recent
technique, bone cement is injected into the
fractured vertebral bodies (vertebroplasty) to
decrease the kyphosis and protect the stability
of the spine.
284 � 2 4 . D i se a s e s of t h e s p i n e

24.S. Tumors
Primary extradural tumors of the spine are
very rare.

24.S. 1. Primary tumors

Benign tumors, osteoid osteoma, osteo­
blastoma and osteochondroma in the spine are
rare (Fig. 24.56.). Vascular tumors of the ver­
tebral bodies (hemangiomas), however are
relatively common. They are generally mani­
fested before the teenage years ; a late onset is
rare. They grow from the vertebral arch to the
canal or to the surrounding soft tissue.
The main complaint is the local pain at rest Fig. 24.56.
(sometimes at night) . They may cause nar­ Osteoid osteoma in the arch (U axial image)
rowing while they expand to the spinal canal,
and may produce signs of a neural deficit by
compressing the spinal cord or the spinal Of the primary malignant bone tumors of
nerves. the spine, the following sarcomas are note­
Bone changes induced by an aneurysmal worthy: osteosarcoma, chondrosarcoma, Ew­
bone cyst (classified as a tumor-like change) ing ' s sarcoma, etc. (Fig 24.57. a, b, c, d).
or disturbances of the bone metabolism (e.g. They expand aggressively and destructively,
Paget' s disease) are extremely rare in the and do not respect the surrounding areas, and
spme. their size and location do not allow their com-

a b

Fig. 24.57.
a, b: Osteosarcoma on the cervical spine (MRI)
c, d: Total segment resection, bone graft and metal plate fixation on biplanar X- ray.

plete surgical removal even at the time of their
discovery. They usually occur in children.
Following a complex oncological assessment,
the operative removal must be supplemented
with chemo- and radiotherapy.
24.8.2. Metastases
The most common form of tumor involve­
ment of the spine is undoubtedly the onset
of metastases (secondaries).
It is believed that almost all kinds of tu­
mors may metastasize to any bone, and most
often to the spine. The reason is the special
blood supply of the vertebrae : the tumor
emboli captured in the end-arteries running to
the end-plates may relatively easily embed
into the cancellous bone and start to grow
thanks to the good blood supply.
The most common localizations of the
metastases are the dorsolumbar segment and
the bodies of the two upper lumbar vertebrae.
In the first stage, the metastasis infiltrates
the medullary cavity of the spongious sub­
stance, and the destruction of the bone
Fig. 24.58.
Pathological stages of vertebral metastasis:
a: tumorous invasion of medullary cavity
b: impression of the endplates (pathologic fracture)
c: segmental instability due to the vertebral fracture with spinal stenosis and compression of the neural ele­
ments.
2 4. D i s e a s e s of t h e s p i n e � 285
trabeculae leads t o compression (pathologic
fracture) . If the vertebra is displaced and the
vertebral canal is deformed due to the tumor,
instability and a tumor-induced deformity are
threatening (Fig. 24.58.) ..
In the event of these pathologic fractures
as urgent MRI assessment is needed. The im­
ages provide information on the extent of the
tumor, the involvement of the spinal canal, the
state of the spinal cord, and the tumorous in­
volvement of the surrounding soft tissues and
spinal segments. Severe spinal cord compres­
sion demands immediate surgery: by six
hours after the onset of flaccid paralysis, the
chance of full neurological restitution has
been lost.
The operative goals are decompression of
the neural elements, full or partial removal of
the tumor, and reconstruction of the spinal
stability. It must be borne in mind, that these
interventions are intralesional from an
oncological aspect (Fig. 24.59.) .
The incidence of tumors is currently in­
creasing, while the diagnostic and treatment
procedures are undergoing constant improve­
ment. Accordingly, an ever increasing num­
ber of patients are reaching that phase of their
286 � 2 4 . D i s e a s e s of t h e s p i n e
a
b
c
Fig. 24.59.
Total segment resection on lumbar spine.
a: metastasis in L3
b, c: Following total segment resection, metal pros­
thesis bone cement, and transpedicular fixation im­
plant; biplanar X- ray.
oncological disease, where spinal metastasis
is likely. Observation, adequate musculo­
skeletal screening of these patients, and the
immediate assessment of early complaints
that point to spinal metastasis (local night
pain) afford some opportunity for the early
treatment of spinal metastases. Surgical inter­
vention is only one (radical) mode of treat­
ment. A complex oncologic approach makes
use of various chemo- and radiotherapeutic
procedures in cases involving bone meta­
stases.
24.9. I nflammations of the spine
24.9 . 1 . Spondylitis tubercu losa
(Pott's disease)
Spondylitis tuberculosa is tuberculotic inflam­
mation of the vertebrae. The i nvolved spinal
segment is painful, and its movements are
restricted. Typical signs are gibbus formation,
the destruction of two or more vertebrae on
the X- ray image with a pre- or paravertebrally
enlarged soft-tissue projection and a cold ab­
scess.
Incidence. Spondylitis tuberculosa is one
of the most common forms of musculo­
skeletal tuberculosis. Mostly the dorsal and
lumbar spine are involved, but it may appear
in the cervical segment too. In the past few
years, its incidence has shifted in the direction
of the elderly. The incidence of pulmonary in­
volvement, and also that of newly identified
extrapulmonary cases increased even in those
countries that have an advanced preventive
network.
Etiopathology (see Chapter 20). This in­
flammatory process typically occurs in the
frontal edge of the vertebral body, next to the
disc. The disc itself is already involved in the
early phase of the disease; an early X-ray sign
is the narrowed disc space.
Following the total destruction of the disc,
the process destroys the end-plates of the
neighbouring vertebrae. The two neighbour­
ing vertebrae collapse on each other because
of the partial destruction of the vertebral bod­
ies, the development of cavities follows, and
sequestra and a gibbus are produced. The for­
mation of an abscess (cold abscess), is com­
mon.
The dorsal spine is surrounded by pus in
the shape of a fusiform abscess, which some­
times breaks through to the surface between
the ribs as a sinus.
When the abscess is formed in the lower
back, in the lumbar segment, it sinks in the
caudal direction among the psoas muscle fi­
bers (psoas abscess), and it may appear as an
inguinal or femoral sinus. The abscess,
sequestrum and gibbus formation may lead to
spinal cord compression symptoms and para­
plegia. The explanation of the neural damage
is the mechanical compression by the abscess
and the sequestrum, and the toxic, vascular
damage induced by the process.

Clinical symptoms. The disease starts
with uncertain symptoms, subfebrility and fa­
tigue. The dorsal or lumbar pain increases in
response to load-bearing. The spinal move­
ments become restricted in every direction
due to the protective muscle spasm. The pa­
tient tries to substitute the painful spinal
movements, and to lean with the hands on the
thighs so as to unload the spine. The spinous
processes are tender, and gibbus may occur.
The muscle power of the lower limbs may
weaken. The cold abscess may be palpable.
Laboratory signs. The sedimentation rate
is increased, and lymphocytosis is common.
The Mantoux test may be positive. The feared
complication of spondylitis tuberculosa is
paraplegia, which demands immediate inter­
vention (see below) .
Fig. 24.60.
Spondylitis tuberculosa. The D7-8-9 i ntervertebral
spaces narrow. The end-plates are destroyed. On
the left, there is marked soft-tissue mass widening.
24. D i s e a s e s o f t h e s p i n e � 287
X-ray signs. The first X-ray sign is the
narrowed disc space. The involved vertebrae
become atrophic, and the end-plates are un­
even. As the process advances, the anterior
parts of the vertebral bodies are destroyed,
cavities and shadows of sequestra appear, and
the neighbouring vertebrae collapse on each
other. The X-ray films reveal an enlarged
soft-tissue proj ection pre- or paravertebrally.
This is due not only to edema, but also to the
development of a tuberculotic abscess con­
taining thick pus that resembles to condensed
milk (Fig. 24.60.).
The X-ray film shows the disappearance
of the psoas muscle edge in the lumbar seg­
ment, or widening of the psoas proj ection in­
dicative of the presence of the abscess. The
sclerotization of the bony structure and de­
marcation seen in the X-ray picture are signs
of the consolidation of the process, together
with the diminishing or disappearing para­
vertebral soft-tissue mass. A bony union be­
tween the neighbouring vertebrae, or block
vertebra formation means consolidation, and
the healing of the process (Fig. 24-6 1 .) .
CT and MRI examinations facilitate deter­
mination of the extent of the process, and the
spinal cord involvement. MRI also helps in
the differentiation of spinal osteomyelitis and
tuberculosis.
Since the clinical picture can vary consid­
erably, establishment of the diagnosis is not
always simple.
The abscess and vertebral focus may be
punctured with a CT-guided biopsy tool, and
specimens can be gained for bacteriology (an­
imal inoculation) and histology.
In the differential diagnosis, all those dis­
eases must be considered which cause spinal
pain and vertebral destruction (osteomyelitis,
spinal tumors, certain cases of spondylitis
ankylopoetica, spondylodiscitis, eosinophilic
granuloma, and compression fractures) (Ta­
ble 24.2.).
It is important to note, that tuberculosis
usually involves two or more vertebrae and
discs. The vertebrae collapse on each other,
abscess formation follows, and neurological
complications may occur.
288 � 2 4 . D i s e a s e s of t h e s p i n e

Treatment. With the exception of para­

plegic cases, the diagnosis is followed by
therapy with combined anti-tuberculotic med­
ication for an appropriate period to relieve the
toxic symptoms and decrease the activity of
the process. In the event of regression of the
clinical, X-ray and laboratory signs, if the im­
aging assessments (X-ray, CT and MRI) do
not prove demarcation and abscess, and if
sequestra are present, operative intervention
may be justified. The surgical aim is focus re­
section and stabilization. When there are
threatening neural signs, paraplegia may ne­
cessitate emergency intervention.
Surgery is always accompanied by anti­
tuberculotic medication. Combined anti­
tuberculotic treatment must continued for
months, since the process may flare up from
the calcified abscesses, and hidden foci.

24.9.2. Pyogenic spondylitis

(spinal osteomyelitis)
Fig. 24.61.
The clinical processes and X- ray signs, of the
Angu lation and gibbus because of the collapse of inflammations of the vertebrae caused by pu­
the vertebrae. The process has healed.
rulent bacteria are so diverse that their recog­
nition is difficu lt. The vertebral infection may
appear as a tempestuous, often life­
Pott ' s triad involves threatening spinal osteomyelitis, causing fe­
- gibbus ver, and a toxic state, or it may be a slowly
- cold abscess progressing form mimicking spondylitis
- paraplegia.

Table 24.2.
Differential d iagnosis: vertebral tuberculosis, osteomyelitis and tumor
Tuberculosis Osteomyelitis Tumor

Narrow disc space + + -

Destruction One vertebra - - +

Severa l vertebrae + + +-
Paravertebral mass Unilateral - - +-
Bilateral + +- +-
Extension of psoas projection + +- +-
Bone proliferation - +- -

Destruction of arches and ribs - - +

 24. D i s e a s e s of t h e s p i n e � 289

tuberculosa. The course of the disease is influ­

enced by the general state of the patient, the
viru lence of the agent, its possible resistance
to the medication, other drugs that may have
been taken earlier, and the efficacy of treat­
ment.

Etiopathology. The most common agents

are staphylococcus, streptococcus, pneumo­
coccus, and sometimes other strains of bacte­
ria. They approach the vertebrae via the blood
circulation. The history may include purulent
diseases, postoperative complications, fever,
disc operations or local paravertebral lido­
caine inj ections, or conditions that may often
seem unrelated to the spine, e.g. pelvic infec­
tion, or inflammations of the kidneys, genita­
lia, gall bladder, etc . A purulent focus leads to
vertebral destruction, which does not respect
the end-plates of the neighbouring vertebrae,
so that at least two vertebrae and an inter­ Fig. 24.62.
vertebral disc are destroyed. The picture may Bacterial vertebral osteomyelitis. The involved verte­
be reminiscent of vertebral tuberculosis, and brae are sclerotic, with X-ray signs of repair; cavity
formation is moderate; in spite of the collapse, the
differentiation can be problematic. vertebrae retain their heig ht (no gibbus formation).
The process may give rise to abscess and
sequestrum formation, and (less frequently
than for tuberculosis) may break into the spi­
nal canal, inducing neurological complica­
tions.

Clinical symptoms. Vertebral osteo­

myelitis is characterized by fever, malaise,
and severe pain in the involved spinal seg­
ment. The excruciating pain often results in an
inability to walk.
The laboratory results demonstrate the
changes in acute osteomyelitis: an increased
sedimentation rate, leukocytosis, a blood pic­
ture shifted to the left, and a high C-reactive
protein level.
Chronic vertebral osteomyelitis in adults
may be similar to spondylitis tuberculosa, but
the clinical picture is more dramatic : higher
fever, greater pain, the progress is faster, and
the laboratory changes are more marked.

X-ray signs. The first X-ray sign is again a Fig. 24.63.

narrowing of the disc space. This is followed MRI shows the borders of the process and the ex-
by the evidence of undefined end-plates, tent of edema.
290 � 2 4 . D i s e a s e s of t h e s p i n e

usuration, more extensive destruction, cavity

formation, and occasionally sequestrum for­
mation within the cavity. The paravertebral
soft-tissue mass may widen because of the ab­
scess or granulation tissue. The surroundings
of the vertebral destruction are more sclerotic
than in tuberculosis. A typical sign of restora­
tion is the osteophyte formation at the edges.
The vertebral bodies may collapse onto each
other, but the process does not lead to a sub­
stantial decrease in the total height of the two
vertebrae; accordingly, in contrast with tuber­
culosis, no gibbus formation is seen (Fig
24.62. and 24.63.).
The structure of the vertebral body be­
comes sclerotic in the X-ray picture, as a con­
sequence of the treatment applied, the de­
struction is demarcated, the cavity dimin­
ishes, the edge osteophytes unite, and the two Fig. 24.64.
vertebrae undergo mutual healing, forming a The block vertebra is evidence of healing.
block vertebra (Fig. 24.64.).
A supplementary examination procedure eT shows the extent of the condition,
is conventional tomography, which visualizes while MRl demonstrates that the widening of
the sequestra hidden in cavities, and also the the paravertebral soft tissue mass is more
extents of the cavities. moderate, and the structure of the vertebral
In the period of disc space narrowing, a body is more homogenous in vertebral
bone scan may contribute to the correct diag­ osteomyelitis than in tuberculosis.
nosis .

24.3.
Differential d iagnosis concerning vertebral tuberculosis, vertebral osteomyelitis, spondylodiscitis and
vertebral tumor
Tuberculosis Osteomyelitis Spondylodiscitis Tumor

History Tubercu losis in the Banal infection Banal infection or Primary tumor
past none
Fever Subfebrility Fever Fever or subfebrility Not typical
or none
We Moderate High Moderate or high, or Occasionally high
normal
(-reactive pro- Normal High Moderate or high Not typical
tein
Leukocytosis Lymphocytosis High, left shift High or low High or normal
Radiological Extensive Moderate Not typical Involves one
destruction vertebra
Gibbus Typical Not typical Not typical Not typical
Structu re Atrophic Sclerotic Sclerotic Lytic or plastic

Diagnosis. When any vertebral process is
detected, the most appropriate and modem
bacteriologic and histological tests (im­
age-guided aspiration and sophisticated bi­
opsy techniques) must be employed to estab­
lish the diagnosis (Table 24.3.).
Treatment. In vertebral osteomyelitis, in
contrast with tuberculosis, conservative ther­
apy may result in healing of the process.
There are a few exceptions : sepsis, abscesses,
sequestra and threatening neurological com­
plications .
Immobilization in a bed or a plaster bed,
depending on the age, is the obligatory
method with which to treat inflammations.
Broad-spectrum antibiotics which ensure a
therapeutic concentration in the bone are
given first, followed by an antibiotic selected
on the basis of sensitivity tests. Indications of
effective therapy may appear within a few
days : the fever and the pain subside, and the
laboratory parameters improve. The improve­
ment in the sedimentation rate is slower than
that in the C-reactive protein.
Regression of the X-ray signs is much
faster, than in tuberculosis, but it occurs only
after the clinical improvement.
When the clinical and radiological regres­
sion of vertebral osteomyelitis have taken
place, the patient is mobilized in a few weeks.
A plaster or plastic corset is fitted for external
support. The appearance of a block vertebra
signifies healing.
Spinal processes that result in abscesses,
sequestra or threatening neurological compli­
cations demand not only medical but also sur­
gical treatment. The abscess is exposed and
the focus is cleaned and drained, similarly as
described in connection with the treatment of
spondylitis tuberculosa.
24.9.3. Spondylodiscitis
Spondylodiscitis is a benign, inflammatory
condition of the discs or vertebral end-plates.
24. D i s e a s e s of t h e s p i n e � 29 1
Apart from the pain syndrome, the nar­
rowing of the intervertebral space is typical,
without maj or destruction. Moderate fever
and an elevated sedimentation rate may be ob­
served.
Most experts consider it to be outcome of a
spinal infection. It may be a torpid osteo­
myelitis, where the agent invaded the disc
secondarily from the end-plates without the
typical picture of acute osteomyelitis.
It occurs in children and young adults. The
intervertebral disc is a bradytrophic tissue.
The poorly nourished disc is unprotected
against infection and is destroyed in a long,
torpid process, and the space narrows. The
richly vascularized vertebral body resists a
not highly virulent agent, and therefore is not
(or only slightly) involved in the process.
Clinical symptoms. The processes de­
scribed above often persist undetected be­
cause of the insignificant local and general
symptoms. However, pain may be experi­
enced in the spinal segment involved.
Bending forward may be hindered because of
the paralumbar muscle spasm, and the child
therefore avoids doing it. Local tenderness
may be detected.
X-ray signs. Because of the slow progress
of the disease, the X-ray picture may initially
be negative. Later, the narrowed inter­
vertebral space becomes visible with
usuration of the end-plates, and intrusion of
the disc at the lumbar spine into the vertebral
body, even in the form of Schmorl 's hernia
(Fig. 24.65., and 24.66.) .
Lymphohematogenic infection may create
space narrowing over a long spinal segment.
A bone scan may facilitate the diagnosis.
CT and MRI tests provide more informa­
tion earlier than the analogous X-ray image.
Treatment. Chronic antibiotic and rest
(bed rest, and a corset) cure the process within
a few weeks. With the exception of the narrow
disc space, which persists, the symptoms re­
solve.
292 � 2 4 . D i s e a s e s of t h e s p i n e
Fig. 24.65.
A 16-year-old girl with spondylodiscitis mimicking
Schmorl's hernia, fever, and a high erythrocyte sedi­
mentation rate.
Fig. 24.66.
MRI reveals that the d isc protrudes into the
cancellous su bstance of the vertebrae.
It has been reported that mild cases of
discitis in childhood may heal spontaneously,
regardless of the treatment. Since this condi­
tion is presumed to be a mild, torpid infection,
antibiotics are advised.
24. 10. Chest deformities
The anatomy, biomechanics and vital
function of the chest make it the most compli­
cated unit in the organism.
24. 10.1. Pectus excavatum (funnel
chest)
This is a congenital chondrosternal impres­
sion, where the mass of the sternal bone and
the attached rib cartilages bend backwards,
creating a funnel-shaped impression, the
deepest point being the xyphoid process
(Fig. 24.67.).
The extent of the impression of the chest
wall can differ. In a severe case, the lower end
of the sternum, the xyphoid process, almost
reaches the vertebral bodies. In girls, the de­
velopment of the breasts may accentuate the
virtual extent of the midline impression.
Etiopathology. This has not been fully
identified. It often occurs in association with
other developmental disorders : Marfan ' s syn­
drome, flat back syndrome, etc. The structural
changes in the chest may disturb the thoracic
organs. The lower section of the sternum and
the ribs move backward on inspiration, and
protrude on expiration: paradox breathing
takes place, with an impaired vital capacity.
The decrease in the sternal - vertebral distance
elicits dislocation of the mediastinum. The
apex beat of the heart may be palpable in the
axillary line for the displacement and rotation
of the heart. Thus, the QRS complex may
change, with R vector deviation to the right,
and T wave inversion.

Fig. 24.67.
Orcu mscribed, symmetrical impression on the fron­
tal chest wall. Pectus excavatum.
Clinical symptoms. The children are usu­
ally asthenic and lean. Their trunk is slightly
tilted forward, the spine is mildly kyphotic,
and the shoulders are turned forward. In re­
sponse to a physical load, fatigue, dyspnea
and tachypnea may arise. The performance of
these children is inferior to that of their con­
temporaries. Their respiration is often para­
dox. They often suffer from influenza, bron­
chopneumonia or asthma.
In adult cases, physical loading may lead
to suffocation and compressive thoracic pain.
Neither the children, nor the adults like to get
undressed in front of others, and they avoid
public places such as swimming pool because
of their deformity.
X-ray signs. The traditional postero­
anterior chest X-ray reveals the displacement
of the heart and mediastinum, and sometimes
the accompanying spinal deformity. The lat­
eral view clearly shows the distance between
the posterior edge of the sternum and the ven­
tral contour of the vertebrae (Fig. 24.68.).
24. D i s e a s e s of the s p i n e � 293
Fig. 24.68.
Lateral chest X-ray: At the deepest point of the im­
pression, the posterior su rface of the sternum ap­
proaches the ventral surface of the spine.
The chest CT appropriately visualizes the
extent of the deformity, and the distance be­
tween the deepest point of the sternum and the
ventral contour of the vertebrae.
The severity of the deformity is indicated
in terms of Keszler 's index (Fig. 24.69.), as
follows :
sternum - vertebral distance
------ · 1 00
posteroanterior chest frontal diameter
With this index, the extent of the defor­
mity is graded as mild (30-3 5 %), moderate
(20-30%) or severe « 20%).
Treatment. As conservative treatment,
breathing exercises and abdominal and dorsal
muscle training are recommended from early
childhood to decrease the deformity, to pre­
vent paradox respiration and to practice cor­
rect thoracic breathing. Swimming and athlet­
ics may improve the vital chest functions and
capacity.
294 � 2 4 . D i s e a s e s of t h e s p i n e
I·
b
_
_ _ - -
_
:::: _c ---t--:C----_-_-_-,'"
>------
- -
I ...
c:+-=J
- - -
Fig. 24.69.
Keszler index = a/b x 100
If the complaints or the deformity worsen,
cardiopulmonary changes are observed, or the
deformity causes psychological complica­
tions, surgery indication may be contem­
plated. About 40% of the patients are operated
on between the ages of 4 and 1 0 years, and
50% under the age of 20.
24. 10.2. Pectus carinatum
(pigeon chest)
I n contrast with funnel chest, pigeon chest is a
protrusion deformity. Less attention is paid to
this deformity, but it may a lso occur in a d is­
figuring form that causes a major disturbance
in the function of the thoracic organs. It may
appear in combination form with funnel
chest, with the predominance of either.
Etiopathology. The etiology is unclear.
The deformity occurs in early childhood and
becomes more sever at puberty (around the
age of 1 0) . In the view of Lester, it may origi­
nate from rickets, but, similarly as for pectus
excavatum, the congenital etiology prevails.
Anomalies of the diaphragm may play a role,
with the difference that the centrum ten-
·1
--
dineum is widened and the tension of the lat­
eral fibers creates the deformity. The antero­
lateral part of the diaphragm is hypertrophied;
this pulls in the rib arch, and paradox breath­
ing results.
Clinical symptoms. These are similar to
those described for funnel chest.
The breath function test proves a reduction
in the vital capacity, especially if the chest ex­
pansion is decreased by the expressed Harri­
son groove and the paradox breathing.
The condition may be accompanied by a
kyphotic back or kyphoscoliosis.
The cosmetic and psychological problems
are similar to those in cases of funnel chest.
X-ray signs. In pectus carinatum ob­
liquum, the protrusion is obligue, whereas
pectus carinatum arcuatum it is arched. It does
not displace the heart. A chronic pulmonary
circulation failure is detected as a late compli­
cation of severe deformities.
Treatment. The surgical therapy and
postoperative care of protrusion deformity
cases are similar to those for pectus ex­
cavatum.
 Jeno Kiss
25. Disorders of the neck and the shoulder
girdle
25. 1. Functional anatomy and
biomechanics of the shoulder
girdle
The main function of the shoulder and the
elbow, via the upper arm and the forearm is to
adjust the main human working tool, the hand
into the wanted position. Any operational dis­
turbance of these two large joints of our upper
limb may impair the working capabilities and
the regular daily activities. The degree of the
function depends on whether the dominant or
not dominant side is involved. A young la­
borer or a competing athlete has different ex­
pectations relative to the elderly, who mainly
use their upper limbs for self-maintenance.
g ----��----
Fig. 25. 1.
Anterior aspect of the shoulder region: a: n. suprascapularis, b: n. axillaris, c: plexus brachia lis, d: hu merus,
e: processus coracoideus, f: acromion, g: clavicula, h: lig. coraco-acromiale, i: m. subscapularis, j: m. supra­
spinatus, k: biceps tendon.
The movements of the shoulder girdle are
combinations of the glenohumeral, acromio­
clavicular and sterno-clavicular movements .
An additional function is the motion of the
shoulder blade on the trunk, the three most
important components of which are the eleva­
tion (lifting the arm in the plane of the shoul­
der blade), external rotation and the combina­
tion of extension and internal rotation which
result in the ability to reach behind the back.
This motion is ensured by the function of the
thoraco-humeral, thoraco-scapular and
scapulo-humeral muscles.
The shoulder itself, the glenohumeral j oint
has the largest range of motion of any joint of
the human body, and at the same time it is the
most unstable joint.
��----- b
296 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s ho u l d e r g i rd l e
e d
a
7 �
k
g " /
.'
>
Fig. 25.2.
Posterior aspect of the shoulder region: a: n. supraspinatus, b: n. axiliaris, c: humerus, d: spina scapulae, e: m.
supraspinatus, g: m. teres minor, h: m. teres major, i: m. deltoideus, k: m. infraspinatus.
Special significance is attributed from an
orthopedic aspect to the rotator cuff (Fig.
25. 1 ., 25.2.). This comprises the tendons of
four muscles that originate in the frontal and
dorsal surfaces of the shoulder blade : the
subscapular, supraspinate, infraspinate and
teres minor muscles. Besides assisting the
various rotational motion of the humerus, the
most important task of the rotator cuff is to en­
sure the central position of the humeral head
against the j oint cavity, in the glenoid fossa, in
any position of the arm.
25.2. Examination of the shoulder
girdle
The most common complaints are pain,
weakness, motion disturbance and instability.
The onset of the complaints, and the circum­
stances of the possible injury must be ex­
plored. The details of the profession, possible
sporting activity and the dominant side are
also important. During the inspection atten­
tion should be paid to an abnormal posture,
deformities, muscular atrophy, possible skin
_ ': ,
·,
_ - ---..I-
\ SH=l-tS
.
v • I b
-+--+- c
h
redness, swelling. The assessment of the
range of motion starts with the range of active
motion, and in case of a deficit, it is compared
with the range of passive motion, since impor­
tant conclusions can be drawn from the differ­
ences. The stability of the glenohumeral joint
is evaluated via the various stability signs
(sulcus sign, apprehension tests, drawer sign).
The individual muscle and muscle group
functions are checked. The muscles of the ro­
tator cuff are to be assessed extremely care­
fully. An indispensable part of the physical
assessment of the shoulder girdle and the up­
per limb is the evaluation of the motion of the
cervical spine and the neurological state of the
upper limb. This may be supplemented if
needed by connectivity testing of the upper
limb nerves (ENG) and the muscles (EMG).
The radiological evaluation requires bi­
directional films, one a-po and one lateral
view. Depending on the actual disease a num­
ber of directed examinations are to be used. A
cheap, swift and reliable procedure is
ultrasonography test to evaluate the soft tis­
sues and especially the rotator cuff. The next
step can be in assessment a native or con-
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
trasted e T and MR assessment of the changes
in the soft tissues, and bones, j oints. In certain
cases, arthroscopic examination of the
glenohumeral, subacromial or subscapular
area may be required, these procedures are
mainly used as the introductory phase of an
endoscopic or open therapeutic method.
25.3. The congenital and acquired
disorders of the of the shoulder
girdle
• Torticollis, muscular congenital (wry­
neck)
The asymmetric posture of the head is caused
by the shrinkage of the sternocleidomastoid
muscle. The head is tilted lateral ly and rotated
in different directions, the face is asymmetri­
cal, the sternocleidomastoid muscle is tensely
prominent. From the age of 1-2 weeks a pain­
ful swelling is observed in the muscle. This oc­
curs in both genders, and is often associated
with other developmental anomalies. The
symptoms are usually observed as early as 1-2
weeks. This is the 3rd most common
musculoskeletal disorder after hip dislocation
and clubfoot.
Etiopathology. This is a congenital disor­
der. According to certain theories injury, or
tearing occurs in the substance of the ster­
nocleidomastoid muscle during birth, leading
to the scarring of the muscle.
Others postulate the role of intrauterine in­
congruence, the neck is forced into an asym­
metrical position, this causes the shrinkage of
the muscle. A further view consider the intra­
uterine ischemia as a causative factor. Besides
the exogenous factors the role of endogenous,
hereditary factors have also been supposed. In
certain families, the wry-neck occurs with in­
creased frequency, often together with other
hereditary disorders, such as hip dislocation,
clubfoot.
Clinical symptoms. In the substance of
the sternocleidomastoid muscle a hazel­
nut-plum sized tender lump is palpable at
� 297
one-two weeks of age. Later the lump disap­
pears from the muscle. It is palpable only ex­
ceptionally after the first or second year, but
the shrunken muscle may still be detected.
The shortened muscle is as tense as a tight
string and protrudes under the skin. The head
tilts to the involved side, while the face turns
in the opposite direction. The shrinkage may
involve one or both muscle parts. The shrink­
age of the sternal part causes the rotation,
while the clavicular part tilts the head (Fig.
25.3.). If the torticollis persists for a long pe­
riod, the skull will become asymmetrical . The
involved part of the face will be smaller,
scoliosis may affect the cervical spine.
Differential diagnosis
• Congenital scoliosis of the cervical spine.
The X- ray shows the possible vertebral
anomalies.
• Ocular torticollis. I n cases of certain vision
anomalies the child may hold the head
asymmetrically to compensate.
• Torticollis caused by i nflammation. Various
inflammatory processes may occur in the
cervical spine. Painful motion restriction
may occur in the neck related to about of
common flu, which resolves within a few
days.
• Traumatic torticollis. Pain commonly
develops in children after sporting activity,
during fighting, in adults following sudden
jerks (e.g. looking backwards), causing an
oblique posture of the neck.
• Rheumatoid torticollis. This occurs together
with other rheumatoid symptoms.
• Spastic torticollis. This is a the possible
consequence of neural illnesses, such as
encephalitis, chorea.
• Scar related torticollis. This is caused by
scars after burns, or various i nflammations.
• Torticollis may be caused by the individual's
occupation.
• Torticollis may a lso be due to psychological
reasons.
Treatment. If the disorder is detected in
infanthood, conservative treatment may be
commenced. The shortened muscle is to be
stretched by means of passive exercises, the
298 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s ho u l d e r g i rd l e
Fig. 25.3.
Right sided torticollis. The right m. sternocleido­
mastoideus is tense, the head is tilted to the right
and rotated to the left.
parents must be educated to do these. The in­
fant should be positioned in such a way as to
have to make an active correction to see the
environment: e.g. in the case of right sided
torticollis the light and sound stimuli must be
located on the right side and above, forcing
the baby to look to the right and upward. The
baby is to be fed in such a way that the head
must move in the direction of correction. De­
pending on the severity of the disorder opera­
tion is advised at the age of 1 -2 years . The
tense part of the muscle is incised-in some
cases the entire muscle is detached from the
sternum and the clavicle. Some experts pro­
pose the recession of the upper attachment of
the muscle from the mastoid process. Surgery
performed at an proper time usually results in
complete correction. If the operation is de­
layed, the facial asymmetry may persist.
Rehabilitation. Postoperatively soft col­
lar support in young individuals is used, or
plaster of Paris fixation in older patients or in
cases of severe deformity. The head is posi­
tioned into overcorrected state. After 2-4
weeks of rest, the neck exercises commence.
Fig. 25.4.
Sprenge/'s deformity. The left shoulder blade is
higher.
� Klippel-Feil-syndrome (short neck
syndrome)
The neck of the patient is very short, and the
range of motion is restricted. The deformed
vertebrae may resu lt in the mechanical irrita­
tion of the cervical nerve roots and occasion­
ally of the spinal cord, neural signs may there­
fore appear.
Etiopathology. This characteristic defor­
mity is caused by a multiple developmental
anomaly. There is no unified etiology, so it is
recommended to consider this anomaly as a
syndrome. The basic disorder is a disturbance
of segmentation, or the unification of the bi­
lateral half-segments of the vertebrae.
Symptoms. In mild cases the X-ray done
for some other reasons uncovers the disorder
of the cervical spine, which is localized to a
few vertebrae and symptomless. In severe
forms of the condition, the short neck is spec­
tacular, which may be due to the extreme
shortening of the neck, or it may also be vir­
tual due to the higher location of the scapulae.
 2 5 . D i s o r d e rs of t h e n e c k a n d t h e s h o u l d e r g i rd l e
The neck motion is restricted, torticollis may
develop. The X-ray shows the vertebral
changes listed above.
Treatment. Only the complaints and neu­
rological symptoms due to the degenerative
changes in adults are to be treated.
� Elevated scapula (Sprengel's
deformity, congenital high position of
the shoulder blade)
This is a shoulder blade anomaly occu rs in
both genders. The shoulder girdle with the
shoulder blade is higher on one side or rarely
on both sides, while the shoulder blade is de­
formed, hypoplastic and often rotated (Fig.
25.4.)
Etiopathology. During the development,
the shoulder girdle does not descend in the
usual way from the height of the cervical seg­
ments. The periscapular muscles are short­
ened due to the position of the bone, their path
changes, in certain muscles fibrosis is ob­
served. From the upper apex of the shoulder
blade a cartilaginous-bony bridge (omo­
vertebral bridge) is connected to the lower
cervical spine in about one third or half of the
cases.
Symptoms. The shoulder blade and the
shoulder are located higher, the upper angulus
Fig. 25.5.
a: X- ray: The right shoulder blade is higher. I n the cervicodorsal junction at many levels spina bifida is present.
b: The shoulder blade limits the elevation of the arm.
� 299
of the scapula protrudes in the line of the neck
and shoulder, the tip of the shoulder is dis­
placed anteriorly. The elevation of the arm is
hindered because of the restricted motion of
the scapula. The X-ray shows the shoulder
blade positioned higher than the opposite one,
in some cases the bony bridge connected from
the angulus superior to the lower cervical
spine is also observed (Fig. 25.5. a-b).
Treatment. This condition typically does
not disturb the function of the limb, is not
painful, therefore its correction is usually jus­
tified from a cosmetic point of view, primarily
in unilateral cases. Quite a few procedures are
recommended, one of the most simple and rel­
atively common interventions is incision of
the omovertebral bridge. If the angulus supe­
rior protrudes considerably, a good cosmetic
result may be achieved by excision of this
part.
� Scapula alata (flying shoulder blade)
This scapula disorder may take place for vari­
ous reasons. In mild forms during elevation, in
severe forms even at rest, the shoulder blade
lifts off the trunk. Since the function of the
upper limb and the u nlimited motion range
of the shoulder girdle require stability and
harmonic movement, the pathologic state of
the shoulder blade may cause severe func­
tional loss.
300 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
Etiopathology. The main motors and sta­
bilizers of the shoulder blade may weaken one
by one (serratus anterior or trapezius muscle
palsy) or simultaneously. The cause of
serratus anterior muscle palsy is a lesion of the
thoracic longus nerve, which may appear after
a viral infection, lifting heavy obj ects, trac­
tion, direct contusion or iatrogenic injury. The
accessory nerve may be injured in a similar
way leading to palsy of the trapezius nerve.
Symptoms. The shoulder blade lifts off
the trunk on elevation of the arm. In severe
forms, this sign is detected even at rest. Be­
cause of the disorder of the shoulder blade, the
elevation of the arm is weaker or very limited
(Fig. 25.6. a-b). In mild cases, only fatigue,
shoulder girdle pain are detected. In case of
facioscapulohumeral dystrophy (FSHD) com­
plex shoulder girdle weakness is observed.
Treatment. In cases of isolated serratus
anterior palsy, conservative treatment is ap­
plied first, including shoulder girdle exer­
cises, selective current stimuli and vitamin D .
If conservative treatment is ineffective after 6
months, surgical exploration and neurolysis is
to be considered.
In cases of total palsy of the scapula stabi­
lizers the fixation of the shoulder blade to the
trunk, the scapulo-thoracal fusion provides
a b
Fig. 25.6.
A young female with facioscapulohumeral dystrophy (FSHD): bilateral scapula alata (a), limiting the elevation
of the arm (b).
the FSHD patients with dramatic improve­
ment in upper limb function and quality of
life.
� Neuromuscular compression syndrome
of the upper limb ( " thoracic outlet " ­
syndrome)
This is a distinctive, complex syndrome caus­
ing complex sensitivity, innervation and circu­
lation disturbances in the upper limb. It is in­
creasingly referred to as the "thoracic outlet
syndrome" (TOS). It is most common in mid­
d le-aged women.
The typical cause may be a cervical rib,
when the brachial plexus and the subclavian
vein and artery are all compressed at their exit
from the chest, before they reach the upper
limb (Fig. 25.7. 25.8.).
Symptoms. These are due to sensory, mo­
tor and circulation changes.
• Sensory changes: Painfu l paresthesiae, often
marked pain, burning sensation appears in
the ulnar part of the hand and fingers.
Hypoesthetic zones may be present in
areas supplied by the ulnar nerve.
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i rd l e
Fig. 25.7.
Pathomechanism of the thoracic outlet" syndrome.
"
a: a. vertebral is, b: m. scalenus medius, c: m. scalenus
anterior, d: a. su bclavia, e: v. subclavia, f: m. pectoralis
minor.
Motor changes: Paresis, muscle atrophy may
�
take place mainly in the small muscles of
the hand. The g rasping force may be
diminished.
� Vasomotor changes: Cyanosis may appear
in the limb, atrophic changes may appear
on the fingers. The radial pulse may
weaken or disappear if the limb is
abducted and externally rotated.
Treatment. In mild cases conservative
treatment is successful. It is important to per­
fonn exercises to strengthen the shoulder
musculature and improve the posture. In stub­
born cases, an operation, removal of the 1 st
rib or cervical rib may be needed to improve
the compression signs.
� 30 1
Fig. 25.S.
Cervical rib (white arrow) and extended processus
costotransversarius (black arrow).
� Cervicobrachial syndrome
Detailed description of this clinical picture
is to be found in the chapter of spinal disorders
(Chapter 24). The symptoms are often similar
those to of other upper limb illnesses, and
therefore an assessment of the cervical spine
is essential, occasionally with X-ray examina­
tion in cases of any upper limb symptoms, and
a basic neurological assessment of the upper
limbs.
� Obstetric paralysis (paralysis of the
lower limbs after birth)
During birth, the brachial plexus or roots of
the p lexus are injured leading to various ex­
tent of paralysis of the upper limb muscle.
302 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i rd l e

The involved upper limb o f the infant lies 25.4. Disorders of the shoulder
motionless, rotated internally beside the
trunk. Later, the clinical picture depends on In the common meaning shoulder joint is
the extent of the paralysis. Three forms are simply taken as the glenohumeral joint. How­
distinguished, regarding the anatomy of the ever, in a wider sense, the shoulder joint refers
plexus : to a joint performing all of the movements be­
- Erb-Duchenne 's form involves the tween the trunk and the humerus.
upper arm
- Klumpke 's form involves the forearm � Subacromial impingement
- the third form involves the entire upper
limb.
This syndrome is caused by the abnormal
contact with and rubbing of the acromion by
Symptoms. The infant is lying with the the g reater tubercle and the attached supra­
floppy, motionless upper limb, internally ro­ spinate tendon. Typical symptoms are the
tated beside the trunk. Later the extent of the painful motion arc during lifting of the arm
paralysis and of the possible anesthetic, and the pain radiating to the upper arm, and
hypoesthetic areas are dependent on the type increases at night. This syndrome is character­
of the paralysis. These changes can usually be istic of the individuals between 40-50 years.
established only in older babies. In larger chil­
dren and adults a growth disturbance of the Etiopathology. These symptoms may be
upper limb is observed, while the humerus or caused by a number offactors. The most com-
in more severe forms the entire limb is
shorter.
Treatment. In the first week after birth the
limb is put to rest. The limb is usually posi­
tioned with the shoulder abducted to prevent
contractures. The simplest way is to tie the
shirt sleeve with a ribbon to the swad­
dling-clothes (Fig. 25.9.) . In the second week,
passive exercises and electrical stimulation
are commenced. After the 2nd year an im­
provement is not expected from further con­
servative treatment, the function of the limb
may often be improved surgically.
)

Fig. 25.9.
\' - \ � I Fig. 25.10.
The paralytic upper limb is fixed in an abducted po­ Painful arc, typical of the middle range of the eleva­
sition to the swaddling-clothes or to the bed. tion (a: painless, b: painfu l motion)
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i rd l e
mon are the anatomical changes o f the
acromion as the age advances. Inflammation
of the subacromial bursa for any reason, but
usually after repeated trauma, tendinitis of the
rotator cuff, may lead to impingement by nar­
rowing the subacromial space. Paralysis or
only weakness of the rotator cuff, contracture
due to shrinkage of the tendons or of the cap­
sule of the glenohumeral joint may cause im­
pingement by displacing the humeral head
cranially.
Symptoms. A typical complaint is the
pain in the middle range of arm lifting, the so
called painful arc (Fig. 25.1 0.). The night pain
is also typical, the patient wakes many times
and is unable to lie on the involved side. Apart
from the painful arc examination reveals crep­
itation during elevation and rotation, and
weakness of the muscles of the rotator cuff
due to the pain.
X-ray shows the possible changes in
shape, osteophytes of the acromion, and scle­
rosis of the greater tubercle (Fig. 25. 1 1.). Ul­
trasound or MRI shows thickening of the sub­
acromial bursa, fluid collection, edematous
thickening or degenerative thinning of the
supraspinatus tendon.
Treatment. Conservative treatment is
first applied, depending on the causative fac­
tors . The possibly decreased range of motion
Fig. 25.11.
Calcified attachment of the coraco-acromial liga­
ment leading to impingement.
� 303
must be restored with active exercises of the
rotator muscles. The pain and inflammation
are alleviated with NSAID, physiotherapy
and a few (at most 3) local steroid inj ections.
The working environment, sport and occupa­
tional activity are to be adjusted. If the conser­
vative method fails, an operation may be con­
sidered. Subacromial decompression may be
done with an open exposure or arthro­
scopically. The operation is followed by a
number of month rehabilitation period, with
the aim of restoring the motion rhythm, range
and the muscle power.
� Tearing of the rotator cuff
Any tendon, but most often the supraspinate,
may be torn partially or in total thickness, typ­
ically near to the attachment. The clinical pic­
ture is characterized by a painful arc during
lifting of the arm, the pain, increasing at night
and radiating to the upper a rm, and the func­
tion disturbance, depending on the muscle in­
volved.
This disorder, most common around the
age of 40, is due to the degenerative changes
of the tendons. Its frequency increases with
the age. It may occur rarely at a younger age
(throwing athletes, laborers) .
Etiopathoiogy. A number of factors may
play a role. The tendons of the rotator cuff,
and specially the supraspinate have a
hypovascular zone in the vicinity of the at­
tachment. The impaired circulation is accom­
panied by collagen degeneration due to aging,
repeated microtrauma relating to the occupa­
tion or sporting, occasionally a significant
trauma. The tear initially is rarely total, but
usually a partial thickness tear (Fig. 25. 1 2 .
a-b), and i t later becomes complete gradually
or after a major injury. Tears are also de­
scribed regarding to their extent as well.
When the tear is larger and persists longer, the
possibly intact residue of the rotator cuff loses
its centralizing effect on the humeral head.
The humeral head is displaced cranially and
gradual secondary degenerative changes take
place.
304 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s ho u l d e r g i rd l e
a b
Fig. 25.12.
Partial tear of the supraspinate tendon in the prox­
imity of the joint (a) and the bu rsa (b)
Symptoms. This condition is also charac­
terized by the pain presenting in the middle
range when the arm is elevated, the so called
painful arc (Fig. 25.1 0.). Night pain is also
common. The patient may experience pain
and cracks, rubbing in certain movements.
Active elevation of the arm is possible even in
cases of massive tears, but examining the
muscle functions one by one, the weakening
is detected. In case the lesion has been present
for a long time, the supraspinal or infraspinal
fossa will be excavated, signaling the inactiv­
ity atrophy and degeneration of the muscles.
X-ray shows the possible changes in
shape, the sclerosis of the acromion, and the
sclerosis of the greater tubercle. In more se­
vere cases, thinning of the subacromial space
and secondary osteoarthritis of the gleno­
humeral j oint are observed. Ultrasound or
MRI shows thickening of subacromial bursa,
fluid collection, and degenerative thinning in
cases of partial tear, and the loss of the
supraspinate tendon in cases of total tear.
Treatment. Depending on the age, occu­
pation, daily activities of the patient, and also
on the extent of the tear, conservative therapy
may be chosen. Active exercises are impor­
tant to strengthen the remaining components
of the rotator cuff and other muscles of the
shoulder girdle, and to improve the active mo­
tion. Pain and inflammation can be reduced
with NSAID, physiotherapy or a few, (at most
3) local steroid injections. Operation is con­
sidered in older patients after failed conserva­
tive therapy, at an active age or even as pri­
mary treatment. In partial tears, open or ar­
throscopic acromioplasty and removal of the
tom periphery are indicated. In cases of a total
tear, rotator reconstruction, reinsertion of the
tendon(s) to the bony base is recommended
(Fig. 25. 13. a-b) .
� Adhesive capsulitis (frozen shoulder)
This is a typical syndrome caused by fibrotic
tissue changes in the soft tissues surrounding
the shoulder. The range of motion is limited,
painful, but no significant X-ray changes are
detected.
It is a common affliction mainly in middle
aged adults, women are involved one and a
half times more often. It is more common in
association with diabetes mellitus and other
metabolic diseases . The condition may be uni­
or bilateral with different severity and pro­
gression.
Etiopathology : a number of factors may
play role.
Two basic forms are distinguished.
- Primary adhesive capsulitis: the
etiology is unknown.
- Secondary adhesive capsulitis: the
clinical symptoms are similar and the
cause is known : injury of the shoulder,
operation, intra- or periarticular
inj ection, infection, etc.
Symptoms. Regardless of the etiology
pain radiating to the upper arm is typical. The
pain is present at rest, often it is worse at rest.
The onset of pain is soon followed by a grad­
ual restriction of motion. As time goes by, the
pain may decrease or disappear, but the re­
stricted motion persists for a long time.
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
a
Fig. 25.13.
Reinsertion of torn supraspinate tendon with
transosseal sutures (a: a-p view, b: upper view).
On examination typically severe restric­
tion of elevation and rotation is found. The ab­
solute diagnostic criterion is that besides the
� 305
limitation of both active and passive shoulder
motions the native X-ray does not prove any
maj or changes.
Treatment. Typically in a large propor­
tion of the cases this disorder heals without
any particular therapy. This healing may last
for 1 -3 years, and while the pain ceases and
the motion improves, some restriction in the
range persists. The aim of the conservative
therapy is to relieve the pain. At the same
time, gradual restoration of the range of mo­
tion should commence with exercises per­
formed a few times every day. In the over­
whelming maj ority of the cases conservative
treatment results in a satisfactory improve­
ment, an additional procedure could be the
manipulation of the joint under anesthesia.
Some prefer operative treatment.
� Glenohumeral arthrosis (osteoarthritis
of the shoulder)
This condition causes a painful limitation of
motion of the g lenohumeral joint it may be
primary or secondary (following inflamma­
tion, degenerative, traumatic changes, infec­
tion, etc.). The primary form involves mainly
elderly a bove the age of 60-65. The secondary
forms may take place at any age, depending
on the etiology.
Etiopathology. In primary arthrosis, the
changes are similar to those for the other large
joints (Fig. 25.14. a) . The rotator cuff is usu­
ally intact.
In secondary osteoarthritis the outcome of
the changes depends on the etiologic factor
(Fig. 25.14. b, c, d) .
Symptoms. Common symptoms of the
primary and secondary osteoarthritis are the
pain and the limited range of motion, which
usually develops gradually. Because of the
chronic instability or the rotator cuff tear,
muscle atrophy takes place around the shoul­
der. In contrast with adhesive capsulitis radio­
logical changes are always detected in cases
of arthrosis.
Treatment. Since the load-bearing de­
mand on the upper limb is less than that on the
306 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i rd l e

d
a

Fig. 25.14.
Primary arthrosis of the shoulder (a), post-trau matic arthrosis (b), avascu lar humerus head -necrosis induced
arthrosis (c), massive rotator cuff tear induced arthrosis (d).

lower limbs, conservative treatment can be
applied for a long period. If an operation is
necessary, it may be a j oint-preserving or a
j oint-replacing procedure (arthroplasty).
Joint-preserving methods include arthroscop­
ic lavage of the shoulder, endoscopic removal
of cartilage debris, osteophytes, tom pieces of
labrum and tendons, and in rheumatoid pa­
tients arthroscopic or open synovectomy. In
severe cases, implantation of a shoulder pros­
thesis will improve the quality of life. De-
pending on the type and severity of the illness
merely the head of the humerus (hemi­
arthroplasty) (Fig. 25.15. a), or both the head
of the humerus and the glenoid surface can be
replaced (Fig. 25.15. b). When both the
glenohumeral joint and the deltoid muscle are
destroyed, glenohumeral arthrodesis (Fig.
25.1 6.) could be the only solution to ensure
pain relief and it provide an acceptable func­
tion by rotation of the scapula over the trunk.
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
Fig. 25.15.
Shoulder hemiarthroplasty (a) and total arthroplasty,
(b) X-ray picture.
25.5. Other painfu l shoulder
conditions briefly
� Tendinitis of biceps brachii muscle
Pain and local tenderness radiate from the
area of the bicipital sulcus to the upper arm.
This disease usually observed among young
� 307
Fig. 25.16.
Glenohu meral arthrodesis, osteosynthesis with
plates.
athletes and heavy laborers, is due to the phys­
ical overload and the special intra-articular
path of the biceps caput longum tendon. If
conservative treatment fails, arthroscopy is
advised, this may show fragmentation or fi­
brillation, which may be smoothened arthro­
scopically. In cases of a severely degenerated
tendon proximal detachment and tenodesis in
the sulcus or more distally can be considered.
� Calcifying tendinitis
This is usually observed in women over
40, who experience very heavy shoulder pain
without any antecedents. The diagnostic crite­
rion is a radiodense structure over the greater
tubercle, which is less dense than the mature
bone, but well circumscribed, corresponding
to the calcium crystal deposits (hydroxy­
apatite) in the substance of the tendon, which
proves an inflammatory process in the area.
The treatment is basically symptomatic, since
the condition heals spontaneously. Pain kill­
ers, NSAID are prescribed. A dramatic im-
308 � 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s ho u l d e r g i rd l e
provement may b e achieved from a few ste­
roid inj ections into the subacromial space (not
into the tendon ! ) . A further conservative tool
is ultrasound shockwave therapy. When con­
servative therapy is unsuccessful, or extensive
depositions lead to subacromial impingement,
the focus is to be removed arthroscopically or
by open surgery.
� Arthrosis of the acromio-c1avicular
(AC) j oint
A localized pain develops in the AC j oint
mainly in middle aged or older patients, radi­
ating typically toward the neck. The local ten­
derness over the joint is also distinctive. Simi­
larly to other joints the AC changes may be
caused by primary or post-traumatic arthrosis.
The treatment is basically symptomatic, con­
servative. In stubborn cases resection of the
lateral end of the clavicle and removal of the
degenerated disc are justified.
� Disorders of the sterno-c1avicular (SC)
j oint
These are relatively uncommon. Instabil­
ity occurs as a part of general joint laxity,
which rarely requires treatment, or as
post-traumatic instability, when surgical sta­
bilization is considered.
General j oint inflammations and degener­
ative processes may involve the SC j oint.
There are some conditions of unknown origin,
that cause deformity of this joint or the medial
end of the clavicle (Tietze ' s syndrome,
osteitis claviculae, etc.), which require con­
servative treatment in cases of complaints.
25.6. Glenohumeral i nstability,
shoulder dislocation
Symptomatic pathologic displacement of the
humeral head relative to the g lenoid surface is
cal led glenohumeral instability. Partial dis­
placement of the humeral head is subluxation.
If the humeral head leaves the g lenoid fossa
in its entirety, dislocation occurs.
The humeral head is usually luxated ante­
riorly and caudally, and can be reduced in the
beginning only with medical assistance, after
multiple repetitions, it can even reduce spon­
taneously. The younger the patient, the higher
the probability recurrence of the shoulder dis­
location. About 96% of the dislocations are
anterior or antero-inferior, the remaining 4%
are posterior or multidirectional.
Etiopathology. The stability of the shoul­
der is ensured by different static and dynamic
factors. The static, passive stabilizers are the
thickening of the joint capsule (glenohumeral
ligaments), the glenoid labrum, the negative
joint pressure and to a lesser extent the joint
surfaces. The dynamic, or active stabilizers
are primarily the muscles of the rotator cuff,
secondarily the shoulder blade stabilizers and
ultimately all the muscles in the shoulder gir­
dle.
Shoulder instability may develop for many
reasons, the type of the instability also varies.
Because of the wide range of etiology the
shoulder instability is classified in many dif­
ferent ways:
� By the extent of the instability
- subluxation
- luxation
� By the direction of the instability
- anterior
- caudal
- posterior
- multidirectional
� By the time of the instability
- acute
- inveterate
- recurrent
� By etiology
- traumatic
- habitual
- voluntary
History. The majority of the patient men­
tion some kind of traumatic dislocation and
recurrent luxations after that. The circum­
stances of the injury are essential. The most
common anterior luxation typically develops
 2 5 . D i s o r d e r s of t h e n e c k a n d t h e s h o u l d e r g i r d l e
) X-ray signs. The direction of the disloca­
Fig. 25.17.
Assessment of shoulder instability with su lcus sign.
following an extension-external rotation
movement, while the posterior dislocation is
induced by anteflexion and internal rotation.
The first anterior luxation most often requires
reduction performed by the doctor, later the
patients are themselves able to reduce the
shoulder. The first dislocation following a real
injury may recur later even following the
movements of the everyday life (dressing,
washing, reaching backward, etc.). After
many repetitions, the patient can even pro­
duce the luxation, this is not necessarily iden­
tical to the voluntary dislocation, which is
usually related to psychiatric problems.
Symptoms. Initially the dislocation
causes a major sensation, with painful swell­
ing, elastic blockade of motion and often
paresthesiae in the upper limb. If the shoulder
is dislocated, the acromion protrudes, a de­
pression is seen under it and the humeral head
is palpated caudally and frontally (anterior
dislocation) or toward the back (posterior dis­
location).
In cases of anterior dislocation, if the
shoulder is externally rotated in 90 degrees of
abduction, at the end point of the range the pa­
tient indicates that this motion causes discom­
fort with the feeling that the shoulder will dis­
locate during the assessment (anterior appre-
� 309
hensive test). In cases of posterior dislocation,
anteflexion and internal rotation provoke a
similar sensation (posterior apprehensive
test) . A further diagnostic sign is the
subluxability of the humeral head caudally
(sulcus sign, Fig. 25.1 7.), which is tested by
axial pulling ofthe relaxed arm. A typical fea­
ture of multidirectional instability is the mo­
bility of the humeral head anteriorly or poste­
riorly (anterior and posterior drawer sign).
tion is detected by traditional X-ray test. After
reduction, the native film gives information
on the shape and direction of the glenoid cav­
ity, and on the possible bony B ankart' s lesion,
(Hill- Sachs ' s lesion, Fig. 25.18. a). In case
CT scan can also be done (Fig. 25. 1 8. b, c),
CT arthrography is needed to clarifY the state
of the bone anatomy and the labrum. MRI or
ultrasonography may provide data on the con­
ditions of the labrum and the rotator cuff.
Treatment. In case of acute luxation re­
duction and fixation of the shoulder in internal
rotation for 3 -4 weeks results in lasting
symptomless outcome only rarely. Following
repeated dislocation the mode of treatment
depends on the type of instability and the
etiologic factors.
If dislocation is intentional, the cause of
the personal identity disturbance must be
identified (psychiatric illnesses, family or
school conflicts) and possibly cured. Disre­
garding the etiologic factors, ignoring the in­
stability complaint (the so called skillful ne­
glect) may be helpful, when the patient' s at­
tention is turned away from the significance
of the instability.
In cases of traumatic dislocation rehabili­
tation care for at least 6 months is essential,
the most important elements of which are
strengthening of the rotator cuff and scapula
stabilizer muscles and improving the proprio­
ception in the shoulder area. Surgical treat­
ment is indicated only after failed conserva­
tive therapy, and the circular loose cap­
sule-ligament system is tightened (lower
capsuloplasty) .
310 � 2 5 . D i s o r d e r s o f t h e n e c k a n d t h e s h o u l d e r g i rd l e

b c

Fig. 25.18.
a: I n a case with rig ht habitual luxation the defect caused by the tear of the glenoid labrum is visible together
with the a nterolateral head defect.
b: Impression fracture at the posterior part of the humeral head (Hill-Sachs lesion): a image.
c: Fracture of the anterior glenoid rim (bony Bankart's lesion) a image.

In case of real recurrent traumatic disloca­ The postoperative rehabilitation is deter­

tion operative procedure is the primary mode
of therapy. The type of surgical method de­
pends on the type of injury and the direction of
instability .
mined by the technique used. The patient may
return to full physical activity (physical work,
sport) usually after 6 months. The modem
surgical procedures prevent recurrent
luxation in 90-95% of the cases.
 Jeno Kiss

26. Disorders of the elbow and forearm

26. 1. Functional anatomy brachii and m. brachioradialis, but to some ex­

tent by practically all the muscles that origi­
of the elbow and forearm nate over the elbow and are attached on the
forearm or the wrist.
While the movements ofthe glenohumeral
The forearm is rotated by the pronator and
joint can be compensated by the sliding of the
supinator muscles with the assistance of the
scapula on the trunk, and similarly the motion
extensors and flexors . It should be pointed out
restriction of the scapula can be compensated
th �t the biceps i � not only a flexor, but also a
between certain limits by the glenohumeral
joint movement, the flexion - extension of the �Ulte strong supmator. When the palm is fac­
mg forward, the axis of the extended elbow
elbow can not be replaced by any other func­
positi ?ne � ? eside the trunk is 5-1 5 ° of valgus.
tion. Pronation - supination of the forearm
It IS cntlcal from a surgical anatomical as­
can be compensated with the elbow extended
pect that all three nerves and blood vessels are
?� the movement of the shoulder. At all ages, in the close vicinity of the elbow.
It IS of utmost importance for every individual
�o be �ble to reach both orifices of the gastro­
mtestmal tract. In case of a severe extension
elbow contracture, the mouth is out of reach' 26.2. Assessment of the elbow
in the event of a considerable flexion elbo�
The basic assessment criteria and methods
contracture, the patient is unable to reach the
are fully identical to those described in con­
perineal area. Besides flexion - extension
nection with the assessment of the shoulder
pronation - supination of the forearm is als �
girdle (see details in Chapter 25 .2). In the
important, which is around 90° each. To per­
. course of the examination, the direction of
form this complex movement, the integrity of
movement and the anatomical features of the
the forearm bones, the attached interosseal
elbow j oint must naturally be taken into con­
membrane, and the proximal and distal ra­
sideration. A complex upper limb examina­
dio-ulnar joints is essential .
tion must always comprise such an assess­
In consequence of the shape of the joint
ment.
surfaces and the complexity of the ligament
system, the elbow is a very stable joint. At the
distal end of the humerus, the joint between
26.3. Significant developmental
the trochlea and olecranon ulnae ensures
flexion - extension, while the joint between anomalies
the capitulum humeri and the caput radii
� Congenital dislocation of the radial
chiefly plays a role in the rotation and the sta­
bility of the elbow. Further important compo­ head
nents of the stability are the processus
coronoides ulnae and the medial - lateral col­ This is a com plex developmental anomaly
lateral ligaments. The extension of the elbow with axial deviations of the radius or the ulna,
is car:� ed ou� � y a single muscle, the triceps and the radial head is in a dislocated position.
brachll, and It IS flexed only by the m. biceps It is relatively rare, and may occur isolated or
3 12 � 2 6 . D i s o r d e r s of t h e e l bow a n d f o r e a r m

in association with other developmental Clinical symptoms. It is typical that the

anomalies. pronation - supination movements are miss­
ing.
Treatment. Attempted restoration of
Clinical symptoms. The deformity is
pronation - supination movements through
rarely a source of complaint in childhood, and
plastic surgery interventions usually fails, fol­
is mostly recognized accidentally. The
lowing removal of the bony bridge, even if a
pronation and supination of the forearm are
soft tissue interpositum is created, recurrence
restricted. In adults, the symptoms are
of the synostosis is common.
arthrotic in character.
Treatment. Reduction should be supple­
mented with correction osteotomy, and occa­
sionally with shortening of the radius or the
ulna. When the symptoms present in adult­
hood, resection of the radial head is justified.

� Congenital radioulnar synostosis

Because of a disturbance in the differentiation

of the cartilaginous substance of the forearm
bones, the separation of the two bones is in­
com plete. The synostosis between the radius
and u l na is usually proximal to various extents
(Fig. 26. 1.).

Fig. 26.2.
Bilateral Madelung deformity; clinical (a) and X- ray
pictu re, demonstrating bending of the radius in the
Fig. 26.1. volar and ulnar directions (b), the dorsal protrusion
Bilateral radioulnar synostosis. of the ulna is clearly visible (c).
 2 6 . D i s o r d e r s of t h e e l bow a n d fo rea r m
� Madelung's deformity
Mode/ung's deformity is a g rowth disturbance
of the distal epiphysis of the radius, resu lting
in volar and ulnar bending of the distal radius.
Its occurrence is often familial. It is more com­
mon in girls and often bilateral.
Clinical symptoms. The radius is short­
ened and the distal end of the ulna therefore
protr�des over the wrist to the dorsal surface
of the forearm (Fig. 26.2. a-c) . The deformity
increases with growth. The symptoms are
caused by the restriction of the extension and
radialduction movements of the wrist, and by
the restriction of the supination of the fore­
arm. When the deformity is severe, the grasp­
ing force of the hand may also diminish.
Treatment. If the deformity is extensive,
osteotomy on the distal metaphysis of the ra­
dius may correct the curvature of the bone.
The ulna is shortened at the same time, or its
distal end is resected.
Fig. 26.3.
Elbow destruction caused by rheu matoid arthritis (lateral and anteroposterior X-ray)
� 313
26.4. Acquired disorders of the
elbow
� Arthrosis (osteoarthritis) of the elbow
Arthrosis is a painful restriction of the move­
ment of the elbow, with radiological changes,
which can be primary or secondary (outcome
of inflammation, degenerative, trauma, infec­
tion, etc.). Primary osteoarthritis involves
mainly individuals over 40 who are engaged
in heavy labor. Secondary changes may occur
at any age, with varying incidence.
Etiopathology. In primary arthrosis the
changes observed are similar to those in other
large j oints . The changes primarily affect the
j oint surface; the involvement of the radio­
humeral j oint precedes the ulnohumeral
changes. In the late stages, osteophytes are
typical at the tip of the olecranon and the
coronoid process, obstructing the movements.
In secondary arthrosis the type of the
changes is dependent on the etiology factor
(Fig. 26.3.).
Clinical symptoms. Pain initially appears
only after the exertion of physical effort, but it
3 14 � 2 6 . D i s o r d e r s of t h e e l bow a n d f o r e a r m

later becomes permanent. Early restriction o f during work (tennis players, typists, etc.). It is
the extension and supination is typical, but common among heavy laborers. Both variet­
later movement is restricted in every dimen­ ies are most frequent between the ages of 40
sion. Cracks van be heard and friction felt and 50. Lateral epicondylitis is 5 times more
while moving the joint. The osteophytes sur­ common than the medial form.
rounding the ulnar nerve may cause the com­
pression of the nerve. In case of rheumatoid
arthritis, swelling and occasional instability
are also observed.
Treatment. In any form of elbow osteo­
arthritis, conservative treatment is applicable
for a relatively long period. Conservative
treatment is employed to relieve pain, to
maintain motion range and to protect the mus­
cles. In cases of systemic diseases (rheuma­
toid arthritis, hemophilia, gout, etc.) treatment
of the basic disease is essential. If operative
therapy is needed, joint- preserving and
j oint-replacing procedures are available.
Joint-preserving surgery involves arthroscop­
ic lavage of the joint, removal of inflamed
synovial villi, and in rheumatoid arthritis ar­
throscopic or open synovectomy. Predomi­
nantly in post-trauma cases, the scarred j oint
capsule and the hypertrophied callus that in­
hibits movements can be removed (arth­ a

rolysis). b
In severe cases, an elbow endoprosthesis
is implanted to improve the quality of life
(Fig. 26.4. a, b). This endoprosthesis ensures
proper movement and stability, but the pa­
tients must refrain from excessive physical
activity.

� Epicondylitis humeri lateralis et

medialis (tennis elbow, golfers' elbow)

Lateral epicondylitis of the humerus is called

tennis elbow, and medial epicondylitis is
cal led golfers' elbow. The pain presenting at
the lateral epicondyle is a consequence of
overloading of the forearm extensors, while
the pain occurring at the lateral epicondyle is
the outcome of excessive use of the forearm
flexors.
Fig. 26.4.
The symptoms are often detected in young
X-ray pictu res of an elbow following prosthesis im­
adults who overload the extensor muscles of plantation in rheu matoid arthritis. Anetroposterior
the wrist and fingers while playing sports or (a) and lateral view (b)
 2 6 . D i s o r d e r s of t h e e l bow a n d fo rea r m
Etiopathology. Repeated trauma and de­
generative changes in the area of the origin of
the muscle result first in microscopic tears,
then, in about 30% of the cases, macroscopic
tears are observed.
Clinical symptoms. In tennis elbow, the
patients complain of gradually developing
pain in the lateral part of the elbow. The pain
initially occurs during active movements of
the wrist and when grasping obj ects, but later
it may be present even at rest. The pain in­
creases when the forearm extensors are ac­
tively stretched. The pain can be so intense
that the patient is unable to grip, and drops
grasped and lifted obj ects. The lateral
epicondyle and the extensor muscles are ten­
der to palpation. Golfers ' elbow causes simi­
lar symptoms in the medial epicondyle area,
and the medial epicondyle is tender.
Differential diagnosis. In the case of ten­
nis elbow, radial nerve compression, cervical
root compression and j oint ailments are to be
considered. In golfers elbow, the compression
syndrome of the ulnar nerve, collateral liga­
ment disorders and also cervical root com­
pression must be differentiated.
Treatment. Conservative treatment, usu­
ally lasting for a number of months, proves
successful in 90% of the cases of both disor­
ders. This includes the avoidance of overload­
ing movements, resting the wrist and hand if
necessary, padding applied distally from the
muscle ongm, nonsteroidal anti­
inflammatory drug creams or tablets, local
lignocaine + steroid injections into the painful
areas, and physiotherapy. If conservative
treatment does not bring relief within a rela­
tively long period, various surgical interven­
tions are to be considered.
� Olecranon bu rsitis
Inflammation developing in the bursa over
the olecranon is a relatively common condi­
tion. The inflammation is caused by chronic ir­
ritation of the tip of the elbow, metabolic dis­
ease, a blow in the elbow region, or
hematogenous bacterial infection.
� 315
Clinical symptoms. Depending in the eti­
ology, in acute cases moderate or severe
swelling of the bursa, fluctuation and tender­
ness occur, in septic inflammation, hyperemia
and warmth are detected. In case of chronic
bursitis, the swelling varies, tenderness is
moderate, and fluid accumulation, thickening
of the wall of the bursa, and loose bodies in
the bursa are palpable.
Treatment. In case of sterile inflamma­
tion, the usual anti-inflammatory methods,
and occasionally the drainage ofthe bursa and
steroid inj ection are considered as conserva­
tive therapy. In stubborn cases, surgical re­
moval of the bursa is recommended.
� Instability of the elbow
Slack col lateral ligaments, usually acquired,
but rarely of congenital origin, or deformed
bones of the elbow result in instability of the
joint.
Etiopathology. The elbow is a relatively
stable j oint, nevertheless, elbow luxation due
to high-energy injuries is the second most
common dislocation after that of the shoulder.
The instability may be acute or chronic.
Chronic instability is relatively rare. With re­
gard to the direction, it may be medial, lateral,
anterior or posterior, or usually some combi­
nation of these.
Clinical symptoms. Acute elbow disloca­
tion is usually not difficult to diagnose, con­
sidering the obvious deformity and elastic
block of movement. It is more difficult to
prove chronic instability, which causes only
subluxation. Even an experienced examiner
can only establish proper diagnosis after sum­
marizing the circumstances of the onset of the
complaints, the results of careful physical as­
sessment and an X-ray examination.
Treatment. If no fracture is involved, an
acute elbow dislocation, requires closed re­
duction and depending on the degree of insta­
bility following reduction, transitional immo­
bilization ( 1 -3 weeks), followed by early
functional treatment. In cases of chronic insta­
bility, depending on the characteristics and
3 16 � 2 6 . D i s o r d e rs of t h e e l bow a n d forea r m

extent and also on the physical activity and

age of the patient, conservative treatment is
advised: modification of the sporting and vo­
cational activities, strengthening of the mus­
cles bridging the elbow and occasionally ex­
ternal orthesis. Surgical procedures depend on
the cause and type of the instability.

� Acquired axial deformities

In consequence of very frequent elbow frac­

tures in child hood, varus deformity is com­
mon due to the damage to the growth plate.

A mild deformity is usually merely a cos­

metic problem. In more severe cases, how­
ever, the entire function of the upper limb is
altered, especially when the varus deformity
is complicated with a flexion contracture of
the elbow.
a
The diagnosis is unmistakable in view of
b the characteristic clinical and radiological
signs. Milder cases do not need treatment. In
more severe cases of varus with a flexion
contracture, correction osteotomy in the hu­
merus distal metaphysis may be justified (Fig.
26.5. a, b) .

� Tunnel syndromes of the elbow

These clinical entities are characterized by

pain and occasional paresthesia radiating
from the elbow to various areas of the fore­
arm or of the hand. The ulnar nerve is most
commonly involved. Compression of radial
nerve is less frequent, and the median nerve
compression is rare.

Etiopathology. Nerve compression may

Fig. 26.5.
40° post-traumatic varus deformity of the rig ht el­
bow of a 10-year-old child (a), and following correc­
tion by humerus osteotomy (b)
take place where the nerve passes certain ana­
tomical locations and is compressed by a scar,
an abnormal muscle belly, an osteophyte, a
callus or a tumor. Stretching or tension of the
nerve may be caused by severe post-traumatic
or degenerative deformity of the elbow (see
also in Chapter 1 7) .
Treatment. Conservative therapy may be
successful only in minor cases. Rest and use
 2 6 . D i s o r d e r s of t h e e l bo w a n d fo rea r m
of a night splint in attempt to decrease the
edema caused by the compression of the
nerve. In more severe cases, surgical decom­
pression of the nerve may be necessary.
26.5. Volkmann's ischemic
contracture of the forearm
and the hand
This clinical picture arises following injuries or
the application of a tight plaster of Paris ban­
dages. The typical contractures are produced
by the ischemic necrosis of the forearm mus­
culature. The syndrome was first described by
Volkmann.
Etiopathology. This syndrome usually
appears following a supracondylar humerus
fracture in childhood, elbow luxation, or use
of a tight plaster of Paris bandage applied for
some other reason, but it can also be caused by
a misplaced tourniquet cuff. Ischemic necro­
sis develops in the foreann musculature, re­
sulting in scarring of the muscles. In certain
cases, damage to the ulnar, radial and median
nerves is observed.
Clinical symptoms. Early symptoms may
be identified merely a few hours after the in­
jury. The hand is cool, swollen and purplish
blue. A change in sensitivity and loss of mo­
tion to various extents may occur in the fin­
gers. The developed Volkmann contracture
displays a typical picture: flexion contracture
of the elbow, pronation ofthe foreann, flexion
and a mild ulnar deviation contracture of the
wrist, extension in the metacarpophalangeal
joints, flexion in the interphalangeal joints,
and an opposition contracture of the extended
pollex. The fingers exhibit a claw hand defor­
mity (Fig. 26.6.) .
� 317
Treatment. Prevention is essential. The
risk of an ischemic contracture must be borne
in mind in cases of elbow injuries, and reduc­
tion and positioning of the plaster of Paris
must be carried out accordingly. A circular
plaster must not be used in elbow injuries. The
circulation must be checked regularly, and the
attention of parents must be drawn to this. I f a
minimal circulatory, sensory o r motor distur­
bance is detected in the hand, the bandage
must be loosened immediately and close ob­
servation must follow. An important clinical
symptom is the pain on passive flexion of the
fingers, due to the increasing pressure in the
muscle compartments. If this state does not
improve quickly, fasciotomy of both flexor
and extensor compartments on the elbow and
the foreann must be perfonned immediately
in order to decrease the pressure in the com­
partments and restore circulation.
When a Volkmann contracture has already
developed, the treatment is long-lasting.
Contracture of the fingers may be improved
by exercises, or in more severe cases by me­
chanical methods. In mild cases, these meth­
ods may even result in full recovery. In severe
cases a surgical solution may be needed. De­
pending on the extent of the defonnity, the
hand function may be improved by
tenotomies, muscle transfers or wrist
arthrodesis.
Fig. 26.6.
Typical clinical picture of the Volkmann's
contractures
 Fe r e n c M a d y

27. Disorders of the hand and the wrist

27. 1. Functional anatomy hypothenar groups . These muscles provide

the fine (but weaker) motions of the fingers .
of the hand and the wrist The peripheral nerves (the median and the
ulnar nerve) reach the hand through pre­
Beside its motor function, the hand also
formed anatomic tunnels (the carpal tunnel
acts as a sensory organ. The bones of the hand
and Guyon ' s tunnel). Both nerves are of
are the carpal bones, the metacarpal bones and
mixed type.
the phalanges. Special attention should be
There is a separate nerve on both sides of
paid to the first metacarpal bone due to the
each finger.
� a?dle joint and the metacarpophalangeal The blood supply of the hand is provided
Jomt. The other metacarpals are characterized
by the end-branches of the radial and the ulnar
by the shape of their distal articular surface
arteries. The anatomic structures of the hand
(ball joint).
are covered by the palmar fascia .
As concerns the joints, the loose ligaments
?� the saddle joint (the first carpometacarpal Clinical examination of the hand. The
Jomt) and the eccentric location of the liga­
easiest and quickest way to examine the hand
ments of the second to fifth metacarpo­
is to apply the scheme described by Erik
phalangeal joints should be taken into consid­
Moberg, which consists of four steps (Fig.
eration.
27.1 a-d) :
The muscles of the hand are divided into
three groups: the thenar, mesothenar and

a b d

Fig. 27.1.
The Moberg's scheme: a method for rapid assessment of the hand-function.
320 � 2 7 . D i s o r d e rs of t h e h a n d a n d t h e w r i st
a) Examination of the integrity of the long
flexors and extensors. The patient is re­
quested to extend the fingers fully, and
then to flex them slowly while keeping the
metacarpophalangeal joints extended. If
this can be accomplished properly, the
long flexor and extensor muscles and ten­
dons are intact. When the superficial
flexor tendons are examined, the fingers
are kept in extension, and the proximal
interphalangeal j oints are then flexed one
by one. If there is a lesion of the superficial
flexor tendon, the finger cannot be flexed
in the proximal interphalangeal joint in
this way.
b) The motor function of the ulnar nerve can
be checked by adduction of the thumb and
ab- and adduction of the long fingers. A
weakened thumb adduction indicates a de­
teriorated function of the ulnar nerve. The
patient is requested to hold a sheet of paper
between the thumb and the edge of the
palm. If the adduction function preserved,
considerable force is required to remove
the paper sheet. The abduction strength of
the fingers is tested against resistance. The
closing strength of the fingers is checked
by attempting to open them. If the patient
resists well, the innervations of the intrin­
sic muscles are intact.
c) The median nerve is responsible for the
opposition-reposition of the thumb. When
testing the opposition, the patient is re­
quested to form a circle with the thumb
and the little finger and to keep them
tightly together. If the fingers can be sepa­
rated easily, the opponent muscle strength
is weakened.
d) The last step is to test the sensitivity of the
hand according to the supply area of the
three nerves . A small blunt obj ect is to be
used for the examination. If the superficial
sensation is preserved, the patient reports
good sensation in the palm, in the lateral
aspects of the fingers and in the palmar as­
pect of the fingertips . The limit between
the sensory areas of the median and the ul­
nar nerves is the midline of the ring finger
toward the wrist crease. The sensory areas
on the dorsal aspect of the hand differ from
those in the palmar aspect. Distally from
the proximal interphalangeal joints, the ar­
eas are the same, but the rest of the dorsal
aspect is supplied by the radial and ulnar
nerves. The border between them is the
midline of the middle finger.
More accurate methods for examining the
sensitivity of the hand are the two-point dis­
crimination test; the touch test, the pain- and
the pick-up tests. Recognition of objects is en­
sured by the simultaneous activity of the ther­
mal, pain and touch receptors. Ifthese are sev­
ered, the patient is not able to recognize
smaller obj ects without eye control. If there is
only a minor impairment, the two-point dis­
crimination increases from the normal 1 -2
mm up to 5-6 mm or even more. In case of
more severe nerve damage, all the sensory
functions are affected and the function of the
hand deteriorates considerably.
Both the regulation of the perspiration and
the sensitivity of the hand are linked to the
digital nerves. This can explain the phenome­
non that, if the digital nerve is severed, the
skin is dry and the perspiration is impaired.
On percussion of the damaged peripheral
nerve, electric shock-like pain is evoked at the
site of the nerve injury or nerve compression
( Tinel' s sign). The site of the nerve damage
can therefore be accurately determined.
The blood supply for the hand is provided
by the radial and the ulnar arteries. The
interosseal artery also provides some
branches flowing from the wrist. These pres­
ent several anatomic variations and do not
provide sufficient blood supply for the hand
by themselves.
The neutral 0 method is used to describe
the range of movement of the hand. The joints
are in the 0 position when the fingers are ex­
tended.
The functional position of the hand:
The optimal grip position is the best for the
function of the hand (Fig. 27.2.).
The functional position is borne in mind
when the hand is immobilized: the articular
ligaments adapt quickly (even within one or
 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
Fig. 27.2.
Functional position of the hand. The metacarpo­
phalangeal joints are flexed at 60° in the second and
90° in the fifth finger. The wrist is in 30° dorsiflexion.
The proximal interphalangeal and distal inter­
phalangeal joints are bent at 20-30°. The best grip is
provided in this position of the hand.
two weeks) to the length they had during im­
mobilization. This explains the fixed deformi­
ties developing during immobilization. These
deformities can be prevented by immobiliza­
tion in the functional position.
Fig. 27.3.
Types of syndactyly
a: Sketch of an X-ray image indicating that in cutaneous syndactyly the bones are not affected and separated
as in a normal hand.
b: In osseous syndactyly the bones are missing or joined.
c: In spoon-hand, all the fingers have a common skin glove; certain bones are missing or deformed.
� 321
Surgery on the hand is performed under re­
gional or general anesthesia and with the use
of tourniquet bloodless .
The hand may be affected b y numerous or­
thopedic disorders . However their incidence
can be different.
27.2. Congenital anomalies
of the hand
The upper and lower extremities develop
simultaneously between the fourth and sev­
enth week of the embryonic life . The hand de­
velops through the growth and differentiation
of the limb buds. If the embryo is exposed to
genetic or direct toxic effects, various devel­
opmental failures may occur in the hand (and
often in the foot) .
� Syndactyly
In syndactyly, two or more fingers are par­
tially or completely j oined. Sometimes only
322 � 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
the skin is common (cutaneous syndactyly),
but in more severe cases, the bones are also
common (bony syndactyly) . In the most se­
vere case, all five fingers are involved (spoon
hand) (Fig. 27.3. a-c).
In cutaneous syndactyly, the otherwise
separate fingers are covered by a common
skin (Fig. 27.4.). The nail may be deformed at
its base. Possible variations of the blood ves­
sels should be clarified before surgery is de­
cided on. Angiography is to be performed to
exclude any vascular abnormalities.
Separation of the fingers needs an experi­
enced surgeon. A preferred age for such inter­
ventions is at the age of 2-3 years . Accurate
reconstruction of the web between the fingers
is essential in order to avoid scars and conse­
quent fixed deformities.
� Ectrodactyly
This is the congenital absence of a fin­
ger(s).
� Cleft hand
This congenital absence of one or more of
the central (second to fourth) fingers is also
called lobster-claw hand. The cosmetic ap­
pearance is usually worse than the functional
impairment.
� Polydactyly
This denotes the presence of an extra digit,
which may be complete or the rudimentary
part of a finger or thumb.
Fig. 27.4.
Cutaneous syndactyly. The middle and
ring fingers are joined in their entire
length. Due to the growth of the fingers,
the otherwise longer middle finger bends
ulnarward. After separation, the fingers
continue growing proportionately.
� Congenital trigger thumb
This involves congenital stenosis of the
flexo tendon sheath ofthe thumb. Surgical in­
cision of the tendon sheath is indicated at an
early age.
Congenital anomalies of the hand exhibit
great variety and many combinations are ob­
served.
The optimal timing for surgery is around
the age of 2-3 years . If several interventions
are necessary, these should be completed be­
fore the first school year.
27.3. Acquired hand disorders
27.3.1. Avascular necrosis of the
carpal lunate bone
(Kienbock's disease)
This d isease comprises necrosis, fragmenta­
tion and collapse of the l unate bone in adults;
it is of unknown etiology. It was first de­
scribed by KienbOck who presumed a blood
supply distu rbance leading to bone necrosis.
The pathomechanism and the outcome dif­
fer from those in juvenile osteochondritis. In
Kienbock' s disease the necrosis of the lunate
bone is definitive, while in juvenile osteo­
chondritis the necrosis is followed by regener­
ation of the bone.
 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
Fig. 27.S.
Avascu lar necrosis of the lu nate bone. The X-ray im­
age clearly shows the smaller, sclerotic, deformed
lunate bone with cystic lesions.
Fig. 27.6.
Pseudo-articulation of the scaphoid bone. The ar­
row indicates the fracture. In the radiograph, the en­
largement of the false joint line is clearly visible. The
bone has not yet deformed.
� 323
The disease occurs most frequently in
males who perform heavy manual work, (re­
peated micro traumas, use of a pneumatic
hammer, or activities in a smithy) around the
age of 3 0 .
Clinical features. Pain o n palpation and a
painful swelling of the dorsal aspect of the
wrist. The patients usually connect the onset
of the symptoms to some trauma in the past.
Pain increases upon exertion.
Movements of the wrist are painful and
limited in all three planes . In a later stage, the
range of extension decreases markedly, and a
fixed flexion deformity and atrophy of the
forearm muscles can be observed.
Radiological signs. In the early stages,
there are no characteristic radiological alter­
ations. Later, the bony structure of the lunate
changes, and it exhibits an increased density
with cystic lesions . In the lateral view, there is
a dip in the lunate (Fig. 27.5.).
Treatment. Physiotherapy and immobili­
zation of the wrist (splint or brace) in the early
stage. The administration of nonsteroidal
anti-inflammatory drugs can relieve the pain
considerably.
If conservative treatment fails, surgery is
indicated.
27.3.2. Cyst and pseudoarthrosis of
the scaphoid bone
Fracture of the carpal scaphoid bone, the
most common fracture of the carpus, can re­
sult in nonunion in a considerable number of
cases.
This fracture is often not diagnosed at the
first examination. The precarious blood sup­
ply of the scaphoid is at risk and pseudo­
arthrosis may develop in the fracture line, fol­
lowed by necrosis of the proximal broken
fragment (Fig. 27.6.). Cystic changes may oc­
cur in the fracture line or even without a frac­
ture.
3 24 � 2 7 . D i s o r d e r s of t h e ha nd a nd t h e w r ist

Clinical features. There is usually an old

injury in the medical history which is often re­
vealed only when the patients are requested to
recall a possible injury that they suffered in
the past. The fovea radialis (fossa tabatiere) is
swollen. Dorsiflexion and radial abduction of
the wrist provoke pain. There is tenderness
over the scaphoid bone.

Radiological signs. If a scaphoid fracture

is suspected, radiographs of the wrist are
taken in four planes (scaphoid views) .

Treatment. If nonunion has developed,

surgical treatment is usually performed. Sev­
eral techniques are available, but their princi­
ples are the same : removal of the dead bone
fragments; filling with cancellous bone grafts;
and internal fixation (pin, screw, Ender 's
plate etc.).

27.3.3. Osteoarthritis (OA) Fig. 27.7.

of the wrist and the hand
Osteoarthritic changes a re more common in
the hand than in the hip or knee joint. They
are of great importance because they can
compromise the function of the hand.
Osteoarthritis of the saddle-joint. The joint su rfaces
of the os trapezium and the first metacarpal that
are facing each other are deformed, the bones are
sclerotic. The first metacarpal subluxates towards
the radial.

Etiology. A previous injury, an articular
fracture or chronic exertion (activities of a
typist or a decorator) may be found in medical
history. It may also develop spontaneously in
the elderly.
Clinical features. There is gradually in­
creasing pain in the j oints of the hand, usually
in the midline of the dorsal aspect of the wrist.
The affected j oint becomes swollen and pain­
ful, and the strength and ability to grip obj ects
diminish. The most commonly affected joints
are the radiocarpal joint, the saddle j oint and
the first metacarpophalangeal joint.
Radiological findings. The usual osteo­
arthritic changes can be detected. In wrist
osteoarthritis, the styloid of the radius be-
comes elongated. The radiocarpal joint space
becomes thin first on the radial side. The
proximal carpal bones flatten and deform, and
degenerative bone cysts may appear.
In osteoarthritis of the saddle joint, the
base o fthe first metacarpal subluxates or may
even dislocate. The radius of the thumb short­
ens (Fig. 27.7.).
Osteoarthritis of the radiocarpal joint
(wrist) is treated first conservatively, with im­
mobilization, pain control and physiotherapy.
(Fig. 27.8.).
In contrast with the earlier practice, ad­
ministration of topical steroids is to be
avoided. The use of cortisone preparations
can result in disintegration of the substance of
the joint capsule, ligaments and tendons, lead­
ing to an impaired mechanical quality.
 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
Fig. 27.8.
Osteoarthritis of the radiocarpal joint. The joint sur­
faces are thin, and the styloid process is pointed.
The scaphoid bone displays pseudo-articu lation
(secondary wrist osteoarthritis)
Immobilization can be achieved by the ap­
plication of plaster of Paris splints or wrist
braces. Other types of braces can involve the
saddle joint kept in opposition.
27.3.4. Inflammatory processes
During work, the hand is exposed to a
wide variety of physical effects. Besides
overexertion, injuries varying in severity can
occur.
Injuries in the skin enable pathogenic mi­
crobes to penetrate the hand, causing various
pyogenic infections. Septic conditions of the
hand are dangerous because the infection can
spread rapidly along the anatomic structures
� 325
(tendon sheath and bursa). Edema and soft­
tissue distension can lead to a deterioration of
the circulation causing necrosis and/or loss of
a part of the hand.
� Tendovaginitis crepitans
This is a non-pyogenic inflammation of the
tendon sheath. Its mildest form occurs in the
dorsum of the wrist due to overexertion of
the hand (typist or pianist activities). Synovial
effusion develops inside the tendon sheath
and fibrin precipitation occurs there.
Clinical features. Pain and swelling in the
dorsal aspect of the wrist. Crepitation (a
snow- crunching-like sensation) may be expe­
rienced along the extensor tendons while the
wrist is moving. There is tenderness on palpa­
tion over the swollen j oint. The grip strength
of the hand diminishes considerably.
Treatment. Immobilization of the wrist
and the fingers for 2-3 weeks and administra­
tion of nonsteroidal anti-inflammatory drugs.
� Stenosing tenosynovitis
This condition is characterized by stricture of
the tendon sheath. Depending on which ten­
dons are affected, De Quervain's disease and
trigger finger (or trigger thumb) are distin­
guished.
De Quervain ' s disease. Chronic inflam­
mation and stenosis occur in the common ten­
don sheath of the abductor pollicis longus and
extensor pollicis brevis tendons at the level of
the styloid process o fthe radius. The mobility
of the thumb is reduced and the grip strength
is greatly diminished due to pain (Fig. 27.9.).
Clinical features. Besides the pain there
is swelling and tenderness on palpation in the
radial aspect of the wrist. The pain is severe
on palpation in the styloid process.
Finkelstein ' s test is usually positive : pas­
sively abducting the hand ulnarward provokes
pain at the site of the tenderness. The inj ection
of hydrocortisone results in only short-term
pain relief, therefore this type of drug should
326 � 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
_ u�- :
:
,
,: "*
,_ _ / �
be avoided. Surgical incision of the tendon
sheath results in a definitive recovery.
Trigger finger and thumb (digitus saltans -
pollex saltans). A nodule is observed on the
tendon with relative stenosis of the tendon
sheath. As the tendon glides through the
sheath, a snapping movement can be seen
and palpated usually accompanied by pain. I n
more severe cases, there is not enough space
for the nodule to glide through the constric­
tion. The patient is u nable to flex or extend
the affected finger. The stenosis is located at
the level of the metacarpophalangeal joint.
At the entry of the tendon sheath, there is a
strong fibrous annulus, stabilizing the tendon
on its base thus providing smooth gliding.
Either the tendon sheath becomes thicker
due to inflammation or the tendon develops a
swelling (intratendinous ganglionic cyst or tu­
mor) . This can lead to relative stenosis there.
The tendon moves through the stenosis only
upon forced flexion or extension, accompa­
nied by a snapping (Fig. 27.1 0.). In adults,
1 on the entry of the tendon sheath or on the
•
2
3
4 \
\ \\.��
�
Fig. 27.9.
The abductor pollicis longus tendon (1)
and the extensor pollicis brevis tendon
(3)in the first dorsal compartment. Close
attention should be paid to the radial
1 nerve and its sensory branches. The sur­
gery comprises the incision of the first
dorsal tendon compartment (2).
trigger finger usually occurs on the long fin­
gers. Trigger thumb may be observed congen­
itally in children. If the constriction is severe,
the thumb remains fixed in flex ion (congeni­
tal pollex flexus ).
Treatment. Conservative treatment usu­
ally fails or provides only temporary results.
Surgical treatments results in an immedi­
ate and definitive recovery, and thus attempt­
ing conservative methods for a longer period
is meaningless. Surgery comprises longitudi­
nal incision of the constricted part of the ten­
don sheath.
27.3.5. Ganglion of the wrist and
the hand
Gangli onic cysts usually arise from struc­
tures lined with synovial membrane Uoints,
bursae and tendon sheaths). They contain yel­
lowish synovial fluid. They have an outer fi­
brous capsule and an inner synovial mem­
brane.
Fig. 27.10.
Mechanism of digitus saltans: the thickening
tendon itself blocks the movement of the ten­
don. Passed this stenosis the tendon moves
freely. 1. metacarpus, 2. flexor tendon,
3. thickening of tendon, 4 and tendon sheath
\
 2 7 . D i s o r d e r s of t h e h a n d a n d t h e w r i st
Fig. 27. 11.
Ganglion of the hand. The cystic lesions arise most
frequently from the dorsal capsule of the
scapholunate joint.
Pathology. The cause of ganglionic cysts
is unclear. They commonly develop on the
dorsal (Fig. 27.1 1 .), or occasionally on the
palmar surface of the wrist. Ganglionic cysts
in the wrist area are usually attached to the
capsule of the radiocarpal joint. While the
cysts are growing, tissue around the cysts is
lifted, i. e. tissue wraps around the cyst. In case
they develop on the palmar surface, they may
encompass the radial artery.
27.3.6. Dupuytren ' s contracture
This is a benign but aggressive superficial
proliferative fibroplasia involving shrinking of
the palmar or plantar fascia and resulting in
contracture of the finger joints.
Clinical features. The lesion usually be­
gins with a small nodule (rarely several nod­
ules) situated in line with the ring or the little
finger (Grade I). After a few months or even a
year, these nodules develop into a cord (Grade
II). In parallel with its thickening, extension
of the affected finger becomes limited and a
flexion contracture develops. Later the meta-
� 327
Fig. 27.12.
Dupuytren's contracture. A strong nodular thicken­
ing affects the palmar fascia above the IV. ray, which
decreases significantly the range of movement in
the MP and PIP joints.
carpophalangeal j oint is fixed in a 90° flexion
(Grade Ill), and then a proximal inter­
phalangeal flexion contracture occurs (Grade
IV, Fig. 27.1 2.) and the patient is unable to
move the finger. The other fingers are much
more rarely involved.
The sensory ability of the fingers is not af­
fected.
Repeated trauma may be a cause but more
than half of the patients have never been
heavy physical workers .
Pathology. The nodules initially consist
of curly connective tissue bundles rich in cells
and poor in fibers . These bundles may extend
to the distal phalanges and start to shrink. At
this stage mature connective tissue with an in­
creasing amount of collagen fibers can be de­
tected. The subcutaneous tissue between the
cord and the skin gradually disappears and the
thick palmar fascia is covered by only a very
thin layer of skin.
Treatment. In the early stages, the pa­
tients usually have no complaints and only ob­
servation is necessary. When the bundles start
to form, surgery can be performed.
328 � 2 7 . D i s o r d e r s o f t h e h a n d a n d t h e w r ist
27.3.7. Tumors and tumorous
conditions i n the hand
Any tumor that may arise in the locomo­
tion system and the dermal tissue may also oc­
cur in the hand, but each has its own charac­
teristic incidence. Malignant tumors are rare.
Among the tumorous conditions, seba­
ceous cysts are often found beneath the skin
of the palm or the fingers. These cysts grow
slowly, lying just underneath the skin. They
are bordered by a white and strong fibrotic
capsule filled with a white mass consisting of
liquefied dermal elements, sebum and
corneous debris.
Among benign tumors, benign
synovioma, the giant cell tumor of the tendon
sheets, is frequent. This tumor is usually
found in the fingers and grows slowly. The
X-ray films reveal a lytic lesion with a sharp
margin (like an impression) on the bones next
to the tumor. When surgically exposed, a
grayish- white lobular structure with yellow­
ish spots in some places can be seen. The tu­
mor can be easily dissected from the sur­
rounding tissue, but often involves as much as
three-quarters of the circumference of the
digit. After excision, recurrence often occurs.
The most common benign tumor is the
enchondroma, which is a tumor of cartilagi­
nous origin (See chapter 2 1 , Fig. 2 1 . 1 5 .).
Malignant tumors in the hand are ex­
tremely rare. Among these, synovial sar­
coma and chondrosarcoma are relatively of­
ten observed.
 Arpad B e l lyei, J6zsef La katos, M i kl6s Sze n d r6i

28. Hip disorders

28. 1. Pediatric hip disorders better developed and covers the femoral head
better than the anterior part (the pubic bone;
Fig. 28.1.). The anterior segment of the femo­
28. 1.1. Normal development of the ral head is only partially covered; the further
hip joint shell is provided by the active anterior
acetabulum wall, the tense substance of
� Development of the acetabulum and
iliopsoas muscle, which is of maj or clinical
pelvis
significance.
The three bones that together form the
Ossification of the proximal and middle
acetabulum and the pelvis develop from three
parts of the femoral bone is organized by three
ossifying centers . The bony center of the iliac
centers : in the second embryonic month, a
bone, which forms the upper part of the
bony center appears in the middle of the
acetabulum appears in the third embryonic
diaphysis and progresses in both directions of
month, followed in the fourth month by the
the diaphysis. The epiphyseal bony center of
ossifying center of the sciatic bone, which
forms the posterior part of the acetabulum,
and in the sixth month by the ossifying center
the of pubic bone, which forms the anterior
part of the acetabulum. The ossifying centers
rapidly develop, and at the time of birth these
three bones are divided by the Y-shaped
growth plate located in the depth of the
acetabulum, ossification occurring at the end
of development, between the ages of 1 4 and
1 6 years . The growth of the edge of the
acetabulum is controlled by an extra ossifying
center (os acetabuli) . The fully developed
acetabulum is a 1 70- 1 75° segment of a sphere
that contains loose fat and connective tissue in
its base (pulvinar acetabuli).
There is a hyaline cover only on that
half-moon-shaped part, which is in contact
with the femoral head. The fibrous - cartilagi­
nous rim on the edge of the acetabulum, the
acetabular labrum, makes the cup deeper, so
that it contains almost three-quarters of the
femoral head. The femoral head is held in a
central position in the acetabulum by the cap­
sule, the enforcing ligaments and the muscles Fig. 28.1.
bridging over the joint. The posterior bony Right hip frontal view: the bone cover is less anteri­
part of the acetabulum (the sciatic bone) is orly than posteriorly.
330 � 28. H i p d isorders
the femoral head emerges i n the fourth-sixth
month of extrauterine life, the ossifying center
of the greater trochanter at the age of three
years, and that of the lesser trochanter at the
age of 8. The epiphysis later forms the j oint; it
is covered by hyaline cartilage, while the
apophysis serves as the origin and attachment
for muscles.
� Progress of collodiaphyseal angle
In adults, the angle between the femoral
neck and stem is 1 25 - 1 3 5 ° . This angle varies
from early embryonic life to puberty. In em­
bryonic, life the angle first decreases, then
gradually increases, and its average value at
birth is 1 40°. During the first year it increases
to 1 48°, and it then gradually decreases to the
adult level. If this angle exceeds the normal
value for the given age, the condition is called
a valgus deformity, if it is less, it is a varus de­
formity. Accordingly, mild physiological
coxa valga is observed in early infancy rela­
tive to the adult situation. When the roof of the
j oint is steep and dysplastic, and its covering
:�
'�
- - . :� �
to
Fig. 28.2.
Right hip frontal view: the collodiaphyseal (a) and
antetorsion (�) ang les; the line d rawn through the
distal femur condyles represents the frontal plane.
pressure effect on the loaded femoral head is
therefore insufficient, valgus hip develops.
� Development of antetorsion
Under normal circumstances, the projec­
tion of the femoral head and neck and
transcondylar axis in the horizontal plane has
an anteriorly open angle; this is the angle of
antetorsion (Fig. 28.2.). The origin of this an­
gle is the forward torsion of the proximal fem­
oral bone (the head looks forward and the
greater trochanter slightly rearward). The av­
erage value of antetorsion in adults is 1 0- 1 4°.
� Blood supply of the proximal femoral
bone
The blood supply in extrauterine life must
be considered in three phases :
- Between the ages of 1 and 3 years, the
blood supply of the proximal two-thirds
of the femoral head, the epiphysis, is
provided via the artery of the liga­
mentum teres capitis femoris from the
arteria obturatoria (Fig. 28.3.). The
growth plate is located distal to the
epiphysis; it is an avascular area pre­
senting a vascular blockage between the
epiphysis and the metaphysis.
Fig. 28.3.
The ligamentum teres capitis femoris with the sup­
ply artery.

- Between the ages of 3 and 1 4 years the
proximal epiphysis of the femur is
subject to relative ischemia, since the
capacity of the arteria obturatoria is then
greatly decreased. From this period, the
blood is supplied to the proximal epi­
physis by the lateral epiphyseal artery
related to the vessel plexus around the
metaphysis (a. circumflexa femoris
lateralis et medialis). This vessel runs
around the growth plate laterally and
posteriorly and then enters the j oint
space, reaching the ossifying center of
the epiphysis and providing the blood
supply directly (Fig. 28.4.). This means
that a short segment of the supply artery
runs unprotected, in an extraosseal and
intracapsular position and is exposed to
injuries. In this period, any process
under the tense hip joint capsule (syno­
vitis or edema) may occlude the
supplying artery, causing necrosis (see
Perthes ' disease).
- Around the age of 14 years, the growth
plate gradually ossifies, and the intra­
osseal arteries of the metaphysis
Fig. 28.4.
The lateral epiphyseal artery runs round the growth
plate and supplies the epiphysis.
28. Hip disorders � 331
gradually invade the distal part o f the
epiphysis and take part in its blood
supply.
� Nerve supply of the hip j oint
About two-thirds of the hip j oint is sup­
plied with sensory fibers by the obturator
nerve, which originates from segments L2-4
and runs in the medial edge of the psoas maj or
muscle, reaching the medial aspect of the
thigh through the lateral - upper aspect of the
canalis obturatoria. It provides sensory
branches to the hip joint, and then runs dis­
tally between the adductor muscles providing
motor branches most of the adductor muscles
and a sensory branch for the small skin area at
the distal - medial aspect of the thigh and the
knee. The essence of these features is, that in
adults, but particularly in children, the pain re­
lated to hip disorders appears not so much in
the hip area, but also in the distal thigh or in
the knee.
28. 1.2. Congenital dislocation and
dysplasia of the hip
Synonyms of this condition are hip
dysplasia, acetabulum hypoplasia, congenital
dislocation of the hip, developmental disloca­
tion ofthe hip, and dislocation of the hip in in­
fants.
Definition. This is congenital dysplasia of the
acetabulum, which may lead to either intra- or
extrauterine consecutive hip dislocation.
Pathologic forms
- The mildest form is hip dysplasia
(acetabulum hypoplasia), when the
femoral head is located centrally in the
acetabulum, but the acetabulum is
underdeveloped, and the acetabulum
angle is steep (Fig 28.5.) .
- Subluxation: In cases of maj or
acetabulum hypoplasia, the femoral
head may be displaced cranially. The
cartilaginous elements of the aceta-
332 � 28. H i p d isorders
() �n
".----.
(
- ronmental factors acting collectively are re­
Fig. 28.5.
Pathologic forms of hip d islocation: dysplasia,
subluxation and luxation.
bulum and the femoral head are still
partially coupled.
- Luxation: The femoral head is displaced
proximally and dorsally from the
acetabulum.
Incidence. Hip dysplasia is classified as a
frequent developmental disorder; its inci­
dence in Hungary is estimated to be around
0 . 5 % . This includes both dysplastic and dislo­
cation cases which require treatment.
Its incidence is dependent on the geo­
graphical regions and on the ethnicity. In Cen­
tral Europe the incidence is high, but in Negro
sub race and in China the incidence is very
low. In Japan however the incidence is very
high. Hip dislocation is 6 times more frequent
in girls than in boys .
This entity was already described by Hip­
pocrates, however, the current nomenclature
and approach are associated with the activity
of Lorenz ( 1 895). He stated, that children are
not born with hip dislocation, but only with a
predisposition; the dislocation occurs only as
a result of walking - loading, due to the under­
developed hip j oint. As early as 1 879, Roser
explained that hip dislocation, or a predisposi­
tion to it, can be recognized in newborns, and
added, that these children may heal, provided
they wear special nappies right after birth,
which ensure an abducted position.
Hilgenreiner introduced the term hip dys-
plasia, denoting the maldevelopment of the
entire hip joint.
As proposed by the European Pediatric
Orthopedic Society, the present terminology
is: developmental dislocation of the hip
(DDH) .
Etiopathology. DDH is a multifactorial
developmental abnormality. The term multi­
factorial means that both the inheritance fac­
tors (mainly a predisposition) and the envi­
sponsible for the occurrence of luxation.
The genetic, hereditary factors (predispo­
sitions) appear as acetabulum hypoplasia,
which is the primary cause of this polygene
inheritance. Hereditary factors may also be
manifested as the dominantly inherited gen­
eral joint laxity.
Both intra- and extrauterine environmen­
tal factors may contribute. Intrauterine causes
include any compartmental disproportion
(such as breech presentation, other position­
ing disorders, or tight intrauterine situations),
and transitional joint laxity is particularly im­
portant. The essence of this is that in the sec­
ond and third trimesters of pregnancy, the lev­
els of estrogen and progesterone increase in
both the mother' s and the embryo ' s blood,
since their circulation is common. It is well
accepted that both estrogen and progesterone
cause j oint laxity. The transitional joint laxity
endorses the expansion of the vaginal mus­
cles, and increases the flexibility of the joints
of the embryo. The newborn ' s hormone level
gradually decreases, and normalizes by the
3 -4th week. Hence, the newborn is in a bor­
derline state for 3 weeks regarding joint sta­
bility, when the femoral head may separate
from the joint (unstable hip). The two most
important extrauterine factors are the inappro­
priate use of nappies fitting tightly on the
lower limbs, and the erect position, i.e. the at­
tainment of walking itself. From the moment
of birth, the newborn assumes the so called re­
laxed - sleeping or sprawled posture, which is
characterized by almost 90° offlexion and ab­
duction of the hip joint (Fig. 28.6.). In a
sprawled position, the femoral head is sunk

Fig. 28.6.
Relaxed - sleeping posture of the newborn: almost
90° flex ion, abduction and external rotation.
deeply in the acetabulum, enhancing the nor­
mal development of the acetabular roof, even
in dysplastic-hypoplastic cases. The treatment
follows the same path. Many clinical findings
relate to environmental factors, e.g. 1 6% of
babies with hip dislocation exhibit breech pre­
sentation.
Etiologic forms of hip dislocation.
1 . Hip dysplasia or consequential dislocation
on the basis of acetabular hypoplasia
(polygenic form) can be well managed
with conservative treatment; the disloca­
tion can be easily reduced.
2 Dislocation is already present at the mo­
ment of birth as a feature of general, inher­
ited connective tissue laxity (dominant
form). Besides the luxation, other signs of
general, inherited connective tissue laxity
are also detected (knee hyperextension
and lax wrist). It reacts well to conserva­
tive treatment; the dislocation is easily re­
duced.
28. H i p d isorders � 333
3 Symptomatic hip dislocation is a symptom
of identified monogenic (dominant or re­
cessive) hereditary abnormality (e.g.
Ehler-Danlos syndrome, Marfan 's syn­
drome, Morquio 's syndrome, congenital
multiplex arthrogryposis, etc.). It reacts
less to conservative treatment, though this
may be attempted. It usually requires sur­
gical intervention.
4 Secondary hip luxation is due to primary
neurological or muscular disorders. The
dislocation is not present at birth, it ap­
pears only during childhood (e.g. infantile
cerebral palsy, poliomyelitis, etc.). It
barely reacts to conservative treatment,
and usually requires surgical intervention.
5 Teratological hip luxation is induced by a
teratogenic factor in the first third of
intrauterine life, it is not inherited. It is of­
ten seen as a part of the multiple develop­
mental abnormality of the limb. The series
of developmental abnormalities is local­
ized only to the involved limb, in contrast
with symptomatic hip dislocation, where it
is part of a general developmental abnor­
mality involving the entire body. It does
not react to conservative treatment, and
the outcome of surgical intervention is
also generally poor.
Pathology of dislocated hip j oint
� Bony elements
- Acetabulum hypoplasia - primary path­
ologic factor.
- Increased antetorsion of the proximal
femur (this is a consequence of
loosening of the active anterior
acetabular wall (iliopsoas muscle).
- Coxa valga (this is a consequence of the
missing pressure of the body weight
transferred to the head and neck by the
acetabulum) .
� Soft-tissue components
The soft tissue at the bottom of the
acetabulum proliferates, the limb is inverted,
the iliopsoas and adductor muscles are tense,
and the gluteus medius and minimus muscles
are insufficient.
334 � 2 8 . H i p d i so r d e r s
Clinical symptoms. The clinical symp­
toms are classified as follows :
� Signs arousing suspicion
� Signs indicating probability
� Hip instability
� Definite signs
� Late signs
� X- ray and u ltrasonographic signs (imaging
signs)
� Signs arousing suspicion
Signs arousing suspicion feature among
the history data obtained from the parents : the
occurrence of hip dislocation or dysplasia, a
pathological pregnancy, an abnormal delivery
or the intrauterine posture (breech presenta­
tion) and other data relating to the behavior of
the newborn: an asymmetric movement of a
lower limb, or any other factors worth men­
tioning.
� Signs indicating probability
- Fold asymmetry. This is considered only
if it is consequently present together
with other signs indicating probability.
The fold asymmetry is presumed to be
consequent when the thigh folds are
deeper and proximal, and perhaps more
numerous . In girls, the most proximal
fold may partially conceal the labium
'.J
Fig. 28.7.
In cases of hip dysplasia and luxation the thigh and
gluteal folds are asymmetric. I n the involved side,
there are more and deeper folds.
maius. When the baby is prone, the
gluteal folds are also asymmetric and
positioned proximally on the involved
side (Fig. 28.7.).
- Adduction contracture. The hip ab­
duction of newborns is between 70° and
90° and symmetrical. An abduction of
less than 70° bilaterally or asymmetrical
abduction is considered a sign indicating
probability.
- An increased greater trochanter mass.
In cases of hip luxation or subluxation,
the greater trochanter is displaced
proximally and laterally, and on
palpation it is felt to be an enlarged mass
(Fig. 28.8.).
- External rotation of the limb. In hip
dysplasia and even more in luxation, the
limb is externally rotated. This has no
specific diagnostic value, but it may be
Impressive.
Fig. 28.8.
The baby is assessed with bent hips and knees. The
thigh is shorter on the luxated side, while the
greater trochanter is displaced proximally and later­
ally, and on palpation it gives the feeling of an in­
creased mass, in the middle of the buttock, there is
a fold.

- Limb shortening (Bettmann 's sign).
Limb shortening is associated with hip
dislocation, since the femoral head is
displaced from the acetabulum
proximally, resulting in limb shortening
(Fig. 28.8.).
- Axial deviation. In Lorenz 's abduction,
the femoral axis points above the
acetabulum instead of its center.
- Atrophy, and flattening of the gluteal
muscles. With the hip flexed to 90° the
buttocks are asymmetrical and on one
side, a dent is visible in the middle of the
buttock (Fig. 28.8.).
• Hip instability (Barlow' s sign)
The essence of this sign is that the hip is
not displaced, but it is dislocatable, luxatable
from the acetabulum. The sign is caused by
two factors : acetabulum hypoplasia and tran­
sitional hormonal laxity. Consequently, it is a
false-positive sign in 90% of the cases in the
first 3 weeks. The mode of assessment is the
following: The hips of the supine baby are
flexed to 90° with knees fully flexed. The hips
are t4en mildly adducted and mild pressure is
exerted backward with the examiner' s thumb.
In positive cases, a click is palpable in the hip,
when the femoral head leaves the acetabulum
Fig. 28.9.
Hip instability (Barlow's sign). The pressure of the
examiner's thumb dislocates the hip.
28. Hip d isorders � 335
(positive Barlow 's sign, Fig. 28.9.) . A s soon
as the force is released, the head slips back
into the acetabulum.
• Definite signs (Ortolani 's sign).
This is the only definite sign of hip dislo­
cation; it can also be evaluated as a reduction
sign. The assessment is performed as follows :
The hips of the supine baby are flexed to 90°
and abducted. During abduction a click is ex­
perienced, signaling that the femoral head is
reduced (Fig. 28.1 0.). In cases involving hips
that undergo reduction with difficulty, this
maneuver is to be supplemented with axial
traction of the femur to facilitate reduction
(Lorenz 's reposition).
• Late signs
Late signs are detected after the child has
started to walk. Fortunately these are seen
very rarely nowadays.
- The Trendelenburg sign. This is de­
tected in all other disorders besides hip
dislocation, where a gluteus medius and
mlll1mus muscle insufficiency 1S
present. The standing patient is
requested to stand on only one leg, and
to lift the other leg up while bending the
hip and the knee. In normal individuals,
Fig. 28.10.
Reposition sign (Ortolani's sign): during a bd uction, a
click is felt and the femoral head is reduced
336 � 28. Hip d isorders

the gluteal muscles o n the supporting walking, and also during assessment in a
side (hip abductors) hold the un­ prone position.
supported side of the pelvis in a - Increased lumbar lordosis. Mainly in
horizontal plane. In case of an in­ high bilateral dislocations, the lack of
sufficiency of the gluteal muscles, the proper hip support results in an increase
pelvis on the unsupported side sinks in the pelvic slope, and this is com­
below the horizontal plane and the pensated by increased lumbar lordosis.
patient tilts with the trunk toward the
et
supported side, because this is the only a
way he/she is able to maintain balance
(Fig. 28. 1 1 .). In unilateral cases, the
trunk tilts toward the supported side
with every step during walking, in
bilateral cases, the trunk tilts in both
directions (duck gait).
- Telescope sign. In cases of total dis­
location, the protruding trochanter (and
the femoral head) moves proximally and
distally in the axis of the femur during

Fig. 28.12.
Ultrasonographic assessment of the hip. The draw­
Fig. 28. 11. ing: the angle alpha is not less than 60° and beta is
Left side positive Trendelenburg sign; the unsup­ not larger than 55° for in normal hips. The image
ported, right side of the pelvis sinks u nder the hori­ shows a phase I Il.a dysplastic hip: a: bony acetabular
zontal plane roof; b: cartilage acetabular roof, c: baseline.

• U1trasonographic and X-ray signs
(imaging signs)
Ultrasonographic signs. Under the age of 4
months X-ray examinations are not per­
formed on the hips, because the ossifying cen­
ter is undetectable. Instead of the harmful
X-ray radiation, a modem and harmless ultra­
sonographic assessment is carried out. This
reveals dysplasia at an early stage and the de­
velopment of the hip joint is easily followed
up (Fig. 28.1 2.). The examination may be re­
peated at any time. Ultrasonography is recom­
mended in case of any existing clinical sign,
or when risk factors are recorded (familial oc­
currence, or breech presentation) . This
noninvasive scan helps in the evaluation of
the bony and cartilaginous elements of the
acetabulum as primary factors.
X-ray signs. Hip X-rays may be per­
formed above the age of 4 months with the
following indications :
- when ultrasonography and physical
assessment lead to a questionable
conclusion,
- in suspected dysplasia or luxation,
- in cases of familial occurrence.
The presence of the ossifying center of the
femoral head contributes a great deal to the
evaluation of the X-ray film. Before its mani­
festation, various ancillary lines facilitate ori­
entation.
Fig. 28.13.
4-month-old baby. Hip X-ray: the acetabular roof is
steep on the left side (dysplasia).
28. Hip disorders � 337
- Acetabular roof angle (Hilgenreiner 's
angle) : this is normally symmetrical and
less than 3 0°, in cases of dysplasia the
value increases (Fig. 28.13.). Assess­
ment of the acetabular roof angle : a
horizontal line is drawn through the
identical points of the Y -shaped car­
tilage, representing the horizontal plane,
and the angle is measured between this
and the line following the acetabular
roof.
- Kopits 's rectangle. This is outlined by
the lines drawn between the two
end-points of the acetabular roof and the
two end-points of the femoral neck
metaphysis. In normal cases, this is a
regular square, and, if the ossifying cen­
ter is already apparent, it is positioned in
the central area (Fig. 28.14.). In cases of
dysplasia or luxation, the square is
irregular, and the ossifying center is
excentric and lateralized.
- Menard-Shanton 's line. The cranial arch
of the foramen obturatorium is normally
the continuation of the lower arch of the
femoral neck. In cases of subluxation or
dislocation, this arch is interrupted, and
the lower arch of the femoral neck is
moved proximally (Fig. 28.14) .
- Ombredanne 's line. This is a per­
pendicular line drawn from the lateral
edge of the acetabulum distally to the
horizontal line connecting the Y -line,
Fig. 28. 14.
Kopits's quadrangle (K), Menard-Shanton's line (MS)
and Ombredanne's line (0).
338 � 28. H i p disorders
described above. These two lines divide
the hip joint into 4 segments. If the hip is
normal, the ossitying center is located in
the inner-lower quadrant; in cases of
subluxation or dislocation it is located
laterally (Fig. 28. 1 4.).
- Hilgenreiner 's H-distance. This is the
distance between the medial spine of the
femoral neck and the sciatic bone, which
is normally parallel with the Y-line and
it is at most 5 mm. Before the age of 6
months, a larger distance may mean
lateralization of the femoral head (Fig.
28.1 5.).
Prevention of hip dislocation . The es­
sence of prevention is to ensure the relaxed -
resting position for the newborn following
birth (Fig. 28.6.). It is not coincidental that hip
dislocation is rare in Far-Eastern countries
and in Africa, where children are carried on
their mother' s back with their legs spread. In
contrast, in countries, where babies are
wrapped in nappies or swaddled with tightly
adducted lower limbs (Eskimos, Canadian In­
dian tribes, in some places in Ecuador and in
Central and Eastern Europe), dislocation is
very widespread.
Fig. 28.15.
Various ancillary lines and ang les for evaluating hip
dysplasia or dislocation on the X- ray film. (Y: Y- line;
vsz: acetabular roof angle; d l,d2: socket -
metaphysis d istance; h: Y-line - metaphysis dis­
tance). On the left, the situation is normal; on the
right, dislocation is seen. Vsz and d values are larger,
and distance h is lower
Prevention and recognition are closely re­
lated. Accordingly, three compulsory infant
screenings are performed in Hungary: The
first is carried out 3 -4 days after birth, usually
in neonatal wards, and is easy to organize. The
next is due at the age of 3 -4 weeks, and the
third at the age of 3 -4 months. The hips must
be checked on all occasions when the child is
due to participate at other regular pediatric
check-ups or inoculation.
Conservative treatment. The methods of
conservative treatment depend on the time
and severity of the diagnosis.
When dysplasia is noticed at the first or
second screening, merely exercises and
a
�(
�J b
�(
�J
Fig. 28.16.
c
a: Right side: normal; left hip: acetabulum hypoplasia
is seen in an extended position. The femoral head is
pressed against the acetabular roof; no improve­
ment is expected.
b: I n the abducted - flexed position, the femoral
head is pressed centrally into the socket, ind ucing
normalization of the acetabulum.
c: Recovered state.
 2 8 . H i p d i s o r d e rs � 339

spreading nappies are recommended (Fig.

28. 1 6. a, c). The method advocated for
spreading nappies is the use of "Rugi pants"
(Fig. 28.1 7.), which ensures continuous ab­
duction with the elastic spongious lining. In
cases of dysplasia recognized at the third
screening and confirmed by ultrasonography
or X-ray examination, the application of
Pavlik ' s harness is advised (Fig. 28.1 8.).
Pavlik 's harness is a functional treatment mo­
dality popular worldwide. The essence of this
functional treatment is that it limits only the
extension of the hip; all the other movements
can be freely performed. Flexion and abduc­
tion centralize the femoral head and enhance
the development of the acetabular roof. The
use of Pavlik 's harness is possible only until
the infant sits up (at the age of 6-8 months),
Fig. 28.18.
since the main effect is eliminated in a sitting
Pavlik's harness. 90° of flexion is secu red in the hip
position.
joint
Instability. As described in the above, at
the time of the first screening, instability is a
pseudo-positive symptom induced by transi­
tional hormonal laxity. At the next screening,
it may be decided whether real dysplasia is
present: if the instability has ceased, the hip is
classified as normal. If the instability persists,
severe acetabulum hypoplasia is in the back­
ground and Pavlik 's harness should be con­
sidered. The severity of the acetabulum hypo­
plasia may be defined by an ultrasonographic
scan. In cases of instability persisting up to the
age of 4 months, Pavlik' s harness is also indi­
cated.
Ortolani 's positive hip (luxation) . If this is
detected at the first screening, abduction exer­
cises and spreading treatment are advised for
a short period, knowing that Pavlik 's harness
is needed (Fig. 28.1 8.). Pavlik 's harness is not
to be applied before the I O- 1 4th day after
birth, since it may harm the newborn and the
skin adaptation is still underway. Pavlik' s har­
ness can be used on an outpatient basis. In the
maj ority of cases (95%), it results in total re­
covery.
Below the age of 6 months, other modes of
conservative treatment may be attempted if
Fig. 28.17. Pavlik 's harness fails (5%). One ofthese is the
Sprawling abduction nappies. "overhead extension" (Fig. 28.1 9.). In cases
340 � 28. H i p d isorders
a ------
Fig. 28.19.
Overhead extension, first vertically (a), and later in the
a bd ucted position (b).
of irreducible or barely reducible hip disloca­
tions, distal traction is applied via cords at­
tached to the legs by adhesive tapes, which
may loosen the hip joints. With gradually in­
creased abduction, spontaneous reduction
may occur. In cases of instability, when the
femoral head leaves the acetabulum at 1 0-20°
of adduction, a more rigid fixation device, the
abduction splint (von Rosen ' s splint) is tem­
porarily applied (Fig. 28.20.).
Fig. 28.20.
Abd uction splint.
Conservative treatment is recommended
until the age of 6 months. Above this age, sur­
gical intervention is advised.
Surgical management. In surgical reduc­
tion, following exposure of the hip joint cap­
sule, the soft tissue that fills the joint is re­
moved, together with the inverted limbus. The
iliopsoas muscle is separated from the lesser
trochanter, and fixed to the capsule at the an­
terior part of the femoral neck. After removal
of the obsolete parts of the capsule, reduction
of the femoral head is easily achieved and, by
narrowing the capsule, the reduced femoral
head can be stabilized. Postoperatively, the
hip must be functionally immobilized in the
abduction - flexion position with a splint or in
Pavlik 's harness.
After the age of 2 years, besides the cor­
rection of soft-tissue components by the open
reduction procedure described above, the
bony components must also be corrected. In
cases of mild acetabular hypoplasia, the cor­
rection of the proximal femur can be satisfac-
 28. Hip disorders � 34 1

Fig. 28.21.
A 2-year-old girl with a developmental dislocation of the left hip. Open reduction, varus and derotation
osteotomy. The steep acetabular roof is normalized, and the head is central. Normal hip at the ages of 6, 8
and 31 years.

tory: varus and derotation osteotomy ensures Correction of the acetabulum can be sub­
the development of the acetabular roof after divided into complete and incomplete pelvic
the centralization of the femoral head (Fig. osteotomies (pericapsular pelvic osteotomy
28.2 1 . a, b) . and acetabuloplasty).

Fig. 28.22.
Pelvic osteotomies for correction of the steep acetabulum: 1: Salter's pelvic osteotomy, 2: Pemberton's in­
complete pericapsular pelvic osteotomy, 3: Chiari's pelvic osteotomy, 4: triple osteotomy.
342 � 28. H i p d isorders
The most widespread and popular tech­
niques are as follows :
- Chiari 's pelvic osteotomy is a complete
pelvic osteotomy over the acetabulum,
the distal part being displaced medially
(Fig. 28.22.). The center of rotation of
this motion is the symphysis. The
disadvantage is that the femoral head is
covered by fibrous cartilage instead of
hyaline. It is nowadays used only in
exceptional cases, when no 'other
procedures are reasonable.
- Salter 's pelvic osteotomy is also a
complete pelvic osteotomy over the
acetabulum, but the distal part is
displaced laterally, providing a hyaline
cartilage cover for the femoral head
(Fig. 28.22.). The center of rotation of
this motion is again the symphysis. The
correction effect is limited, depending
on the mobility of the symphysis, and it
can therefore be used under the age of 6.
- Pemberton 's incomplete pelvic oste­
otomy, acetabuloplasty, is a rounded
and arched pelvic osteotomy starting
above the upper edge of the acetabulum
and extending to the Y -shaped cartilage
in the depth of the acetabulum (Fig.
28.22.). The steep acetabulum is folded
Fig. 28.23.
A 5-year-old girl with subluxation; one-stage osteotomy which corrects the bony and soft tissue elements in
a single session.
down over the femoral head, and the
correction achieved is ensured by a bone
wedge inserted into the gap. The center
of rotation of this motion is the
Y -shaped cartilage. It may be ideal until
the closure of Y -shaped cartilage.
The surgical intervention on the hip joint
which corrects the bony and soft tissue ele­
ments in a single session is called one-stage
osteotomy (Fig. 28.23.). The outcome is ex­
cellent if it is done in time and with good tech­
nical conditions. Later, the child may take part
in sports activities and gymnastic exercises.
28. 1.3. Osteochondritis capitis
femoris juvenilis (Perthes' disease,
Legg-Calve-Perthes' disease)
This is necrosis of the proximal femur epiphy­
sis in childhood, leading to a deformity of the
femoral head.
Incidence. It occurs between the ages. of 3
and 1 3 years; it is the second most common
hip illness with an incidence of between
0, 1 - 1 %. It is 3 times more common in boys

than in girls. It is usually unilateral, but in
1 5%, it is a bilateral condition.
Etiology. The definite cause of the disease
is unknown, there is however both direct and
indirect evidence pointing to a disturbance of
the blood supply of the femur proximal epiph­
ysis. The condition is unquestionably related
to occlusion of the lateral epiphyseal artery,
which supplies the epiphysis and it is apt to
occlude in response to a pressure increase in
the hip joint, on the short and vulnerable seg­
ment of the artery, where it runs on its
intra-articular and extra-osseal path. It has
been proven, that in 5% of the cases, this con­
dition is preceded by transitory hip arthritis. It
occurs in the temperate zone, in both the
northern and southern hemisphere. It is not
observed in cold and hot zones where the cli­
mate is usually more balanced. The influenza
common in spring and autumn may be the
etiologic factor. It is also characteristic, that
the incidence of this condition in childhood
corresponds to that of transitoric coxitis.
Pathology. In consequence of primary or
reactive intra-articular irritation, the synovial
membrane swells and becomes hyperemic. In
the early stages, the histology of the epiphysis
ossifying center reveals an enchondral ossifi­
cation disturbance besides necrosis. The
osteocytes expire and the necrotic foci are
gradually surrounded by scar tissue, while os­
teoclasts form from monocytic elements . The
necrotic bone substance is gradually degraded
and the osteoblasts produce new bone (keep­
ing substitution). In the regenerative stage, the
bone production is increased. In the stage in
which the necrotic areas undergo degradation,
the bone is not sufficiently solid enough, and
the granulation tissue gradually occupying the
necrotic bone does not have appropriate me­
chanical rigidity, and is therefore not suitable
for weight-bearing. Accordingly, the femoral
head becomes flattened and mush­
room-shaped, and may protrude laterally.
History. A typical feature is an intermit­
tent, strange gait or limp lasting for weeks or
28. Hip disorders � 343
months. I n a number of cases however, the
child has no complaints. In early stages, the
strange gait or limp temporarily ceases after
resting. The child locates the pain in the thigh
or in the knee instead of the hip (due to the
obturator nerve). As a general rule therefore,
in case of knee pain in childhood, the hip must
be thoroughly assessed. Occasionally, the
child presents acute hip pain, and is unable to
bear weight on the involved limb. In these
cases, primary or secondary synovitis pre­
dominates.
Clinical symptoms. The first obj ective
sign is a decrease in the internal rotation of the
involved hip compared to the contralateral
side. In advanced cases, the limitation of the
internal rotation becomes more severe, and an
external rotation contracture may develop to­
gether with the limited extension. This is a se­
lective limitation of movement, since the pro­
cess always involves only one plane of the
three axial planes of movement of the hip
j oint. The child limps protectively; atrophy of
the thigh muscles is also observed. In ne­
glected cases, the flattening of the femoral
head and the shortening of the metaphysis
cause moderate limb shortening.
Imaging diagnostics. When Perthes ' dis­
ease is suspected, bilateral anteroposterior
and Lauenstein X-ray films are to be taken.
Lauenstein ' s position is the flexed, abducted
and externally rotated hip. On the basis of
X-ray picture, 4 stages are defined:
Early stage. The early X-ray image does
not show bony changes; medially, the j oint
space is wider than on the other side. The
cause is the synovitis and edema of the
pulvinar acetabuli.
2 Sclerotic stage. The structure of the bony
center of the proximal epiphysis is cloudy,
and the density is increased; it becomes
sclerotic, which is the radiological sign of
necrosis (Fig. 28.24.). The bone center is
moderately flattened.
3 Fragmentation stage. The previously ho­
mogenous bone center is even more flat-
344 � 28. H i p d isorders
Fig. 28.24.
Perthes' disease: sclerotic stage.
Fig. 28.25.
Perthes' disease: fragmentation stage.
tened and fragmented (Fig. 28.25.).
Radiolucent areas appear in the bone cen­
ter as the granulation tissue disintegrates
the necrotic bone. The entire epiphysis
may flatten, it widens and protrudes later­
ally, and the widening of metaphysis in­
creases (Fig. 28.26.).
4 Final stage. The femoral head is flattened
to some extent, and its height is less than
the contralateral height. The structure of
the newly built bone center is normalized.
In untreated or severe cases an enlarged,
Fig. 28.26.
Perthes' disease: epiphysis flattened and widened.
Fig. 28.27.
Perthes' disease: end stage.
Fig. 28.28.
Bone scan: no perfusion of the involved epiphysis
flat, protruding femoral head is formed
(coxa magna, Fig. 28.27.).
In cases of clinical suspicion, when the
X-ray finding is negative, a bone scan may
confirm the diagnosis, showing no or only
limited perfusion of the involved epiphysis
(Fig. 28.28.).
 28. H i p d isorders � 345

Laboratory tests are negative.

Treatment. The course of Perthes ' dis­

ease lasts 3-5 years; the treatment is influ­
enced to a large extent by the known risk fac­
tors listed below.

I . Catterall 's stages (Fig. 28.29.) .

� Stage I: 25% of the epiphysis is involved.

� Stage 11: 50% of the epiphysis is involved.
� Stage I l l : 75% of the epiphysis is involved.
� IV. stage: The entire epiphysis is necrotized.

The more extensive the necrosis, the

worse the prognosis.
2. Lateral pillar sign. Even if the necrosis is
extensive, in cases when the small lateral
segment of the epiphysis remains un­
harmed and its structure is intact, the prog­
nosis is expected to be good (Fig. 28.30.). Fig. 28.30.
The small lateral pillar of the epiphysis remains un­
harmed and its structure is intact.

3
Fig. 28.29.
CatteraWs stages of Perthes's disease according to the extent of the affliction.
346 � 28. H i p d isorders

3 . The age. This is the most important factor

regarding therapy. Comparative clinical
studies of treated and untreated cases have
proved that below the age of 6 years, a
good outcome is to be expected, almost re­
gardless of the method applied. This is
probably due to the good regenerative ca­
pability of the young organism. After the
age of9- l 0, the outcome is poor in the ma­
j ority of cases, regardless of the therapy.
4 . Metaphyseal involvement. If the process
involves the metaphysis, the prognosis is
poor (Fig. 28.3 1 .).

The current treatment strategy relates to

two major points: B elow the age of 6 years,
the outcome is expected to be favorable even
without treatment. Hence active therapy
should only be considered in cases of
Catterall 's stage IV of complete necrosis. Af-
ter the age of 1 0 years, the result is poor with Fig. 28.31.
or without treatment. Above this age, there- Metaphysea l necrosis.
fore, only those interventions are indicated
that ensure centralization of the femoral head
proven by functional X-ray examination; oth-
erwise only observation and palliative treat-
ment should be provided.
The centralizing ( containment) treatment
has become predominant in both conservative
and surgical methodology.

Methods of centralizing treatment

Conservative techniques. The basis of
the treatment is that abduction and internal ro­
tation of the hip ensure the central location of
the femoral head in the acetabulum. This posi­
tion decreases the load by distributing it
evenly on the surface of the biologically softer
femoral head and enhances its new
modelation to the shape of the acetabulum (re­
ciprocal remodelation) .
To achieve this obj ective (in either uni-, or
bilateral cases), various calipers have been de­
vised to ensure abduction and internal rotation
(Fig. 28.32.). Walking in these calipers and
their utilization for 3 - 5 years involves a sub­
stantial restriction of activity for the child.
Fig. 28.32.
An alternative approach is the use of
Calipers used in Perthes' disease to ensure abduc­
crutches or bed rest, but in view of the 3-5 tion and interna l rotation.

years progress, this appears impracticable and
maybe even impossible to implement.
Operative therapy. Varus derotation
osteotomy of the proximal femur. This
method is popular in Hungary and in all of Eu­
rope, since 8 weeks after the surgically
achieved centralization and following the
healing of the osteotomy full, weight-bearing
and activity may commence. The osteotomy
close to the pathology increases the decompo­
sition and rebuilding of the necrotized femo­
ral head and virtually halves the usual
3-5 -year period of progression (Fig. 28.33.).
� Pelvic osteotomies
A full cover of the femoral head and a de­
crease of the load on the surface can also be
achieved by pelvic osteotomy, which is an­
other mode of centralization. Mainly Salter ' s
osteotomy has become widespread. I t has a
biomechanical disadvantage since it increases
the pressure on the femoral head. Further­
more, the author recommends that it is to be
Fig. 28.33.
Perthes's disease on the right side, following varus
derotation osteotomy: full recovery. The head is
round.
28. Hip d isorders � 347
performed before the age of6. After the age o f
1 0, Chiari ' s complete pelvic osteotomy may
be performed mainly as a palliative interven­
tion (especially when a large lateral femoral
head protrusion is detected, Fig. 28.22.).
Perthes ' disease is one of the causes of hip
osteoarthritis in adults . Therefore, after the
healing of Perthes ' disease, school gymnastic
exercises and sports are advised as follows :
1 . Cases that have healed without deformity:
all activities are allowed.
2. A slightly flattened, but round, central
femoral head (physiologic incongruence) :
all activities (including sports) are al­
lowed.
3. A considerably flattened, laterally pro­
truding deformed femoral head (coxa
magna) : moderate gymnastic exercises,
swimming and cycling are allowed.
28. 1.4. Slipped capital femoral
epiphysis (SCFE, epiphyseolysis
capitis femoris juvenilis, coxa vara
adolescent)
The origin of this entity is the slipping of the
capital femoral epiphysis backward and medi­
ally on the femoral neck. The process is often
bilateral.
The types:
- Epiphyseolysis lenta: This is a gradual
slipping, with intermittent knee, thigh
and hip pain; the limb is in a position
similar to that when the neck of the
femur is fractured: mild shortening and
external rotation are seen with a
restricted range of internal rotation.
- Acute epiphyseolysis: The slip happens
suddenly, following a fall or a faulty
movement. It is accompanied by acute
hip and knee pain. The limb is in a
position similar to that when the neck of
the femur is fractured. The limb is
shortened and externally rotated, with a
severely restricted range of motion.
348 � 28. Hip d isorders
I ncidence. This is the third most common
hip condition after hip dysplasia and Perthes '
disease. I t occurs i n the age interval 1 0- 1 5
years. It i s twice as common in boys, as in
girls. It is often bilateral, and therefore close
observation of the other hip is crucial. The
lenta form of epiphyseolysis predominates
(95%) .
Etiopathology. It is currently believed
that the background of this condition involves
the development of a latent hormonal dys­
function between the growth hormones (that
control the ossification) and the sex hormones
in puberty. In response to the effects of the
growth hormones, the proliferation of carti­
laginous elements in the growth plates in­
creases, and the growth plates widen. At the
Fig. 28.34.
Shearing forces acting on the growth plate (ny),
since the load (R) is not perpendicular to the growth
plate.
end of growth, the secretion of the growth
hormone gradually diminishes. The sex hor­
mones lessen the proliferation of the cartilagi­
nous elements, and the growth plates become
thinner and ultimately disappear.
The diverse secretion activity undergoes a
crossover in puberty. If the secretion of sex
hormones starts later, the growth plates persist
and can not resist the biomechanical load pro­
duced by the considerable body weight and
height gain in puberty, and may be displaced.
This is common in the hip joint, because this
is the only epiphysis in the body that is not
perpendicular to the axis of the body weight,
but is inclined obliquely and medially. It is
therefore subj ected not only to pressing
forces, but also to substantial shearing forces
(Fig. 28.34.).
Fig. 28.35.
Dystrophia adiposogenital constitution
of a 14-year-old boy with right-sided
epiphyseolysis.

The condition is common in two constitu­
tions. One exhibits all the features of hypo­
gonadism: fat, a lack of secondary sexual
signs, an a eunuchoid constitution (relative
hypogonadism; Fig. 28.35.). The other type is
a tall, thin constitution with long powerful
arms (relative growth hormone overproduc­
tion).
History. In cases of epiphyseolysis lenta,
the child presents chronic complaints. Usually
knee and thigh pain, but occasionally hip pain
and fatigue are mentioned and the child
avoids physical activity. The gait is peculiar,
and an occasional limp is observed.
In acute cases a stabbing hip, thigh or knee
pain occurs following a fall or faulty move­
ment and the child is unable or hardly able to
stand.
Clinical symptoms. Walking with a slight
limp is usually detected with an externally ro­
tated lower limb. One of the constitutions de­
scribed above is observed (eunuchoid relative
hypogonadism, or a tall, thin constitution) .
When the hip is assessed in a supine posi­
tion, the internal rotation is observed to be re­
stricted, and in most of the cases an external
rotation contracture is present. The abduction
and extension may also lessen, especially in
major slips. Drehmann ' s sign is distinctive:
on passive flexing of the hip, constrained ab­
duction and external rotation occur. This sign
is a consequence of the dorsal and medial dis­
placement of the femoral head. Another com­
mon feature is the crossing sign: the child is
asked to kneel, and the legs will cross each
other.
In acute cases, besides the intense pain the
symptoms are similar to those detected when
the neck of the femur is fractured: the limb is
shortened and externalIy rotated, and any at­
tempted movement elicits severe pain.
X-ray signs. Similarly as in other hip con­
ditions, bilateral anteroposterior and
Lauenstein films are to be taken. On the
Lauenstein views, slippage of the epiphysis is
noticeable earlier, since the backward slip is
28. H i p d i s o r d e rs � 349
then detected on this view and this is always
larger than the medial displacement. In the
anteroposterior views, the very early signs
(preceding the significant slipping) is the wid­
ening and unevenness of the growth plate
(Fig. 28.36.) . A moderate slip is indicated if
the decrease in height of the bony center of the
epiphysis is mild, compared to the other side.
Another valuable sign is that when the lateral
contour of the femoral neck is extended, nor­
mally, this line cuts few mm-s off the edge of
the epiphysis (Fig. 28-36.). If the line is pe­
ripheral to the epiphysis, this may be a radio­
logical evidence of a mild slip.
If a line drawn along the lower contour of
the femoral neck on a Lauenstein film is prox­
imalIy elongated, and this line marks off a
small segment from the posterior edge of the
epiphysis, this is evidence of a mild slip (Fig.
28.37.).
The extent of the slip can be defined by an
exact angle on the Lauenstein films : the angle
between the axis of the femoral neck, and the
line drawn through the edges of the epiphysis.
Normally, its value is 90° (Fig. 28.37., and
28.38.).
Treatment. Both forms of epiphyseolysis
require surgical treatment. Until the operation
is performed, immediate bed rest is ordered so
as to prevent progression, but a further slip
may still occur.
Fig. 28.36.
A 12-year-old boy with left sided chronic
epiphyseolysis: anteroposterior. X-ray.
350 � 28. Hip d isorders

Fig. 28.37.
Same boy: Lauenstein X-ray.

Fig. 28.39.
Epiphyseolysis with moderate slip: " in situ "
epiphyseodesis with 2 spongiosa screws.

In cases involving a slip of less than 30° in

epiphyseolysis lenta, surgical fixation in situ
is indicated, ifthis is technically possible. One
or more K-wires, cancellous screws or
cannulated screws (Fig. 28.39.) are intro­
duced through the neck into the epiphysis.
Because of the stabilization of the epiphy­
sis, the pain and the contractures rapidly sub­
side postoperatively. The child may get out of
bed on crutches the day after the operation, or
in the event of pain, a few days later, and al­
lowed to bear weight and go to school after 3
a weeks.
When the slip is more than 3 0°, wedge
b
osteotomy of the neck after Dunn can be car­
ried out, and the reduction is supplemented
with corrective shortening of the femoral
neck.
In cases of acute si ip, gentle open or closed
reduction and fixation is indicated in the first
week. The attempt at reduction however, may
result in a blood supply deficit and necrosis of
the head.

28. 1.S. Coxa vara infantum

Fig. 28.38.
A 14-year-old girl with acute epiphyseolysis. antero­
posterior (a) and Lauenstein radiographs: total slip
(b).
(congenital)
This is a congenital ossifying malfunction of
the femoral neck: the collodiaphyseal angl
 28. Hip d isorders � 351

e gradually decreases and causes limb short­

ening. The condition is al ready present at
birth, but is usually recognized at the age of
3-4 years. It involves both genders and is usu­
ally bilateral. It is a rare congenital condition.

Etiopathology. The etiopathology is un­

known. It is not inherited. The pathological
process is progressive; the collodiaphyseal
angle gradually decreases during childhood,
often to under 90°. The growth plate becomes
almost vertical, and may even create a state
corresponding to acute epiphyseolysis be­
cause ofbiomechanical factors, since the ver­
tical growth plate is subj ected almost exclu­
sively to shearing forces. The limb is gradu­
ally shortened, and the maj or trochanter
moves cranially. The origin and attachment of
the gluteus medius and minimus muscles
come closer to each other, and they become
insufficient, Trendelenburg ' s gait develops.
Clinical symptoms. The condition may
already be discovered in infants by a skilled
orthopedist, or at least the suspicion arises.
Fold asymmetry and limited abduction are
typical. The child is usually referred to a spe­
cialist at about 3 -4 years of age, with a pain­
less peculiar gait and limp. A higher and
thicker trochanter mass, limb shortening and
Trendelenburg ' s gait are observed. The hip
abduction is slightly restricted.
X-ray signs. Before the appearance of the
ossifying center of the femoral head epiphy­
sis, early films merely indicate the irregularity
of the proximal metaphysis. Later, the de­
creased collodiaphyseal angle is unequivo­
cally determined, and it is less than the aver­
age for the age group . The direction of the
growth plate changes, it becomes close to ver­
tical. A characteristic X-ray sign is the pres­
ence of a triangle-shaped component between
the distal-medial part of the epiphysis and the
medial part of the neck (Fig. 28.40.). The
growth plate may close earlier, around the age
of 1 0- 1 2 years, because of the ossifying error,
Fig. 28.40.
Bilateral coxa vara infa ntum.
or permanent pseudo-arthrosis may often de­
velop in the femoral neck.
Treatment. This deformity can only be
treated surgically. In progressive cases, val­
gus osteotomy is to be carried out in the early
years. It must be realized however, that a
gradual recurrence may follow because of the
progression of the condition. In these cases,
valgus osteotomy must be repeated at a later
age.
28. 1.6. Sym ptomatic coxa vara
A number of illnesses and general conditions,
such as epiphyseolysis capitis femoris, fibrous
dysplasia, Paget's disease, etc., may ind uce
secondary varus hip.
Clinical symptoms. The clinical symp­
toms are similar to those described on coxa
vara infantum (congenital) : limited hip ab­
duction, limb shortening and Trendelenburg' s
gait.
Treatment. If the deformity is part of a
systemic disease, the latter should be treated,
which may or may not cure the ossifying dis­
turbance.
352 � 28. H i p d isorders
28. 1.7. Growth disturbances
of the proximal fem u r
Various extents o f hypoplasia or aplasia o f the
proximal femur may occur for u n known rea­
sons. A primary teratogenic noxa is presum­
ably in the background during embryo­
genesis. It is not inherited.
The main point of this disorder is that ossi­
fication of the proximal femur is delayed. Os­
sification of the epiphysis is late and hypo­
plastic, and occasionally involves more than
one ossifying center; moreover, the proximal
ossification of the diaphysis is retarded (Fig.
28.4 1 .) . The hypoplastic or aplastic proximal
femur is often dislocated.
Treatment. In infants, no treatment is
needed but observation is recommended. Af-
Fig. 28.41.
Right congenital femur hypoplasia.
ter the start of walking, conservative treat­
ment is advised, depending on the extent of
limb shortening. If the shortening amounts to
3 -5 cm, length equalization using surgical
shoes is necessary. If the shortening is more
extensive, equalizing calipers are prescribed.
28. 1.8. Transitory arthritis coxae
(transitory hip joint inflammation)
This is an acute, painful hip arthritis, which re­
solves by itself within a few days. It occurs
most frequently between the ages of 2 and
14 years, affecting both genders equally.
Etiopathology. In the majority of the
cases, the arthritis is preceded within 1 -3
weeks by influenza. It may occur after any
other inflammation or trauma. As an immune
response to an inflammation, reactive syno­
vitis may occur in any joint (not purulent).
This is usually a condition in the temperate
zone (similarly to Perthes ' s disease), and the
incidence of the two disorders is also very
similar.
Clinical symptoms. It usually appears as
an acute condition, the child is unable to bear
weight on the upper limb in the morning or
daytime, indicating hip, thigh or knee pain.
The child protects the hip, and this indicates
the hip involvement, despite the fact that the
pain seems to be distal . The condition often
occurs in a less acute form: the parents com­
plain that the child limps and avoids loading
the involved limb, but the pain is not domi­
nant. Examination reveals a selective restric­
tion of movement is similar to that in Perthes'
disease. The most outstanding sign is de­
creased internal rotation, and occasionally an
external rotation contracture; the abduction
(or adduction) and extension may also be lim­
ited. The laboratory tests are usually negative;
a mildly increased sedimentation rate and a
left shifted differential blood test may possi­
bly be observed in consequence of the earlier
influenza.

Imaging diagnostics. On ultrasono­
graphy, the accumulation of fluid in the joint
is detected unmistakably. It is more important
to evaluate whether the hip synovitis and fluid
accumulation have caused any impairment to
the circulation of the femoral head. The bone
scan may demonstrate perfusion loss in the
proximal femur area, but this is rarely neces­
sary. In all cases of transitory arthritis, bilat­
eral anteroposterior and Lauenstein X-ray im­
ages are advised, and the diagnosis should be
confirmed in case of negative X-ray findings.
Treatment. The patient must rest and the
basic condition (influenza) is to be dealt with.
If not contraindicated, nonsteroidal anti­
inflammatory drugs are advised. The com­
plaints cease within a few days. Walking and
weight-bearing are allowed after the motion
of the hip has become unrestricted.
28. 1.9. Juvenile acetabular
protrusion
Protrusion is the invasion and distension of
the medial wall of the acetabulum toward the
pelvis. Juvenile acetabular p rotrusion, which is
usually unilateral, causes intermittent intense
hip joint pain.
Fig. 28.42.
Protrusio acetabuli in an elderly person with sec­
ondary arthrosis. The Wieberg CE angle is greater
than the normal 20-40°.
28. H i p d isorders � 353
Three types of acetabular protrusion are
differentiated:
1 . Juvenile protrusion : This is a rare condi­
tion, with intermittent hip complaints.
Exonerative rheumatoid arthritis or
non-differentiated collagenosis is possible
in the background. The condition involves
only the hip joint.
2. Acetabular protrusion in the elderly: Sec­
ondary osteoarthritis of the hip gradually
develops on the basis of bilateral
acetabular protrusion, usually in subj ects
over the age of 5 0.
3 . Secondary acetabular protrusion : The pro­
trusion is due to some other identified ill­
ness (osteoporosis, osteomalacia, sys­
temic bone disorders, etc.).
History. There is a gradual onset of inter­
mittent hip pain, the hip pain sometimes being
severe. The pain is localized to the hip and
groin and possibly the thigh and knee too. It
occurs 4 times more frequently in girls and
young females, than in males.
Clinical symptoms. The restriction of hip
motion and pain in response to passive move­
ments are typical. The restriction of hip mo­
tion is not selective, but homogenous. Both
the external and internal rotation and also the
ab- and adduction are limited to some extent.
The flexion - extension usually remains intact.
As the condition progresses, the hip gradually
becomes uniaxial : flexion - extension is possi­
ble, but other movements may completely dis­
appear.
The end-stage of the process is osteo­
arthritis in early adulthood with joint space
narrowing and destruction.
X-ray signs. The medial wall of the
acetabulum is thinner, and in advanced cases
it protrudes toward the pelvis. K6hler ' s drop
figure disappears or is crossed over. The fem­
oral head is seated deep in the acetabulum,
and the Wieberg ' s CE angle is larger than 40°
(Fig 28.42.).
354 � 28. Hip d isorders
Treatment. I n case protrusion is diag­
nosed, consultation with a rheumatologist or
clinical immunologist is needed to verify any
possible background disease. Most cases are
seronegative, and the rheumatologist gives a
negative opinion. In these cases, treatment
with nonsteroidal anti-inflammatory drugs is
advised according to the complaints, supple­
mented by a few days of rest. If the hip com­
plaints persist, intra-articular depot cortisone
may be administered. The result is usually
spectacular: the complaints cease almost im­
mediately. If the complaints and arthrosis per­
sist, hip replacement is indicated, even in
early adulthood.
28. 1. 10. Snapping hip, external
coxa saltans
Coxa saltans i s present when t h e tractus
iliotibialis, the thick, strong bundle of the fas­
cia lata, jumps over the tip of the g reater
trochanter with a click. It takes place in late
childhood or early adulthood and involves
both genders. It is relatively rare.
Etiopathology. The occurrence is usually
unilateral if it is due to an accident or scar. If
its etiology is related to congenital develop­
ment, or to a connective tissue deficiency,
then it is observed bilaterally. Coxa saltans
has no relation to the hip joint, though the pa­
tients dramatically report that their hip "jumps
out".
The bursa, normally located between the
greater trochanter and the iliotibial tract, de­
creases friction of the fascia lata. In cases of
coxa saltans the smooth friction turns into
stretched click, resulting in bursitis.
History. The patients recount, that their
hip "jumps out" upon certain movements first
on one side, an then possibly bilaterally with
varying intensity.
Clinical symptoms. The patients can re­
produce the click while walking or squatting,
and the sign is well palpable when the exam-
iner ' s hand is placed on the trochanter. It is of­
ten palpated better with the patient in a supine
position, while the hip is flexed and extended,
with simultaneous adduction and internal ro­
tation. The greater trochanter may be tender,
indicating bursitis.
Treatment. When the pain and bursitis is
predominate, conservative therapy is advised
with some days of rest and nonsteroidal
anti-inflammatory drugs. When the click is
the main complaint, the tense bundle of the
tractus iliotibialis may be incised.
28. 1 . 1 1 . Inward or outward rotation
of the legs
Child ren may walk rotating their legs inward
or less often outward. This is common and is
not pathological, but rather a normal variant.
Its incidence is 15-20% between the ages of 2
and 6. It affects both genders.
Etiopathology. It may be regarded as a
physiological episode, since the gait is stabi­
lized this way. The intoeing gait terminates or
improves in 95% of the cases in consequence
of the changes in muscle power. The anatomi­
cal and clinical factors may be as follows :
- The decrease in the retrotorsion of the
acetabulum and in the antetorsion of the
femoral neck is delayed. These levels
are more pronounced in childhood,
gradually decreasing to adulthood.
- Overactivity or weakening of the ilio­
psoas muscle, the active component of
the anterior acetabular wall. The former
may result in intoeing, and the latter in
an externally rotated gait.
- An imbalance between the external and
internal rotating hip muscles.
- A valgus knee deformity (more frequent
in girls). The compensatory gait in the
valgus knee could be a mild intoeing
gait.
- A rotation, ossification deformity of the
leg, when the leg is internally rotated

while the kneecaps look anteriorly. It is
often accompanied by varus leg, proving
the mild ossification disturbance of the
leg.
- Flatfoot, when the chi Id compensates
the valgus heel with internally turned
feet.
- A structural or dynamic form of pes
adductus. (In the dynamic form, the
deformity is seen only during walking
due to the overactivity of the abductor
hallucis muscle, it discontinues at rest).
Clinical symptoms and treatment. The
child is taken to the doctor because of the
strange gait, with inward turned legs. The
walking pattern frequently shows a familial
accumulation (an inherited alternative gait).
Other features of underdeveloped muscles are
often seen (e.g. flatfoot, valgus knee, sagging
belly or a negligent posture).
If no obvious etiology is found, spontane­
ous correction is expected by puberty. In
cases of flatfoot or valgus knee, application of
a supinating heel wedge to the footwear is ad­
vised. Sporting activity, swimming and ball
games should be encouraged. Exemption
from physical exercises at school is inappro­
priate.
In cases of extreme rotation of the legs,
with a valgus knee of more than 1 50, a marked
metatarsus varus operative correction may be
required.
28. 1. 12. Limb shortening
Limb shortening is considered t o exist, if the
difference in length between the lower limbs
exceeds 1 cm.
Types
� Functional shortening: This is caused by
an adduction and flexion contracture of the
hip. It is resolved if the primary source is
eliminated (apparent shortening). An
abduction contracture of the hip results in
a relative elongation, which is also solved
when the primary source is eliminated.
28. H i p d i s o r d e r s � 355
� Absolute shortening: The distance
between the anterior superior iliac spine
and the lateral (or medial) ankle is shorter
than on the other side. It may be caused by:
- Hip dislocation
- Coxa vara
- Perthes ' disease
- A congenital local ossification
malfunction in the growth plates
- Inflammation of the growth plates
- Trauma of the growth plates
- Radiotherapy or the action of an
iatrogenic noxa on the growth plates
- Congenital limb hypoplasia
Shortening may involve the upper or the
lower limbs. A major functional problem is
caused in the lower limbs. Due to the asym­
metric load, lumbar spine changes may occur.
Treatment. The treatment is determined
by the age of the patient, the cause and the ex­
tent of the shortening. It is also influenced by
the height of the patient relative to the average
height of his age group.
Limb shortening categorization :
- 1 -3 cm: conservative treatment, foot­
wear correction
- 3 - 1 5 cm: surgical intervention is con­
sidered
- over 1 5 cm: amputation at the middle leg
is considered, and application of a mo­
dem light prosthesis.
Surgical solutions:
Epiphyseodesis, obstruction of growth on
the longer limb.
Staples bridging the distal femur and prox­
imal tibia growth plates may achieve tempo­
rary epiphyseodesis. This procedure may be
repeated.
� Limb lengthening or shortening
procedures in one session
This involves metaphyseal or diaphyseal
shortening of the longer limb. Metaphyseal
shortening may be carried out at the proximal
and distal metaphysis of the femur and at the
356 � 28. H i p d isorders

a c

Fig. 28.43.
Met ho ds of metaphyseal shortening on femur (a, b) and tibia (c) a: Proximal femural metaphysis, b: distal
femural metaphysis, c: proximal tibial metaphysis.

proximal metaphysis of the tibia

(Fig. 28.43.). Diaphyseal shortening
is performed at the midshaft on both
the femur and the tibia (Fig. 28.44.).
This is advocated in case of tall chil­
dren when the length discrepancy is
less than 4 cm.
Elongation osteotomy at the
midshaft of the femur or the tibia in
one sessIOn:
- This comprises transverse osteo­
tomy in the middle part of the
femur diaphysis. With use of a
distractor, an elongation of 2-3 cm
can be achieved.
- Corrective osteotomy of the femur
b
distal metaphysis with moderate
elongation is performed. This is
particularly indicated when mild
shortening is associated with
substantial valgus knee. These
Fig. 28.44. procedures are carried out rela­
Methods of diaphyseal shortening of the longer limb in one ses­ tively rarely, since merely 2-3 cm
sion. a: femur, b: tibia. elongation is achieved.

� Continuous limb lengthening in
multiple sessions:
- This procedure is applied most
frequently nowadays; the original limb
length can be restored. It is indicated for
shortenings between 3 and 1 5 cm. The
larger the shortening, the longer the
procedure. It may be performed either in
childhood or in young adults. The
collaboration of the child may be
expected after 8 years of age, the
procedure demands continuous
cooperation between child, parents and
physician.
� Devices for continuous limb lengthen­
ing (Fig. 28.45. a, b) :
- Wagner type telescopic external
fixateur: Transverse osteotomy is
performed in the middle of the femur or
tibia diaphysis. The fragments are fixed
with strong screws (Schanz screws)
driven into the distal and proximal
metaphysis, and the screws are held by
the external fixateur. This procedure is
able to exert either distraction or
a b
Fig. 28.45.
Devices for continuous limb lengthening. a: Wagner's diaphyseal lengthening, b. llizarov's metaphyseal
lengthening.
28. Hip d isorders � 357
compression. The modem systems have
elastic telescopic solutions built in.
- When Ilizarov type rings are used, the
fragments are stabilized with stretched
K-wires. The rings are fixed by 3 axial
telescopes parallel to the limb axis. Both
distraction as well as compression can
be achieved. This device is more useful
in fixing metaphyseal osteotomies,
where the ossification is better.
Both procedures have advantages and dis­
advantages. Ilizarov rings have recently be­
come more popular.
� The steps in continuous elongation :
1 Psychological training: This is neces­
sary to convince the child, together
with detailed, repeated discussions of
the working process between physi­
cian, parents and physiotherapist. This
is the only way compliance of the child
can be assured.
2 Special exercises are implemented be­
fore the operation in order to elongate
the neighboring muscles and tendons .
358 � 28. Hip d isorders
This may decrease the necessity of sup­
plementary operations.
3 The procedure of continuous elonga­
tion: After the insertion and stabiliza­
tion of the external fixateur, the trans-
Fig. 28.46.
a: Following a d istal femur metaphysis fractu re: the
growth plate is closed on the left, resulting in a
1O-cm limb shortening.
b: After the limb lengthening, ossification is seen
between the frag ments.
verse osteotomy of the diaphysis or
metaphysis is carried out via a small in­
cision. On the operating table, an im­
mediate lengthening of 1 cm is accom­
plished. After a few days rest, further I
mm lengthening may be achieved once
or twice daily. This is continued until
the required length has been attained,
or contracture takes place in the neigh­
boring joints (Fig. 28.46. a, b). In cases
of severe shortenings, the continuous
elongation can be repeated once or
more after an interval of one year.
The rate of complications continuous
elongation is relatively high (3 0%). Osteo­
myelitis, or transient or permanent paresis
may occur, as may damage and restriction of
motion in the neighboring joints .
28. 1. 15. Congenital hemiatrophy
and hemihypertrophy
This is a condition with unknown etiology
and is not uncommon; one side of the body is
deficiently grown and hemiatrophic. The limb
length discrepancy may already be apparent
in the first year and usually progresses slowly.
Very minor length and circumferential differ­
ences are sometimes detected in both the up­
per and lower limbs; on other occasions, the
dissimilarity progresses. Congenital hemi­
hypertrophy is diagnosed when ill-defined
growth is observed on one side. It is often dif­
ficult to decide whether the length difference
is due to hemihypertrophy or hemiatrophy.
28.2. Hip disorders in adu lts
28.2 . 1 . Functional anatomy
The hip joint is a spherical or limitless
joint. The socket is the acetabulum, and the
head is the femoral head. The elements of the
acetabulum (the hyaline-covered C-shaped
facies lunata, the ligamentum transversum
acetabuli, bridging the incisura acetabuli, the

pulvinar, the fat tissue filling the fossa
acetabuli, and the circular labrum) surround
the femoral head over its equator, and the j oint
is therefore classified as an enarthrosis.
The capsule is very strong, reinforced by
three ligaments, the iliofemoral, pubofemoral
and ischiofemoral ligaments. These are at­
tached to the femoral neck in the same direc­
tion, turning from the caudal and posterior di­
rection. This is important from a mechanical
point of view. The ligamentum capitis femoris
plays a role mainly in the blood supply of the
femoral head.
Flexion and extension of the hip are ac­
complished around the transverse axis con­
necting the centers of the femoral heads. Dur­
ing flexion, the spiral twisted external liga­
ments loosen, during extension they tighten.
In the hip, 5- 1 5 ° of extension is possible. Ab­
duction and adduction are achieved around
the sagittal axis. The internal and external ro­
tation are performed around the construction
axis. Combination of the above-mentioned
motions effects the conical circumduction.
The movement of the hip j oint is powered
by a number of inner and outer muscles. Since
these muscles have a mixed function, it is use­
ful to discuss them according to their main
function.
- M. iliopsoas : the only real flexor of the
hip, which also has adduction and
internal rotation effects.
- M . tensor fasciae latae: a flexor of the
hip, a knee extensor and a hip internal
rotator.
- M . gluteus maximus : a hip extensor.
- M. gluteus medius and minimus: hip
abductors.
- M. piriformis : an external rotator and
abductor.
- M. obturator internus, gemellus superi­
or, inferior, obturator externus and
quadratus femoris : external rotators.
- Adductor muscle group : m. adductor
longus, adductor brevis, gracilis and
adductor magnus : adductors, internal
rotators.
28. Hip d isorders � 359
28.2.2. Deformities of the hip -
prearthrosis
Prearthrosis is a condition in which the joint
cartilage cover is present, and the complaints
a re minimal (fatigue and intermittent pain),
but early osteoarthritis is expected, consider­
ing the deformity, changed biomechanics, or
joint incongruence as a resu lt of earlier ill­
nesses or trauma.
Cartilage damage is a consequence of essen­
tially the fol lowing major mechanisms:
� A pressure increase on any given loading
surface
� A decrease of the loading surface
� Incongruence between the joint su rfaces
� Direct damage to the cartilage su rface
Etiologic factors.
- Pediatric hip diseases that healed with
deformity (congenital dislocation of the
hip, epiphyseolysis capitis femoris,
Perthes ' disease, etc.
- Changes in collodiaphyseal angle (coxa
vara, valga, or increased anteversion)
- Acetabular protrusion
- Post-traumatic conditions
- Inflammation (bacterial, rheumatic,
etc.)
- Metabolic diseases with direct cartilage
damage (gout or ochronosis)
- Tumors destroying the joint surfaces
The most common factor, the effect of the
increased collodiaphyseal angle, due to the
valgus deformity is analyzed in Pauwels '
biomechanical system a s follows.
In certain phases of the gait, the ipsilateral
limb is loaded, while the contralateral limb is
advanced. At these moments, the j oint is sub­
j ected to forces, which maintain balance and
prevent pelvic tilt on the other side, the pelvis
therefore remaining horizontal. This is ac­
complished by the hip abductor, namely the
gluteus medius muscle.
Pauwels' demonstrated that at these mo­
ments the following forces are acting (Fig.
28.47. a-c) .
360 � 2 8 . H i p d i so r d e rs
a b
A B
Fig. 28.41.
Loading conditions
A: A normal col lodiaphyseal angle (ratio of force /
lever arm (b:a) 3:1).=
B: Coxa valga (b:a 6:1). =
C: I ncongruence (a) congruence (b) (following cor­
rection osteotomy).
The system works by the lever principle.
In the balanced position:
force x lever arm = load x load arm.
The lever arm is the horizontal proj ection
of the distance between the hip center and the
force vector of the balancing abductors (Fig.
28.47. A). The load arm is the projection of
the distance between the weight line and the
hip center. Under normal circumstances, the
ratio of these two arms is 1 : 3 . The hip abduc­
tors balance a 50 kg load with a 1 50 kg force.
The rotation point of the lever (the hip joint) is
loaded by 1 50+50 kg. If the power arm is
� '­ ./
a b
352 kIVcm 2 25,2 kIVcm 2
shorter, the force in hip abductors increases.
In cases of a steep (valgus) femoral neck, the
ratio may even increase to 1 : 6 (Fig. 28.47. B) .
Thus the load acting on the hip in this case
may reach even 3 5 0 kg !
The extent of cartilage wear is dependent
on the pressure, friction and congruence of the
joint surface. If a small area is subjected to
high pressure, or certain areas do not touch the
other joint surface, degeneration takes place
(Fig 28.47. C). Deviations of the collodia­
physeal angle (coxa valga, coxa vara, incon­
gruence or subluxation) may lead to hyaline
degeneration, and this condition may there­
fore be considered as prearthrosis.

28.2.2. 1. Valgus hip, coxa valga
If the coliodiaphyseal angle in adults is g reater
than 130- 135°, the condition known as valgus
hip is determined (Fig. 28.48. c).
The collodiaphyseal angle depends on the
age: the nonnal range at the age of 9 years is
1 3 5 - 1 38°, at 15 years, it is 1 3 3 ° , decreasing to
1 20° in elderly ages. The values for valgus de­
fonnities are therefore dependent on the age.
Coxa valga is rarely an independent entity,
it is often a component of other diseases, such
as congenital dislocation and dysplasia, if
these persist after treatment, or i f subcapital
coxa valga develops. Infantile cerebral paresis
and any noxa affecting the lateral part of the
proximal femur growth plate also cause
valgus defonnity. If the coverage of the femo-
Fig. 28.48.
Collodiaphyseal angle ranges in adults.
a: Varus deformity (95°), b: Normal (126°), c: Va lgus
deformity (150°)
Fig. 28.49.
Right-side subluxed femoral head; 3D a image.
28. Hip disorders � 361
ral head i s only partial because o f acetabular
dysplasia, coxa valga subluxans is present
(Fig. 28.49.).
Depending on the extent of subluxation, a
number of groups are differentiated (after
Hartofilakidis) :
Stage 1 : Dysplastic hip. The head is attached
to the original acetabular hyaline (inde­
pendently of the extent of subluxation) .
The X-ray picture shows an upper seg­
mental defect: the acetabulum is shallow
because of the osteophytes padding up the
fossa acetabuli.
Stage 2: Low dislocation. The head is in a sec­
ondary socket, partially connected to the
primary acetabulum. The X-ray picture
shows an anterior/posterior segmental de­
fect: the acetabulum is shallow, with in­
creased socket and femur anteversion.
a
b
Fig. 28.50.
The left hip is in high dislocation, with severe hip
osteoarthritis on the right side.
a: Anteroposterior X-ray of pelvis.
b: 3 dimension a reconstruction.
362 � 28. Hip d isorders

Fig. 28.51.
��� a (_10 ° ) b ( 12 ° ) c (45 ° )

Different types of torsions of the proximal femur. a: Retrotorsion. b: Normal. c: Antetorsion.

Stage 3: High luxation. The X-ray picture - congenital developmental deformities of

shows a cranial and dorsal secondary the femur
socket, which is totally independent of the - fibrous dysplasia, Paget' s disease,
primary acetabulum. A segmental defect osteomalacia or rickets
is seen in the entire acetabular edge. - malunited fractured neck of the femur

The highly luxated hip (Hartofilakidis 28.2.2.3. Antetorsion and retrotorsion

Stage 3) is a special form of prearthrosis. The
of the hip (coxa antetorta and coxa
femoral head is located 5- 8 cm proximally, in
the substance of the gluteus medius muscle, retrotorta)
attached in a pseudoarthrosis to the iliac crest
(Fig. 28.50. a, b). The patient overloads the Rotation of the proximal femur around the
intact, longer limb (especially if the length longitudinal axis of the bone is torsion. This
discrepancy is not equalized), and early may take p lace in two directions. In ante­
osteoarthritis of the uninvolved hip therefore torsion, the ventral angle of the line connect­
ing the centers of the head and the greater
occurs (Fig. 28.50. a) .
trochanter exceeds the normal 15°. In retro­
version, this angle is less than 15° (Fig. 28.51.
Constitutional varus or valgus hips are of­
ten detected in adults with well-developed
a, c).
sockets and congruence. These are not consid­
ered to be prearthrotic conditions.
Antetorsion is much more common. It
rarely occurs on its own, usually being de­
tected in association with infantile cerebral
28.2.2.2. Va rus hip, coxa vara

Coxa vara deformity is present, when the

collodiaphyseal angle in adults is less than
120° (Fig. 28.48. a). Coxa vara is a conse­
quence of pediatric hip afflictions, which leave
residual symptoms despite treatment:

- osteochondritis after the treatment of

congenital hip dislocation
- femoral head deformities after Perthes '
disease
- coxa vara deformity after epiphyseolysis
capitis femoris
- congenital developmental deformities of Fig. 28.52.
the femur (coxa vara infantum, or a Infantile cerebral palsy: bilateral coxa valga antetorta
proximal femoral deficiency) deformity.

palsy (Fig. 28.52.), or hip dysplasia with a
valgus deformity. In cases of maj or ante­
torsion, the patient walks with typical inter­
nally rotated lower limbs, in order to achieve
that their hip j oints act in normal position.
28.2.2.4. Other p rearthroses
Inflammatory illnesses in childhood may
also be considered as prearthrotic conditions.
The systemic inflammatory illnesses are in­
cluded here : rheumatoid arthritis, epiphyseal
osteomyelitis, or purulent arthritis, which
even after healing may end up with osteo­
arthritis in adulthood.
The primary acetabular protrusion occurs
in puberty (see Chapter 2 8 . 1 ) . Medialization
of the femoral head leads to a bio­
mechanically disadvantageous state, which
may result in severe arthrosis in adulthood
with monoaxial (hinge) hip motion.
The secondary acetabular protrusion may
develop in consequence of an inflammatory
hip disease (e.g. rheumatoid arthritis); it de­
stroys the bottom of the socket, causing
medialization of the head. This is the "thin
wall" protrusion. It bears significance in rela­
tion to bone grafting.
28.2.2.5. Surgical treatment of
prearthrotic conditions
The objective is to restore the normal ana­
tomical and biomechanical situation and axis.
This way the arthrosis is prevented, or the pro­
cess may be slowed down.
Preventive operations in the hip area:
� Femur osteotomies
a) Intertrochanteric varus osteotomy
b) McMurray's medialization osteotomy
c) Valgus intertrochanteric osteotomy
d) Subtrochanteric osteotomy
� Pelvic osteotomies
� Femur osteotomies
Femur osteotomies may be carried out in
the intertrochanteric or subtrochanteric seg-
2 8 . H i p d i s o r d e rs � 363
ment. They normalize the axial deformities
and power arms. Each one involves a different
way to achieve the same goal, namely to
change the morphology or static of the limb,
thereby improving the function (sub­
trochanteric osteotomies).
a) Intertrochanteric varus osteotomy
This intervention is performed in
prearthrosis or early arthrosis, to correct the
axis of the steep femoral neck, to improve the
loading aspects and j oint congruence (Fig.
28.53. a, c). A medially based triangular
wedge is sawn out from the intertrochanteric
area of the femur. After removal of the wedge,
the bone ends are united and firmly fixed (sta­
ble osteosynthesis). During the operation, si­
multaneous derotation is possible to decrease
the enlarged antetorsion of the femur so as to
further improve the congruence ofthe femoral
head to the socket.
Precondition ofthis operation is the appro­
priate abduction of the j oint, since the varus
mechanism decreases part of the abduction
range of the joint.
Another precondition is, that the femoral
head must be more centralized on the abduc­
tion film, with better joint congruence.
b) McMurray's medialization osteotomy
The femur is cut through in the
intertrochanteric area in a plane inclined
slightly upwards, and the distal bone end is
displaced medially by half the width of the
bone and fixed in this position. No wedge is
removed.
Through the displacement of the lower
bone end, the muscles relax, causing an ad­
vantageous effect. This operation is currently
rarely performed because of the availability of
arthroplasties. The bone displacement causes
a deformity in the medullary canal, which
may make it impossible to insert of the stem of
the artificial hip.
c) Valgus intertrochanteric femur
osteotomy
The aim of this procedure is to correct a
varus femoral neck to enhance the congru-
36 4 � 28. H i p d isorders
b c
ence, to decrease the tension of selected mus­
cles (adductors and iliopsoas) and to increase
the tone of the hip abductors (Fig. 28.54. a, b).
In the operation, a laterally based wedge is
sawn out from the intertrochanteric area of the
femur. After removal of the wedge, the bone
ends are reduced and firmly fixed with stable
osteosynthesis. Due to the valgus, the femoral
head is turned laterally in the socket, the con­
gruence improves and the distance between
the origin and attachment of the muscles
changes beneficially.
A precondition of this operation is the
adduction reserve of the joint, since the varus
mechanism decreases part of the adduction
range of the j oint. Simultaneous derotation is
also possible.
The intertrochanteric femur osteotomy is
preceded by a careful examination of the pa-
Fig. 28.53.
a: Outline and effect of varus and medializing
intertrochanteric osteotomy (1: hip abductor m.,
2: m. iliopsoas, 3: hip adductor m.).
b: Femur valgus deformity - preoperative X-ray.
c: After varus osteotomy.
(Because of the shallow socket, Chiari's pelvic
osteotomy was performed later.)
tient and geometrical planning based on the
X-ray films. The size of the wedge removed is
determined by the measured collodiaphyseal
angle.
The effects of intertrochanteric femur
osteotomy may be summarized as follows:
- By centralization of the femoral head,
the joint congruence improves, the
loaded surface increases, and the
pressure is better dispersed, and
therefore decreases.
- The power of the abductors, adductors
and iliopsoas is altered advantageously
by the changed distance between the
origin and the attachment of the
muscles.
- The alteration of the collodiaphyseal
angle changes the length of the power
arm (varus osteotomy) in accordance

3
a
Fig. 28.54.
a: Outline and effect of valgus intertrochanteric
osteotomy.
b: X-ray of valgus intertrochanteric osteotomy.
with Pauwels' theory, and consequently
the pressure generated by the abductors
on the joint decreases.
- The consolidation of the osteotomy
rearranges the circulation of the pro­
ximal femur, and decreases venous
stagnation, giving rise to an advan­
tageous, not negligible biological effect.
� Pelvic osteotomies
Pelvic osteotomies are discussed in the
chapter on congenital hip dislocation. Chiari ' s
osteotomy i s performed i n cases o f pre­
arthrosis in early adulthood (Fig. 28.55. a, b).
On the same principle, acetabular roof con­
struction is also considered (see Chapter
28. 1 ) .
2 8 . H i p d i s o r d e rs � 365
Fig. 28.55.
Chiari's pelvic osteotomy in hip dysplasia:
a: X-ray picture of dysplastic hip.
b: After medial displacement of the proximal part of
the iliac bone, the coverage of the femoral head and
the loading conditions are better.
28.2.3. Idiopathic fem u r head
necrosis (aseptic, avascular fem u r
head necrosis)
This is ischemic necrosis involving the frontal
and u pper lateral weight- bearing area of the
femoral head, leading to severe joint defor­
mity.
Symptoms. Pain in the hip, restriction of
the range of motion and a limp. The X-ray pic­
ture shows a wedge-shaped sclerotic area in
the upper perimeter of the femoral head,
366 � 28. H i p d isorders
which may collapse. I n a large proportion o f
the cases (30%) the process is bilateral.
Incidence. Zizic and Hungerford report
that every thousandth individual is exposed to
the possibility of developing avascular femur
head necrosis. It is common between the ages
on 0-60 years. The male - female ratio is 4: 1 .
The pathophysiological basis of the condi­
tion was discovered in 1 948 by Chandler, who
compared the circulation disturbance of the
femur head with the occlusion of coronary ar­
teries in the heart and called the avascular fe­
mur head necrosis "the coronary disease of
the hip".
Etiopathology. This has not been clarified
precisely. Data in the literature point to vari­
ous factors which predispose avascular femur
head necrosis:
� chronic steroid therapy
� alcohol a buse
� cytostatic treatment and X-ray irradiation
� a n occupation involving a risk of caisson
disease
� sickle cell anemia or other he mo­
globinopathy (thrombus production)
� metabolic diseases (lipid metabolic
d isorder, hyperg lycemia, hyperuricemia)
� storage illnesses (Gaucher's disease)
a b
Fig. 28.56.
Bilateral femur head necrosis: Stage 3 on the right-side, Stage 1 on the left. a: X-ray. b: MR image (horizontal
plane).
� autoimmune diseases (rheumatoid arthritis,
systemic lupus erythematodes, etc.)
� injuries
Chronic steroid therapy and alcohol abuse
are among the causes in 65-75% of idiopathic
femur head necrosis. The blood supply of the
femoral head is ensured by the circumflex ar­
tery of the femur. The arteria capitis femoris
only rarely functions in adults. The anastomo­
ses ensure the blood supply of the femoral
head, but the circumflex branches are re­
garded as terminal arteries, in the event of oc­
clusion, the supplied area necrotizes.
Clinical symptoms. The onset of the ill­
ness is uncertain: pain is felt in the hip, the
thigh, or possibly in the knee, and becomes
stronger on loading. As the affliction pro­
gresses, the pain intensity fluctuates. Fluid ac­
cumulation is associated with joint inflamma­
tion, increasing both the pressure as well as
the pain. Resolution of the inflammation may
ease the soreness. Thigh muscle atrophy is
also seen.
The hip internal rotation, abduction and
adduction become limited. Flexion - exten­
sion is maintained for a relatively long time.
The limp is caused primarily by the pain,
but later by the limited motion.

X-ray signs. The radiological phases of
this condition were determined by Arlet and
Ficat. These phases affect the therapeutic ac­
tivity.
Since the changes in the early phase of the
condition are difficult to identify on an X-ray
picture, not only an anteroposterior film, but
also Lauenstein views are taken. Both hips
must be observed because of frequent bilat­
eral involvement.
Ficat 's phases:
Phase 0: This is the preclinical and pre­
radiological phase ("silent hip", no clini­
cal or radiological signs). The intra­
medullary pressure is increased. In 60% of
the cases, the condition later progresses to
Phase 1.
Phase I. There are early clinical symptoms,
but no radiological signs. A bone scan is
advised. MRI is invaluable. The leading
symptom is a sudden groin pain that radi­
ates to the thigh, which intensifies during
the night. Movements are restricted (Fig.
28.56. a, b).
Fig. 28.57.
Left side: Stage 3 femur head necrosis: with im­
ploded wedge-shaped sequestrum.
28. Hip disorders � 367
Phase /1. Typical X-ray changes are observed:
a wedge-shaped area with a sclerotic mar­
gin; the joint surface is intact (precollaptic
state) . The clinical symptoms stagnate or
progress.
Transient phase If-Ill: S ickle sign: a
sickle-shaped line appears under the j oint
surface due to a subchondral fracture. Seg­
mental collapse is seen.
Phase Ill: The joint surface has collapsed, and
a sequestrum has separated. The j oint
space is intact or wider. There is evidence
of inflammation and fluid accumulation.
The pain is severe and motion is limited
(Fig. 28.56. a, b, 28.57).
Phase IV: This is the terminal phase, evidence
of secondary arthrosis is seen. The j oint
space progressively narrows; osteophyto­
sis; the head is deformed. Limited j oint
motion.
Differential diagnosis. Having identified
the clinical and radiological signs the diagno­
sis is not problematical, especially if the his­
tory has been precisely identified. This condi­
tion is to be differentiated from hip algo­
dystrophy, inflammation and tumors .
Besides the X-ray image, the bone scan
helps to verify the bone necrosis. CT defines
the contours of the head. MRI and 3D CT tests
provide particularly valuable assistance in
confirming the diagnosis and in deciding on
the treatment.
Treatment. In the early phases non­
weight-bearing, swimming, gymnastic exer­
cises, nonsteroidal anti-inflammatory drugs,
pain killers, physiotherapy and medical spa
treatment may be recommended. However,
the condition progresses and the conservative
treatment does not lead to substantial results.
The surgical treatment varies in the differ­
ent phases.
- In Ficat phases 1. and 11. , when, despite
the bone necrosis, the j oint surface is
still intact, drilling of the necrotized
area, cancellous grafting and a vas-
368 � 28. Hip d isorders
cularized implant may produce
reasonable results .
- In Ficat phase Ill. , unloading and
transposition of the collapsed area may
be attempted. This is done by inter­
trochanteric flexion osteotomy. A pre­
condition of this intervention is that the
extent of the necrosis must be less than
50%. If it is more (in the maj ority of
cases), total prosthesis implantation is
the method of choice.
- In Ficat phase Ill . , the condition
presents as secondary osteoarthritis, and
total prosthesis implantation is in­
dicated.
28.2.4. Coxarthrosis
(hip osteoarthritis, arthrosis
deformans coxae)
This entity is characterized by deformity, pain
and limited movement in the hip due to carti­
laginous and bone degeneration.
Symptoms. Pain, with progressing restric­
tion of movement, contractures and a limp.
The j oint space becomes thinner, the joint
contours are uneven, the j oint surfaces are de­
formed, and osteophytes, and degenerative
cysts may develop.
Incidence. In the population aged 65-74
years, X-ray signs (Kellgren) of hip osteo­
arthritis are present in 1 6-25%. Symptoms re­
quiring treatment are considerably less fre­
quent: 6- 1 0%. Male - female ratio is 1 : 2 . 5 .
The ratio of primary and secondary hip
osteoarthritis in the operated population is 65
and 3 5 % respectively.
Etiopathology. Hip osteoarthritis has two
different forms :
I . Primary hip osteoarthritis: Its cause is un­
known, however literature data suggest
that the condition is determined. In paral-
lel with aging, the function of the cartilage
cells changes, their proteoglycan produc­
tion decreases, which leads to a decrease
in the water content of the intercellular
matrix, and to damage of the integrity of
the collagen network. Behind the genetic
predisposition, gene mutations producing
faulty collagen, or ill-defined antigen as­
sociations may be part of the cause.
2 . Secondary hip osteoarthritis : This devel­
ops on the bases of other hip disorders.
These may originate from childhood or
adulthood. The roles of congenital dislo­
cation, Perthes ' disease, epiphyseolysis
capitis femoris, primary acetabular protru­
sion and inflammatory diseases were dis­
cussed in the chapter on prearthrosis.
In adults, inflammatory diseases (septic
arthritis, rheumatoid arthritis and spondyl­
arthrosis ankylopoetica), idiopathic aseptic
necrosis of the femoral head, fractures involv­
ing the joints, post-traumatic conditions near
joints, metabolic diseases, bone dysplasia,
and arthropathy may all result in secondary
hip osteoarthritis.
The pathology of osteoarthritis was dis­
cussed earlier in connection with diseases of
the j oints (Chapter 1 9).
Macroscopically, the cartilage is no longer
gleaming, its mother-of-pearl white color has
turned to yellowish brown. The hyaline is
fiberized and softened in places. The areas
subj ected to the greatest surface load have no
cartilage cover, and ebumated (condensed)
bone is visible. The subchondral cavities and
degenerative cysts often communicate with
the j oint cavity.
The femoral head gradually loses its ball
shape, and widens due to the marginal osteo­
phytes. The appositions that grow around the
facies lunata in the center of the acetabulum
may block the fossa, and a double acetabular
bottom may be formed. These central
osteoarthritic products (socket bottom osteo­
phytes) literally displace the femoral head and
secondary subluxation is observed.
B esides the changes in the cartilaginous -
bony structures, thickening, inflammation or

shrinking of the capsule may also play a role
in the complaints and symptoms.
In consequence of the changes listed
above, the condition known as hip osteoarth­
ritis or arthrosis deformans coxae develops.
Clinical symptoms. The main complaints
are pain, limited movement and a limp. Ini­
tially, the pain presents only after getting out
of bed in the morning, or upon standing up
from a chair. It may subside during moving.
Later on, the pain increases and becomes con­
tinuous, both in the daytime and at night.
The pain may be located deep in the hip, in
the groin, around the greater trochanter or the
sacrum, or in the front of the thigh, radiating
to the knee.
The patient' s quality of life depends on the
extent of the pain. As the condition pro­
gresses, the patient' s working and walking
ability diminishes, and he / she may ulti­
mately become unable to take care of him­
selflherself.
The limiting of their movements is recog­
nized while they attempt to carry out everyday
tasks. In the beginning it may be difficult to
put on a pair of socks, or to get on a bus . The
limits of movements of the joint gradually
narrow. First the extension becomes limited,
i.e. a flexion contracture arises. Later the in­
ternal rotation decreases, which is followed
by abduction and adduction. Monoaxial
movements may succeed when the hip joint
has only flexion and extension, but ultimately
the flexion also becomes limited, and the hip
is in such a state that only very limited move­
ment remains . Flexion, adduction and an ex­
ternal rotation contracture usually prevail.
The limp is due to the clinical symptoms
listed above, the pain resulting in a protective
limp. Because of the flexion contracture, the
hyperextension phase of the step is missing.
Adduction flexion contractures cause an ap­
parent limb length difference, which increases
the limp.
X-ray signs. In the evaluation ofthe radio­
logical picture of the primary and secondary
forms of hip osteoarthritis are differentiated.
2 8 . H i p d i s o r d e rs � 369
� Primary forms (Fig. 28.58) :
- The j oint space is narrow.
- The j oint contours are uneven.
- Subchondral sclerosis is observed (on
loaded areas) .
- Degenerative cysts develop.
- Osteophytes are seen at the socket
margin and on the head - neck border
(collar osteophytes).
- A double socket bottom may be present.
The primary form may be associated with
protrusion. The head may be lateralized, when
the thickened double socket bottom sublux­
ates the head from the central position.
Fig. 28.58.
X-ray image of bilateral primary hip osteoarthritis.
� Secondary forms (Fig. 28.59) :
- When the head is not central and the
incongruence is severe, the hip
osteoarthritis is usually secondary.
- The same X-ray signs are present as in
the primary form.
- The X-ray signs depicting former
disorders, which have not fully
recovered anatomically may be
identified (e.g. congenital dislocation
(Fig. 28.60.), epiphyseolysis capitis
femoris, Perthes disease or other types
'
of head necrosis, j oint infection, etc. ) .
Differential diagnosis. This condition is
identified without any difficulty thanks to the
3 70 � 28. Hip disorders
Fig. 28.60.
Secondary hip osteoarthritis on basis of bilateral de­
velopmental dislocation of the hips with typical
short neck and high trochanter.
clinical and radiological characteristics.
Symptoms of diseases of the neighboring re­
gions may occasionally hinder establishment
of the clinical diagnosis. These can include
degenerative involvement of the lumbar spine
accompanied by root irritation (e.g. lumbar
disc hernia), diseases of the sacroiliac joint
(arthritis and inclplent spondylarthritis
ankylopoetica), or knee diseases (meniscal
tear, arthrosis, etc.). On the other hand, pain
radiating from the hip to the knee may suggest
the presence of a knee disorder.
Treatment. Before an account of the
treatment methods, mention should be made
of the means of prevention. In the prevention
of both types of hip osteoarthritis, a healthy
lifestyle, based on healthy nourishment and
regular exercise, is essential . Body weight
must be kept at the optimal level or weight
loss should be considered if necessary. Even
in the presence of a prearthrotic state, regular
swimming, gymnastics and sports are those
factors, which are able to protect the compen­
sated state of the hip musculature for a long
period and to slow down the progress of the
disease, which eventually requires either con­
servative or surgical attention. Apart from
changes in lifestyle, it may also be necessary
to change schools or working conditions.
To prevent secondary hip osteoarthritis,
the best possible treatment of the primary dis­
ease is indispensable. The incidence of con­
genital dislocation of the hip has decreased
significantly in recent decades due to the early
screening and successful treatment. The ef­
fective management of epiphyseolysis capitis
femoris and Perthes ' disease has also led to a
decrease in the incidence of hip osteoarthritis.
Septic arthritis and hip tubercolosis, which
earlier resulted in ankylosis (full stiffness of
the joint) have now become conditions that
rather induce hip osteoarthritis.
Early diagnosis and early and appropriate
treatment may delay severe hip osteoarthritis.
The results are modest in cases when the hip
osteoarthritis develops after femoral head ne­
crosis and fractures involving the joint.
Conservative treatment: is applied in hip
osteoarthritis to decrease the pain, to ease the
contractures and the limp, and to increase the
range of motion, and muscle power. Medica­
tion, physiotherapy and spa treatment may be
employed.
The following medications or their combi­
nations may be effective :

� Pain killers
� Nonsteroidal anti-inflammatory d rugs
� Steroid d rugs
� Muscle relaxants.
The most important goal is to relieve the
pain. Instead of general pain killers, non­
steroidal anti-inflammatory drugs are pre­
ferred, since the osteoarthritis-induced in­
flammation is a significant source of the j oint
pain. Muscle spasm and contracture may be
alleviated by a combination of these drugs and
muscle relaxants.
Steroid therapy is a very effective element
of the anti inflammation treatment. It does
have harmful local and general side effects,
however, caused by the chronic steroid ther­
apy: gastrointestinal ulcer, osteoporosis, ste­
roid necrosis, steroid diabetes and Cushing 's -
syndrome. Local steroid use (intra- or
para-articular) may be indicated for a certain
time period and in a limited dose.
A traction device is often applied to unload
the hip joint, and to decrease the spasm and
contractures and the pain. It can be used even
at home, combined with other methods. A
walking stick is also useful.
Surgical treatment. In prearthrosis or
moderate hip osteoarthritis, femoral and pel­
vis osteotomies are performed to improve
congruence and containment (see Chapters
2 8 . 1 . and 2 8 .2 . 2 . 5. ).
I n advanced cases, the obj ective of surgi­
cal treatment is to decrease pain and restore
movement and stability. Primarily a hip endo­
prosthesis is implanted; occasionally, stiffen­
ing of the hip or (after prosthesis removal)
pseudoarthrosis formation is carried out.
- Hip arthroplasty
- Hip arthrodesis
- Resection, or excision arthroplasty
� Hip arthroplasty
The total hip endoprosthesis embodies the
most noteworthy achievement in orthopedics.
A patient with a painful restriction of move­
ment can by this means be freed from all the
complaints, and the mobility and quality of
life restored to normal.
28. Hip disorders � 371
The system of hip endoprostheses i s the fol­
lowing:
� surface replacement endoprostheses
� cervicocapital endoprostheses
� total hip endoprostheses
cemented
uncemented
hybrid
Surface replacement endoprostheses. The
key procedure of this method is double cup
arthroplasty. After the removal of the dam­
aged joint surfaces, a plastic socket is ce­
mented into the acetabulum, and a matching
metal cup is cemented onto the femoral head.
Earlier, the results of this operation were
modest, but it has nowadays been resurrected
in a form involving a metal cup - metal head.
Cervicocapital endoprostheses. Follow­
ing removal of the head and the neck, a
cervicocapital endoprosthesis can be inserted
into the medullary cavity of the femur. The
metal head, moving in the cartilage-covered
bony acetabulum, ensures the joint function.
This procedure is mainly employed in opera­
tions involving the elderly, following frac­
tures of the femoral neck. This endoprosthesis
is also manufactured in a bipolar form: a large
metal ball is positioned in the cartilage cov­
ered bony acetabulum, inside this ball is a
plastic bushing containing a small metal ball.
The bipolar design increases the range of mo­
tion and the lifetime of the implant.
(:;,���total hip endoprosthesis. Augmentation
of the acetabular roof with bone block a nd screws.
3 72 � 28. Hip d isorders
Total hip endoprosthesis / cemented (Fig.
28.61). After exposure of the hip j oint, the
head and neck are resected. The acetabular
cartilage is removed, the socket is deepened
and the artificial cup is cemented home. After
the preparation of the femur medullary cavity,
a corresponding prosthesis stem is fixed. A
metal head is assembled onto the cone of the
metal neck, which is reduced to the polyethyl­
ene artificial socket.
Bone cement (consisting of polymethyl­
metacrylate) is made from two components
(fluid + powder). After mixing of the compo­
nents, they polymerize, and the cement sets
through an exothermic reaction (ca. 80 QC) the
cement is setting. This substance fixes the
prosthesis firmly in the bone cavity surface.
Total h ip endoprosthesis / cementless
(Fig. 28.62.). The cementless implants are
different in shape and surface from the ce­
mented ones. No bonding material is utilized
in these cases. The cup consists of two com­
ponents : an external metal ring and a plastic
lining. The surface of the external metal ring
is threaded, and can be screwed into the bony
acetabulum, but may also be designed to be
pressed firmly into the bony bed (press fit).
The plastic lining covers the head of the pros­
thesis.
The stem of the femoral components
matches the anatomical configuration of the
medullary cavity (anatomical prostheses).
The medullary cavity is prepared with ream-
Fig. 28.62.
Cementless total hip endoprosthesis.
ers matching the shape of the prosthesis,
which will therefore wedge into the cavity
(press fit). After the operation, the wedge ef­
fect and the press fit will ensure primary sta­
bility; later, especially in cases of a porous
surface, bony ingrowth takes place, and sec­
ondary, biological fixation evolves. The po­
rous surface ensures attachment to the bony
bed over a large surface.
Total hip endoprosthesis / Hybridfixation.
One component is fixed with, while the other
without cement.
The expected survival of both cemented
and cementless endoprostheses is similar:
90% at 1 0 years .
After the setting of the cement, the ce­
mented prosthesis is totally stable and ready
for weight bearing. This prosthesis has the
negative feature, that if the implant becomes
loose, removal of cement may cause bone
loss. The biological fixation of cementless im­
plants may take some time, but in the event of
its loosening, the exchange does not create
bone deficiency. The indications of a
cementless implant are young age and a good
bone structure ensuring the proper primary
stability. In cases of poor bone quality (osteo­
porosis or necrosis), defective anatomical sit­
uation (previous operations, a dysplastic
acetabulum, etc.) the cemented procedure is
recommended.
Minimally invasive endoprosthesis tech­
nique. This is the newest enterprise of pros­
thetic surgery. In a selected patient cohort
(young, active patients with no obesity, etc.),
the surgical incision is short (under 10 cm),
and the joint capsule is preserved as much as
possible, possible using minimal trauma­
tization. The postoperative pain and blood
loss are expected to decrease, mobilization is
swift, with a better cosmetic outcome. There
are also some risks to be considered, e.g. mis­
placed components due to the limited view,
unexpected tissue damage and bleeding.
� Fusion of the hip j oint, arthrodesis
This has almost totally been abandoned.
The procedure, which ensures pain-free sta­
bility, was used in young patients who ac-

quired a severe unilateral hip condition, while
performing standing or physically demanding
jobs. A good function of the lumbar spine and
the knee are preconditions of this arthrodesis,
since these structures will take over the lost
motion of the hip and their load will increase.
The increased load will sooner or later result
in the degenerative changes in the mentioned
joints, and the ipsilateral knee and contra­
lateral hip osteoarthritis will also develop.
These are the drawbacks of the arthrodesis.
� Resection (excision) arthroplasty
Resection (excision) arthroplasty is the fi­
nal solution in septic/aseptic failures of hip
endoprosthesis procedures. The stem,
Fig. 28.63.
Girdlestone situation following removal of total hip
endoprosthesis.
28. Hip disorders � 373
acetabulum (and possibly the cement) are all
removed ( Girdlestone operation; Fig. 28.63 . ) .
This state was earlier considered t o be final.
Today however, the patients demand a good
quality of life, therefore if the eradication of
the infection is safe and the anatomical cir­
cumstances allow, prosthesis replantation
may be attempted.
� Sterile loosening of a hip endoprosthesis
and revision arthroplasty
According to wide-range statistics, 90% of
cemented and cementless endoprostheses re­
main stable at 1 0 years . This proportion is
lower for young adults and in certain cases of
secondary hip osteoarthritis (75-80%).
The cause of the sterile loosening is that
the bone, the cement and the material of the
implants all have different moduli of elastic­
ity. The wear on the substances of the im­
plants is also a contributing factor. Granules
of polyethylene, metal and cement can accu­
mulate in the joint, together with hystiocytes
and foreign body giant cells, which may gen­
erate tissue reaction. This persevering granu­
lation tissue invades between the implant and
the bone and induces osteolysis, leading to
loosening of the implant. In these cases revi­
sion arthroplasty is advocated, which ac­
counts for 20-25% out of all endoprosthesis
interventions.
The success of the revision is dependent
on the anatomical circumstances perceived
during operation. If no bone loss is revealed,
revision is not problematical. Some years ago,
in the event of maj or bone loss, only the
Girdlestone state was reasonable : the patient
walked on the pseudo-arthrosis with a sup­
porting walking aid, with a pronounced limp
but without much pain. Today there are de­
vices to replace bony defects of both the
acetabulum as well as of the femur. The new
bone bed receives the new implant with a sta­
bility matching that ofa primary intervention.
The 1 0-year survival rate of revision
arthroplasties is similar (about 80%) to that of
the primary interventions .
 Laszl6 Hangody, M i kl6s Szen d r6i

29. Disorders of the knee

29. 1. Structure and functional
anatomy of the knee
The knee is the largest joint of the body,
with one of the most complicated structures; it
is classified by its motions as a trocho­
ginglymus. The longitudinal axes of the femur
and the tibia in the frontal plane (anatomical
axis) enclose a lateral angle (the physiological
valgus angle of the knee, which is approxi­
mately 1 73°). The knee is slightly eccentric:
the lateral condyle is somewhat flatter, while
the medial one is larger. The loading axis,
however, runs along the line drawn from the
femoral head through the center of the knee to
the ankle joint, and the loads on the medial
and lateral half of the knee are therefore equal
under normal circumstances.
The knee joint may be divided into 3 com­
partments : patellofemoral, medial and lateral
tibiofemoral j oints. The practicality of this di­
vision is that certain disorders (e.g. osteo­
arthritis) have different affinities for different
compartments.
During motion and walking, the joint sur­
face is loaded by extremely large forces. The
stability of the joint in each phase is provided
not by the bony structure and shape, but by
strong ligaments with complicated paths (pas­
sive stabilizers) and muscles (active stabiliz­
ers) (Fig. 29. 1 .) . The medial stabilizers that
prevent valgus displacement are the following
structures : the dorsomedial capsule, the su-

1 --��------��.� ______*H�_ 2
�
15 3
1. anterior cruciate ligament,
2. posterior cruciate ligament,
4
14
3. medial collateral ligament,
6 4. semimembranosus muscle,
5 5. medial superficial collateral ligament,
13 6. menisci,
12 --+'���
7. patellar tendon,
7 8. gracilis muscle,
9. semitendinosus muscle,
10. sartorius muscle,
8 11. membrana interossea,
12. tractus iliotibialis,
9 13. tendon biceps femoris,

11 ----t--_+_ 10
14. lateral collateral ligament,
15. popliteus tendon

Fig. 29.1.
Anatomy of the knee joint
3 76 � 2 9 . D i s o r d e rs of t h e k n e e
perficial layer of the medial collateral liga­
ment, the medial capsule ligament, the poste­
rior oblique ligament, the muscles of the pes
anserinus and the semimembranous muscle.
The latter muscles are also internal rotators;
they produce 20 an internal rotation of 20° at
the knee bent to 90°. The lateral stabilizers
are : the dorsolateral capsule, the lateral collat­
eral ligament, the m. popliteus, the iliotibial
tract and the m. biceps femoris. (The popliteus
muscle has an important function in addition
to stabilization: it rotates the tibia inward at
the start of flexion.) The other muscles ensure
the approximately 40° of external rotation of
the leg with the knee bent. The active rotation
around the longitudinal axis of the leg is pos­
sible only with the knee bent and relaxed col­
lateral ligaments. To achieve this, the flexors
attached to both sides of the tibia like reins ro­
tate the knee in and out. The internal rotation
is limited to 20° by the tense cruciate liga­
ments, and the external rotation to 40° by the
collateral ligaments.
The central stabilizing system of the knee
involves the menisci and the cruciate liga­
ments. The anterior cruciate ligament origi­
nates from the mediodorsal part of the lateral
femoral condyle and is attached widely in the
intercondylar eminence, between the anterior
horns of the two menisci. It contains 3 bun­
dles, which are partially twisted around them­
selves. In each stage of flexion, a different
bundle comes under tension. The posterior
cruciate ligament originates from the ventro­
lateral part of the medial femoral condyle and
runs backward and down to the tibial inter­
condylar fossa; it contains 2 bundles.
Functions:
� Both cruciate ligaments participate in
protecting the lateral stability.
� The anterior cruciate ligament prevents
a nterior subluxation of the tibia, while the
posterior cruciate ligament prevents the
posterior subluxation of the tibia and the
a nterior skidding of the femur over the fi­
xed tibia d u ring walking.
� I n every phase of the knee flexion, when
the collateral ligaments are somewhat
loosened, they ensure the stability of femur
on the tibia, and the axis of the control led
rolling-slide movement d u ring knee flexion.
The foremost motion of the knee is exten­
sion - flexion, which is effected around the
transverse axis connecting the epicondyles.
The extent varies individually; it is generally
around 1 3 0°, depending on the tension of the
extensor apparatus, and the soft tissue-mass in
the popliteal fossa. In extension, the collateral
ligaments are stretched, for the radius of the
sagittal curvature of the femoral condyles is
greater frontally. The end-point of extension
is secured by a number of factors, at the same
time inhibiting hyperextension. These com­
ponents are the collateral ligaments, the ex­
tremely strong posterior capsule, and the tense
cruciate ligaments. In the normal knee, about
1 0° of end-rotation occurs externally at the
end of extension. In this situation, the passive
stabilizers are tense, and the active ones (mus­
cles) may relax; thus standing with extended
knees does not require much muscle activity
to stabilize the joint. In this position, the joint
is stable. Apart from flexion, all other move­
ments are pathological and relate mostly to
damage to the passive stabilizers .
Physical examination of the knee: the follow­
ing factors are to be assessed:
� The axis (anteroposterior. and sagittal
plane)
� Fluid accumu lation
� Stability (anteroposterior and lateral,
rotational)
� Range of movement
� Muscle power
� Contractures (soft tissue, skin, muscle,
capsular or bony)
� The patel la reflex
� Meniscal tear
� Chondropathy and arthrosis signs
The technique of assessment is discussed
in the section on each disorder.

29.2. Congenital developmental
disorders
29.2. 1. Congenital knee dislocation
This rare disorder, which is frequently associ­
ated with other developmental anomalies,
may occur bilateral ly. The tibia is subluxed o r
fu lly dislocated anteriorly in t he sagittal plane.
The tibia plateau is attached to the ventral
surface of the femu r condyle.
Clinical symptoms. This disorder is usu­
ally detected at birth or in babyhood; the se­
verity varies. In cases of subluxation, the
proximal tibia is positioned anteriorly on the
femoral condyles to different extents, the fe­
mur and the tibia of the patient enclose a fron­
tally open angle. In mild cases, the knee
flexion is generally full, but in more severe
forms it may be badly limited. When luxation
is total knee flexion is possible, the flexor
muscles are displaced in front of the joint and
they act as extensors enhancing the hyper­
extension. In severe bilateral cases, the patient
is unable to walk, or occasionally stands and
walks on the posterior surface of the leg and
popliteal fossa.
X-ray signs. The lateral views clearly re­
veal the ventral subluxation of the tibial
condyles. The confronting joint surfaces may
be flattened.
Therapy. The hips and the entire lower
limbs must also be assessed, since the knee
disorder may be associated with multiple de­
velopmental disorders . In minor cases, early
conservative treatment may be successful.
Gentle reduction may be attempted even in
the first week after birth, followed by a circu­
lar plaster cast up to the groin.
With a frequent change of plaster and pas­
sive exercises, the knee flexion can gradually
be increased. In cases of subluxation or full
dislocation, surgery is indicated. Elongation
of the quadriceps tendon, and incision of the
shrunk ventral capsule are followed by appli-
29. D i s o r d e r s of the k n e e � 377
cation of a plaster cast for 4-6 weeks. After
that, intensive rehabilitation is essential.
29.2.2. Genu recurvatum
The range extension o f the knee surpasses
the normal 5- 0°.
Causes :
- Congenital: the tibial plateau is inclined
forward
- Idiopathic: abnormal load, standing
with a loose posterior capsule
- Post-traumatic: the ventral part of the
tibia growth plate is blocked
- Compensatory: in neuromuscular
disorders (quadriceps palsy caused by
poliomyelitis, and forced knee hyper­
extension in cases of equinus foot) .
Therapy. Depending on the etiology
osteotomy or orthosis may be selected.
29.2.3. Developmental disorders
of the patel la
Complete absence of the patella (aplasia
patellae) or hypoplasia is a rare congenital de­
velopmental disorder. The knee extension
strength may be weakened, but usually does
not cause a major functional deficit; the pas­
sive range of movement is full.
It is more common that the patella devel­
ops from two or more ossifying centers, and
they do not unite at the end of growth (bipar­
tite or tripartite patella). The hyaline surfaces
of the patella are even, and the disorder is not
a source of complaints, or only in the event of
overload.
A common finding is the high or deep po­
sition of the patella (patella alta or patella
baj a) . Together with the factors discussed
above, a high patella increases the chances of
habitual dislocation; in the baj a position, the
patella is compressed to the femur on flexion,
leading to chondropathy and chondromalacia.
3 78 � 2 9 . D i s o r d e r s of t h e k n e e
29.3. Repeated patella dislocation
and subluxation
29.3.1. Habitual dislocation
of the patella
The patella dislocates laterally on every occa­
sion, when the knee is flexed. It is clearly seen
and palpated. This occurs between the ages
of 5 and 9 years, i n older i nd ividuals it is ob­
served only occasional ly.
Etiopathology. The explanation is the
contracture of the quadriceps femoris muscle.
The contracture may be congenital or the ef­
fect of palsy, but the most common reason is
an inj ection (mainly antibiotics) into the
quadriceps femoris muscle in infancy or early
childhood. The inj ections lead to fibrosis,
which is the cause of the extension knee
contracture in the maj ority of cases; patella
dislocation also occurs in around 20% of the
patients.
C linical symptoms. At every flex ion of
the knee, the patella slides down from the lat­
eral femoral condyle to the lateral side. Ifknee
flexion is attempted while the examiner man­
ually holds the patella in place to prevent the
lateral luxation, only 3 0-40° of flexion can be
achieved and the muscle is very tense.
Treatment. The patella slides over the
prominent edge of the lateral condyle at every
luxation, and its hyaline surface may therefore
be damaged after a time. Surgery is recom­
mended to elongate the quadriceps tendon, to
incise the lateral capsule and to tighten the
medial capsule.
29.3.2. Recurrent dislocation and
subluxation of the patella
The patella occasionally undergoes dislocation
or subluxation lateral. Fol lowing the acute epi­
sode, swelling and pain are observed. This
disorder occurs in pU berty. It is 3 times more
common among girls than among boys.
Etiopathology. Similarly to habitual dis­
location, this is lateral and presents first as a
traumatic luxation. The history may indicate
and injury, but this is usually not a direct ef­
fect on the patella. The dislocation is induced
by a critical knee movement with distortion
during some activity, jumping or sport. In re­
current cases, the patient knows exactly what
movement causes luxation and tries to avoid
it. The dislocation recurs again with various
incidence, but usually increasingly more com­
mon. It may initially present once or twice a
year, but without a surgical solution it may be­
come a daily event.
An important factor in this disorder is the
loosening of the medial capsule and reti­
naculum. Contributions may also be made by
the following factors : a high patella, a hypo­
plastic lateral femoral condyle or lateral part
of the patella, valgus knee or a lateral position
of the tibial tubercle due to torsion of the leg.
Clinical symptoms. It is not easy to estab­
lish from the history whether the patient origi­
nally suffered a patella dislocation or a
meniscal tear. If the situation recurs the diag­
nosis is easier.
Following an acute incident, the knee is
swollen, and an accumulation of fluid or
blood may be present. After a time, patella
chondromalacia may develop. The medial
edge of the patella is tender, and pressing the
patella in a lateral direction elicits sudden and
severe pam.
X-ray signs. Serial films taken with dif­
ferent flexions of the knee of the prone patient
demonstrate hypoplasia and a tendency to dis­
location.
Treatment. The patient usually arrives
following reduction of the dislocated patella
(or it may have reduced spontaneously). If
blood has accumulated it is evacuated and a
plaster cast is applied. Active quadriceps ex-

ercises are recommended later. Even after
multiple luxation, a conservative method can
be attempted, especially active quadriceps
training. A special orthosis can be prescribed,
enabling the patient to play sports . If the com­
plaints persist, surgical methods follow.
Many procedures have been published, the es­
sence being incision of the lateral retina­
culum, tightening the medial and correcting
the path of the ligamentum patellae by trans­
posing the tibial tubercle medially.
29.3.3. Luxatio patellae congenita
This disorder is well differentiated from
the above form, since it is present at birth. The
patella lies on the lateral part of the femoral
condyle (Fig. 29.2.), and is not reduced during
motion; in fact, it is often irreducible even
manually. The patella is hypoplastic, and the
ligament is shortened.
The knee is in valgus and externally ro­
tated.
Swift operation is warranted, but it is not
easy to reduce and fix the patella even surgi­
cally.
Fig. 29.2.
Congenital patella luxation with characteristic
lateralized patella, and shallow patellar groove on
the femoral surface.
29.4. Developmental disorders of
the menisci
� Discoid meniscus
The embryonic meniscus has a discoid shape.
If this persists after birth, the condition is
called discoid meniscus: instead of the normal
2 9 . D i s o r d e r s of t h e k n e e � 3 79
sickle shape, the meniscus resembles a disc. In
95% of the cases, the lateral is involved.
Clinical symptoms. Discoid meniscus is
detected among toddlers. At the beginning of
knee flexion and at the end of extension, a typ­
ical clicking sound is heard, with a few de­
grees of valgus - varus or external - internal
rotation movement. This condition is more
vulnerable than with the normal meniscus,
and it is symptomatic if a central tear appears
after minor trauma or distortion.
Treatment. The weak, thin, tom central
part is excised via arthroscopy, leaving the pe­
ripheral part intact.
29.5. Developmental disorders of
the tibia and fibula
29.5. 1. Tibia, fibula aplasia,
hypoplasia
Tibia hypoplasia results in a shorter leg.
Partial or total aplasia often presents together
with other developmental disorders (see
Chapter 1 5) . The leg is usually bent to varus,
while the foot is in equinovarus because of the
missing medial ankle.
29.5.2. Congenital tibia
pseudarthrosis
At birth, the leg may be bent in an anteriorly
convex shape. Fradure o r pseudoarthrosis de­
velops later, usual ly in childhood. The typical
findings are the angulation between the mid­
d le and distal third of the leg and occasional
pathological movements.
Clinical symptoms. Curvature with fron­
tal and lateral angulation is seen between the
middle and distal third of the leg. If fracture
has occurred, acute angulation and irregular
movements are detected.
The X-ray picture shows the described
bend of the tibia with sclerosis or cystic zones,
380 � 2 9 . D i s o r d e r s of t h e k n e e

o r interruption of the bone integrity. The fib­

ula may be intact, curved or also pseudo­
arthrotic.
The etiology is unclear. It often accompa­
nies neurofibromatosis (see Chapter 1 3) . Cafe
au lait spots are then found on the skin, and
neurofibromatosis nodules. It may also be as­
sociated with fibrous dysplasia.
Spontaneous fracture often occurs at the
location of the curve detected after birth. The
tendency of pseudoarthrosis to heal is ex­
tremely poor. Surgery (possibly repeated)
succeeds. Otherwise, a caliper splint is pre­
scribed.

29.6. Axial deformities

29.6. 1. Genu varum - crus varum Fig. 29.3.

This deformity i s characterized b y the medially
convex angle enclosed in the frontal plane
between the axes of the femur and the leg
(Fig. 29.3.).
Genu varum; the loading axis of the lower limb is
displaced from the medial part of the knee in the
direction of the medial line of the body; the medial
knee compartment is overloaded.

- Medial damage to the tibial proximal or

Symptoms. The distance between the me­
dial surfaces of the knees, which is normally a
few centimeters, may be greatly increased.
The condition may be present unilaterally, but
more often it is bilateral, giving rise to
bowleg.
Incidence. This situation is normal in in­
fants and toddlers, and gradually turns to a
mild valgus position (knock knee) by the age
of 8- 1 0 years. Varus deformities that persist
after this age and progress are considered to
need correction. The disorder appears in both
genders, but is more common among boys.
Etiopathology. Varus deformities may be
idiopathic or of known origin. A number of
conditions may lead to varus knee :
- Metabolic bone disease (the weakened,
occasionally soft bone bends under a
load), e.g. rickets, fibrous dysplasia,
Paget ' s disease or osteomalacia.
- Congenital connective tissue weakness,
or loose ligaments
- Tibia vara epiphysarea
femoral distal growth plate
- Malunited femur or tibia fractures with
axial deformity
- Compensation (as a consequence of an
abduction hip contracture)
- Medial osteoarthritis
Clinical symptoms. The deformity is usu­
ally bilateral. It is symptomless in childhood,
and the parents take the child to the doctor
merely because of the deformity. Later the
leading symptoms are quickly developing
tiredness, pain after loading, and an uneven
gait. Common further signs are internal rota­
tion of the leg and flatfoot.
In untreated cases, overloading of the me­
dial part of the knee joint (Fig. 29.3.) results in
early arthrosis and instability. In severe cases,
the lateral ligaments may become distended,
and the knee will wobble.
X-ray signs. The extent and the punctum
maximum of the axial deformity are best visu­
alized on a film of the entire lower limb. In

later stages the X-ray signs of osteoarthritis
are seen in the medial joint surfaces (narrow­
ing of the joint space, marginal osteophytes,
sclerosis, etc.) (Fig. 29.3.).
Treatment. The etiology should be identi­
fied and the deformity should be prevented
(e.g. recognition and treatment of rickets,
osteomalacia and osteoporosis). A mild defor-
a
b distance between the medial ankles with the
Fig. 29.4.
Bilateral severe Blount disease with varus deformity.
a: Clinical picture. b: X- ray image
2 9 . D i s o r d e r s of t h e k n e e � 38 1
mity detected in a toddler does not require
treatment. At a later age, the marked varus is
presumed to be a prearthrotic factor, warrant­
ing a surgical solution. Corrective osteotomy
is performed at the metaphysis near the
punctum maximum of the deformity, most of­
ten on the tibia.
29.6.2. Tibia vara epiphysarea
(Blount's disease)
This is a relatively uncommon disease,
which involves dysostosis of the proximal
epiphysis metaphysis of the tibia, resulting in
a varus knee deformity. The rapidly progress­
ing varus deformity usually starts in child­
hood, occasionally around the age of 1 0 years
(Fig. 29.4. a, b). The treatment in tall children
is temporary closure of the distal growth plate
with staples (epiphyseodesis), or usually
valgus tibia osteotomy. Because of the pro­
gression of the condition the operation must
sometimes be repeated.
29.6.3. Genu valgum - crus valgum
I n cases o f valgus knee, the axis o f the thigh
and the axis of the leg enclose a lateral con­
vex angle i n the frontal plane (Fig. 29.S.). The
knees closed together is considerably in­
creased. In childhood, this deformity is often
physiological, and at the age of 8- 10 sponta­
neous correction is expected.
Etiopathoiogy. The condition may be of
idiopathic or known origin. The causes can be
similar to those of varus knee, but resulting in
the opposite deformity:
- Metabolic bone disease e.g. rickets (but
this usually causes a varus deformity).
- Dyschondroplasia, and multiple exo­
stosis.
- Connective tissue weakness, and loose
ligaments.
- Damage to the lateral part of the distal
femoral growth plate (osteomyelitis, tu­
mor, trauma or injuries).
382 � 2 9 . D i s o r d e r s of t h e k n e e
- Malunited femur o r tibia fractures with
an axial deformity.
- Muscle paralysis.
- Loosening following medial collateral
ligament injuries.
- Compensation (as a consequence of an
adduction hip contracture) .
Clinical symptoms. When the knees are
closed together, the medial ankles can not
come into contact, and the patella is turned
upward. This is recorded in centimeters. The
valgus is the most common deformity of the
knee joint; it is often associated with
recurvatum.
In toddlers, this situation is not considered
abnormal if the distance between the medial
ankles is not more than 5 cm; it corrects spon­
taneously by the age of 8- 1 0 years. Valgus
knee is usually associated with mild valgus
heel, and the parents take the child to the doc­
tor not because of any complaints, but be­
cause of the ugly gait. In these cases, only a
supination heel elevation and foot exercises
are needed.
In adults, a pronounced valgus deformity
is a prearthrotic factor (Fig. 29.5.). Early
-' UIII�....lI..Q9&
Fig. 29.5.
I n valgus deformity, the loading axis is lateralized,
and the lateral compartment of the joint is there­
fore overloaded.
chondropathy and osteoarthritis can develop,
with knee instability, leading to serious com­
plaints.
X-ray signs. The extent of the deformity is
well visible on weight-bearing films. In a later
stage, evidence of osteoarthritis is seen in the
lateral compartment.
Treatment. It is important to determine
the cause and to treat this condition early, be­
fore the deformity develops. In more serious
cases, surgery is advised: If the growth plate
persists, temporary epiphyseodesis with sta­
ples in the medial side (Blount ' s procedure) is
possible. Corrective varus osteotomy can be
carried out on either the femur or the tibia, as
described in the Chapter 29.6. 1 .
29.6.4. Torsion of the tibia
Varus and inward torsion of the tibia is a
common condition under the age of 1 year,
and later it can improve spontaneously (see
Chapter 1 2) .
29.7. Aseptic osteochondroses
around the knee
29.7. 1. Osteochondritis dissecans
genus
This entity i nvolves aseptic bone necrosis
which occurs in the joints. In the majority of
cases it affects the lateral part of the medial
tibial condyle. The necrotized cartilage - bone
piece may be detached, forming a loose body
and leaving a crater-like hollow 1-2 cm in di­
ameter in its place. It is observed in young
adults, i n half of the cases bilateral ly.
Etiopathology. The cause is not clear:
roles may be played by constitutional factors,
repeated micro injuries (athletes), circulation
anomalies, and the closure of end-arteries.
Clinical symptoms. Initially mild pain
and swelling are experienced, the flex ion may
 29. D i s o r d e r s of the k n e e � 383

be restricted, and the limit may be painful. If

the necrotized osteochondral piece is partially
detached, the condition may mimic a meniscal
tear; if it is fully detached, forming a loose
body, it may cause a joint block or become
palpable.

X-ray signs. The anteroposterior views of

the knee, or rather the special views, show a
bean-shaped bony body measuring about 3
cm on the lateral side of the femoral condyle.

Treatment. If the disorder is in an early

stage, the extent is limited and the joint con­
gruence is intact (MRl scan), the cessation of
overloading (sport) may result in spontaneous
healing, especially in adolescents. If partial
detachment occurs, the surface of the crater is
refreshed, the necrotized parts are removed
and the fragment is fixed with absorbable
"pins" in an arthroscopic procedure. If the
crater is too large and predominantly involves
the weight-bearing surface, autologous or ho­
mologous grafting may be indicated.

29.7.2. Osteochondritis of the tibial

tubercle (Schlatter-Osgood's
disease)

Schlatter-Osgood's disease is juvenile

osteochondrosis of the tibial tubercle. It is
most common in the age range 1 1- 16 years
and affects both boys and girls. Schlatter-Osgood disease.
a. The lateral X-ray picture shows the tibial tubercle
as an independent bony fragment. b. Characteristic
Etiopathology. This is aseptic bone ne­ prominent tu bercle when the knee is bent.
crosis of the tibial tubercle.

Clinical symptoms. The complaints often
start following heavy loading or after direct
pressure on the tibial tubercle. Forced knee
extension against resistance may elicit pain. A
swelling is seen over the tibial tubercle, but
other signs of inflammation are missing.
X-ray signs. The lateral view of the knee
the apophysis of the tibia is fragmented, scle­
rotic and protruding (Fig. 29.6. a, b).
Treatment. The ossification disorder usu­
ally heals in 2 years. It responds well to con­
servative treatment, several weeks or months
of rest, avoidance of physical exercises, occa­
sionally cold compress, creams, and non­
steroidal anti-inflammatory drug administra­
tion. In stubborn cases, a plaster cast up to the
groin may be applied for 4 weeks. The com­
plaint may recur up to the end of growth at
around 1 8-2 1 years, when the tibial tubercle
384 � 2 9 . D i s o r d e rs of t h e knee

unites with the tibia. If the tibial tubercle pro­
trudes to a maj or extent· and disturbs the pa­
tient during kneeling, the persisting bone frag­
ment is excised.
29.7.3. Adu lt aseptic femur condyle
necrosis (Ahlbiick's disease)
Spontaneous aseptic osteonecrosis usually
starts after the age of 60 years on the load­
bearing area of the medial condyle of the fe­
mur, less frequently of the tibia . .

Fig. 29.7.
A 71-year-old female with necrosis of the right medial
femoral condyle (M. Ahlbiick disease).
a: Anteroposterior X-ray picture; the joint su rface of the
medial femoral condyle is flattened and cracked (arrow).
b: MR shows the intraosseal extent of the necrosis.
c: I ntraoperative picture: the almost separated femoral
su rface is visible; the cartilage of the knee is intact else­
where.
d: Unicondylar prosthesis inserted (immediate postopera­
tive film).

Etiopathogenesis. The reason is unclear.
It is usually unilateral, and the extent is al­
ways larger than in juvenile osteochondritis. It
often results in varus knee.
Symptoms. The knee becomes progres­
sively more painful, and the range of move­
ment is extremely restricted. The diagnosis is
established by X-ray examination.
The surface of the condyle is uneven, later
becoming flat. The MRI scan illustrates the
extent of the defect (Fig. 29.7. a-d) .
Treatment. Condyle necrosis in the el­
derly warrants sledge prosthesis insertion.
29.8. Knee contractures
Flexion may be restricted (extension
contracture), or extension may be restricted
(flexion contractu re), or both may be limited,
for many reasons. The contracture may be
due to a muscu lar disorder, an innervation
anomaly or shrinkage of the capsule, the liga­
ments or the skin. A special form is postoper­
ative arthrofibrosis (fibrous a nkylosis).
Etiopathology:
- Inflammation and swelling
- Trauma and distortion
- Neuromuscular diseases (Heine-Medin
disease, or infantile cerebral paresis)
- Congenital anomalies (knee dislocation)
- Bums or scars after operations or
InJunes
- Arthrosis
- Tumors adj acent to j oints
- Iatrogenic or repeated knee operations
Flexion contractures and spasms of the
knee muscles are most common in cases of in­
fantile cerebral palsy
Extension contractures of the knee are dis­
cussed separately.
Etiopathology. The cause of its congeni­
tal form is unknown; it often appears with
other developmental abnormalities. The
2 9 . D i s o r d e r s of t h e k n e e � 385
flexion is limited t o 1 0-20°. The quadriceps
femoris muscle is fully or partially shortened.
The most common cause of the acquired form
is the scarring that follows antibiotic inj ec­
tions into the thigh, or the m. vastus lateralis
or intermedius in infancy.
Clinical symptoms. The child walks with
a stiff knee, and the hip is therefore abducted
and externally rotated. The child is unable to
squat, and keeps the knee extended while sit­
ting. The thigh circumference is less than on
the other side, and the quadriceps muscle is
atrophic, and scarred.
One special form of this extension
contracture is isolated shortening of the m.
rectus femoris, which can be congenital or a
consequence of inj ections. The symptoms are
similar to those mentioned above. This mus­
cle bridges over two joints, and the physical
findings are therefore different when the hip
flexed or extended. If the child is supine, the
knee flexion seems to be complete with the
hip flexed. In the prone position with the hip
extended the knee flexion may be only 5 - 1 0°,
and further flexion is possible only with
flexion of the hip.
Treatment is surgical, the tendinous part
of the hip flexor/knee extensor muscles being
lengthened above the knee.
29.9. Fluid accumulation (effusion)
i n the knee
This condition causes swelling of the
suprapatellar pouch, patella ballottement and
restriction of the range of movement. The
fluid may be serous (hydrops), bloody
(hemarthros) or inflammatory (serous, sero­
fibrinous or purulent) .
Genicular synovitis may be due to:
- bacterial infection (via direct or
hematogenous spread)
- viral infection
- specific conditions (e.g. tuberculosis)
386 � 2 9 . D i s o r d e r s of t h e k n e e
Genicular abacterial synovitis may b e due
to:
- postbacterial allergic processes (tran­
sitory arthritis)
- rheumatic diseases (rheumatoid
arthritis, etc. )
- metabolic diseases (gout, ochronosis,
pseudo-gout, etc.)
- osteoarthritis
- post-traumatic joint deformities
- neural diseases (syringomyelia, neuro-
syphilis, etc. )
- tumors adj acent t o j oints
Hemarthros may be due to:
- hemophilia (spontaneous bleeding or
microtrauma)
- hemorrhagic diathesis
- trauma (fractures involving the j oint or
cruciate ligament and meniscus rup­
tures)
- a tumor or tumor-like condition in­
vading the j oint (e.g. villonodular
synovitis ) .
Clinical symptoms. In acute synovitis,
the knee is swollen and hot, and the skin is
red. The suprapatellar pouch is swollen, and
patella ballottement is detected. Diffuse ten­
derness and strong pain are present. The range
of movement is severely restricted; the joint
may be blocked in mid-position.
In chronic synovitis, the severe pulsating
pain and the hot skin may be missing. The
joint may be "dry", when patella ballottement
is absent, but the knee is swollen due to the
thickened synovial membrane, and the
parapatellar fossa is smoothed. In the form
where considerable amounts of synovial fluid
are secreted, the patella ballottement is pres­
ent, the amount of fluid may reach 1 50-200
ml. The range of movement is moderately re­
stricted.
The X-ray signs are not characteristic; the
j oint space may be widened, but there is no di­
rect evidence relating to the basic disease.
MRI and ultrasonography may give more in­
formation (loose body, tumor, etc .) .
Treatment. The exact diagnosis and the
underlying condition must be established. In
cases of post-traumatic hemarthros (if this is
proven by joint puncture), acute knee
arthroscopy may be performed. Diagnostic
joint puncture is usually considered neces­
sary. The aspirated fluid is sent for laboratory
tests (to detect crystals, proteins, leucocytes,
fungi, LE cytes, bacteria, etc.). The local
treatment depends on the source illness, e.g.
conservative treatment is preferred in primary
osteoarthritis, whereas in rheumatoid arthri­
tis, if conservative treatment fails, syno­
vectomy is advocated. If pus is evacuated via
the puncture, the subsequent therapy is in ac­
cordance with the treatment of acute or
chronic bacterial synovitis outlined in Chap­
ter 1 9. 3 . 5 . 1 .
29.10. Cl'.sts in the knee region
Cysts are common in the knee region.
They may communicate with the joint, have a
synovial lining and contain thickened
synovial fluid.
29. 10. 1. Ganglion
This type of cyst is common in the knee re­
gion, and also in the wrist, ankle and foot. It
originates from the tendinous parts of the
extensor muscles attached to the fibula head,
with a size between 4 and 7 cm, and is easy to
palpate. Exposure reveals a membranous cyst
with a yellow, gelatinous content. Surgical re­
moval is advised, but recurrence is common.
29.10.2. Meniscus cyst
This is a degenerative cyst, ongmating
from the meniscus; it is 7 times more common
laterally.
Clinical symptoms. In young adults a
thick nodule may be palpated at the lateral
j oint line, possible be accompanied by im­
pingement, a click, and a blocking sign, char-

acterized by a meniscal tear. It may be ob­
served at the edge of the meniscus or within
the meniscus, when it is not palpable, then ne­
cessitating arthroscopy or an MRI scan.
Treatment. Surgical removal of the me­
niscus cyst is recommended, in some cases
with the entire meniscus. The residual menis­
cus may result in recurrence.
29.10.3. Popliteal cyst (Baker's cyst)
This cyst, containing thickened synovial fluid,
appears in the popliteal fossa on t he basis of
processes causing chronic synovitis in the
knee joints of adu lts. It is usually connected
with the joint. Both genders are affected. It is
also frequent in chi ldren. The cause is un­
known.
Etiopathology. This is not clear. Weak­
ness of the posterior capsule is assumed, for
the synovial membrane may protrude by this
route. On the other hand, chronic synovitis is
considered to play a role, and based on an
intra-articular cause (osteoarthritis, loose
body, tom meniscus, etc.). It is often seen in
rheumatoid arthritis.
Clinical symptoms. At the outset the
complaints are vague. Squatting is difficult,
a b
a:
b: Intraoperative picture of the cyst, with a membranous wall and a transparent content.
2 9 . D i s o r d e r s of t h e k n e e � 387
and some radiating pain may appear. The cyst
"expands" in osteoarthritis, following an
overload, longer weight-bearing, and be­
comes palpable in the popliteal fossa (Fig.
29-8. a, b); during rest it decreases in size.
The cyst is connected with the knee joint and
an increase in knee j oint pressure pumps
synovial fluid into the cyst. Later, the connec­
tion may become unidirectional and work as a
vent, when no fluid may return from the cyst
to the joint space. The size of the cyst may be
constant or increasing. It must be differenti­
ated from aneurysms and tumors by ultra­
sonography, which is easy to perform and has
95% surety. In the event of doubt, an MR scan
is performed.
Treatment. The cause of chronic syno­
vitis of the knee must be eliminated first. If
there are complaints, surgical removal is ad­
vised. In adults, recurrence is common, espe­
cially if the chronic synovitis of the knee per­
sists.
29. 10.4. Bursitis praepatellaris
This is chronic inflammation of the
prepatellar bursa, usually due to excessive
mechanical loading (kneeling occupations,
mason, or parquet layers). The inflammation
may be acute, chronic, serous or rarely puru-
388 � 2 9 . D i s o r d e r s of the k n e e
lent. The skin over the kneecap is hot, red, ten­
der and hyperkeratotic; the size of the swell­
ing varies between 7 and 1 0 cm.
Treatment. In acute cases, the limb is put
at rest, and a cold compress is applied, which
together with aspiration, may heal the pro­
cess. In purulent bursitis, incision, drainage
and surgical excision may be the solution.
29. 1 1. 1 . Col lateral ligament injuries
Etiopathology. These injuries may be
classified in terms of the extent of the force
and the damage: overstretching of the liga­
ment which does not result in instability
(sprain or distortion); or partial or total rup­
ture. The latter may involve the ligament itself
or the bony attachment can be broken out. In
ruptures, the damage usually disrupts differ­
ent layers of the ligament at different heights .
A purely lateral force may result in a
contralateral collateral ligament rupture to­
gether with tearing of the adj acent capsule,
causing simple or uniplanar moderate knee in­
stability. More common is the complex rota­
tional knee instability that arises when the
force acts in several planes . Flexion - valgus ­
external rotation injuries lead to anteromedial
instability, usually with simultaneous tearing
of the medial collateral ligament, anterior
cruciate and medial meniscus ("unhappy
triad ''), sometimes associated with tearing of
the capsule and the posterior oblique liga­
ment. Less frequent are the flexion - varus -
internal rotation injuries, when the lateral cap­
sule, lateral collateral and anterior cruciate
ligament are damaged, with consequent
anterolateral knee instability.
Hyperextensive forces or injuries forcing
the head of the tibia dorsally cause posterior
instabilities, which may likewise be combined
with medial, lateral and rotation components,
as above.
In extremely high-energy injuries (e.g. a
traumatic knee dislocation), the complexity is
even greater: the anterior and posterior com­
ponents are combined with lateral elements,
possibly aggravated by bone, vascular, neural
and tendon injuries.
Clinical symptoms. In fresh injuries pain,
swelling, hemorrhage and direct tenderness to
palpation are detected along the path and over
the attachment of the collateral ligament. Iso­
lated collateral ligament rupture can not be
identified with the knee fully extended, since
the strong posterior capsule partially takes
over the function of the collateral ligament.
Accordingly, the test for the integrity of the
collateral ligaments is performed at 1 0-200 of
flexion, with valgus stress for the medial, and
the varus test for the lateral collateral liga­
ment. In the event of damage, mild ' opening'
is detected in response to the above stresses,
denoted by , +, ++ or +++, depending on the
-
extent of the ill-defined movement. In cases
of isolated collateral ligament tears the intact
cruciate limits the mild ' opening' to + or ++ ;
in cases of +++ ' opening ' , therefore, cruciate
and oblique posterior ligament ruptures are
also present. To substantiate these findings,
X-ray examinations can be performed, the
joint being hold in a stressed position (the
pain-induced muscle spasm may hide the in­
stability) . In cases of doubt, ultrasonography
and MRI can be added to confirm the diagno­
sis.
Treatment. Isolated tears of the collateral
ligament can be treated conservatively be­
cause of the excellent blood supply due to the
location of this ligament. Bone disruptions are
treated surgically.
29. 1 1.2. Chronic collateral ligament
insufficiency
Etiopathology. This is exceptional as iso­
lated damage; it usually occurs following the
partial healing of a complex ligament rupture.
In the majority of cases, it is an element of an
anteromedial or posterolateral instability. The
following additional factors may lead to a
chronic knee collateral ligament insuffi­
ciency:

- a marked varus or valgus deformity
- a marked hip abduction or adduction
contracture as a compensatory symptom
- rheumatologic diseases
- arthrosis
Clinical symptoms. An isolated collateral
ligament insufficiency elicits a moderate
varus or valgus instability feeling, which pres­
ents during a physical load, sports activity or
walking on uneven ground as an occasional
knee distortion. The main complaint is the
feeling of a lack of stability. Stress X-ray ex­
amination, ultrasonography or MRI may con­
firm the diagnosis.
Treatment. The treatment depends on the
degree of instability and naturally the associ­
ated injuries. Ifthe lateral instability is signifi­
cant, surgical treatment of the associated inj u­
ries (anterior or posterior cruciate ligament re­
placement, and replacement of the collateral
ligament with autologous tendon fixed via
bone holes) can be considered. The latter is a
very delicate task, how with regard to how to
position the graft isometrically. The best re­
sults are achieved laterally by cutting the bi­
ceps tendon in half, and medially by using one
of the hamstrings.
Fig. 29.9.
Outline of the anterior cruciate ligament rupture.
2 9 . D i s o r d e r s of t h e k n e e � 389
29. 1 1.3. Cruciate ligament inju ries
These lesions are o bserved following a
trauma; they lead mainly to various extents of
sagittal i nstability of the knee.
Etiopathology. Cruciate injuries occur
from the action of complex forces exerted in
the sagittal plane; they may be isolated or as­
sociated with injuries of other components
(Fig. 29.9.) . Injury to the anterior cruciate lig­
ament (ACL) is 1 0 times more common than
one to the posterior cruciate ligament (PCL).
The ACL usually undergoes tearing in the
middle third of the ligament, and the ends are
fringed, resembling a broom. Bony abruption
is distal and more frequent in children.
Clinical symptoms. These include pain,
swelling, blood accumulation and instability,
which may cause an inability to bear weight.
In fresh cases, locking of the joint or an exten­
sion deficit may also occur, caused by the im­
pinged ligament parts. In chronic ACL or
PCL inj uries, the patient often describe an in­
stability feeling, even on horizontal ground
(the knee occasionally gives way), but it is
more pronounced on uneven ground when the
direction is changed.
\
Fig. 29. 10.
Drawer sign.
390 � 2 9 . D i s o r d e rs of t h e knee

In chronic cases, there are only slight signs
of instability. The sagittal stability of the knee
is characterized by the drawer sign, which is
limited in fresh cases in muscular patients be­
cause of the pain due to the fixing ability of
the active stabilizers. In cases of an ACL tear,
the head of the tibia can be moved anteriorly
on the femoral condyles to various extents
with the knee flexed at 90°. This is the anterior
drawer sign, and its extent is denoted simi­
larly to the lateral instability, on a +, ++ and
+++ scale (Fig. 29.1 0.). In PCL injuries in the
same flexed position the head of the tibia may
be displaced posteriorly because of the rup­
ture. From this position, it may be reduced to
normal and redisplaced posteriorly (posterior
drawer sign). This may be pseudo-positive
due to the general joint laxity: a comparative
assessment with the other knee is advised.
Ultrasonography may suggest cruciate lig­
ament injuries; the MRI almost certainly
proves them.
therefore degenerate faster and severe carti­
lage wear may take place within a few years.
The main role is definitely played by
autologous grafts. Occasionally (especially in
revisions), allografts may be used, but the ear­
lier popular plastic ligaments are no longer
recommended. Ten years ago, the bone -
patellar tendon - bone graft was applied al­
most exclusively, whereas the positive experi­
ence has resulted in the replacement being
carried out nowadays with four times folded
semitendinous tendon, twice folded se­
mintendinous + gracilis tendon, or rarely
quadriceps tendon grafts (Fig. 29. 1 1 .). During
this procedure, which is usually performed

Treatment. Acute reconstruction of the

ligament is not advised, even in fresh cases,
because of the frequent failures. The only ex­
ception is distal bony abruption of the liga­
ments, which is currently treated with anchor­
ing of the abrupted bone and the attached liga­
ment. In the other cases, the first procedure is us grafts: the semitendinosus tendon is
arthroscopy (treatment of other intra-articular seen above, and the bone - patella tendon - bone
injuries, meniscal tears and hyaline surface
damage, and careful limited resection of the
tom, impinging fibers of the cruciate) ; then
conservative therapy is advised. Satisfactory
stability may be achieved by training of the
thigh muscles. When long-term conservative
treatment does not terminate the patient ' s
complaints of instability and a limited ability
to bear weight, if the patient is younger than
50, ACL or PCL replacement may be per­
formed.
The improving results of arthroscopic lig­
ament reconstruction have led to these proce­
dures becoming increasingly popular all over
the world. This is supported by the observa­
tion that injured patients who have no instabil­ , - -..,- - � - ---,
ity feeling may exhibit a certain "micro­ Arthroscopic picture 1 year after insertion of a four
instability", and ACL or PCL-injured knees times folded semitendinosus tendon graft.

arthroscopically, the most important goal is
the isometric position and stable fixation of
the graft. To achieve this, interference screws
are utilized in bone - patellar tendon - bone
grafts, and (semintendinous + gracilis tendon)
plates, staples, anchors and other special fix­
ing elements are used in cases involving ten­
don use (Fig. 29.1 2.).
29. 1 1.5. Meniscal tear
These are very common i nj u ries of the knee.
The meniscus becomes trapped between the
femoral and tibial condyles and sustains rup­
tures varying in extent and loca lization.
Etiopathology. The mechanism of the in­
jury is usually a rotational force : while the leg
Normal menisci (a). Longitudinal fissu re (b) and
bucket-handle tear in the medial meniscus (c).
2 9 . D i s o r d e r s of t h e k n e e � 39 1
is fixed, the femur is rotated inward or out­
ward. The femoral condyles push the menisci
in front of them, and the meniscal edges may
become trapped between the contacting joint
surfaces. Predisposing factors are earlier or
concomitant ligament injuries (most often the
ACL), and axial deformities of the knee (genu
varum, valgum or recurvatum), and the exten­
sively loose joints.
In cases of osteoarthritis or rheumatologic
conditions and in the elderly, degenerative
changes of the menisci may result in frequent
tears as a result of the repeated microtraurnas .
I n the majority of cases (90%) the medial me­
niscus is involved, which is the less mobile, as
it is anchored to the medial capsule and collat­
eral ligament. The tear takes place most often
at the posterior horn and in the middle part of
the meniscus (Fig. 29. 13. and 29.1 4.).
Regardless of whether if the tear is lobular
or bucket-handle-shaped, its interposition be­
tween the loading surfaces causes gradual
hyaline cartilage damage.
Clinical symptoms. In a typical case, the
patient has a history of sports injury or
trauma. Impingement during physiological
motion relates to previous degeneration. The
injury causes strong pain and is accompanied
by the accumulation of serous j oint fluid.
Blood accumulates when the meniscus is tom
Arthr(),rnnir picture of a bucket-handle tear and
impingement of the medial meniscus.
392 � 29. D i s o rders of the knee
a t the base. One of the most important symp­
toms, which is often missing, is the joint lock
due to the impingement of the tom part of the
meniscus. In chronic cases, very little or no
fluid may be accumulated, and the patient ex­
periences repeated locking and painful clicks
in response to certain movements . The joint
space is tender to direct pressure. In cases of
suspected meniscal injury, the ligaments of
the knee must also be assessed.
X-ray signs. The ultrasonographic and
MRI images reveal the location of the
meniscal tear with relatively high accuracy.
Treatment. Resection and particularly
full excision of the menisci predisposes to
osteoarthritis . The basic principle of the surgi­
cal treatment is to remove only the irreparably
tom parts. Unfortunately, partial resection of
the meniscus is a necessary intervention in
most cases. At present the meniscus operation
is almost exclusively performed arthro­
scopically.
In young individuals, fresh and not too
long tears may be reconstructed if they are lo­
calized near the base (this meniscus substance
has a blood supply); this involves arthroscop­
ic resuturing of the meniscus. Reinsertion of
the meniscus is possible only in a small pro­
portion of the cases and resection is usually
considered necessary. The practice in these
cases is removal of the tom, impinging parts
of the meniscus and smoothing of their base
with a shaver. If there are extensive ruptures
near to the base in youngsters, and the j oint is
otherwise intact, the expected osteoarthritis
may be prevented by implantation of a fresh
meniscus allograft.
� Cartilage damage
The sliding surfaces of the knee j oint are
covered with hyaline cartilage, which has ex­
cellent mechanical and weight distribution
qualities, but a very poor disposition to regen­
erate (Chapter 1 9.8).
There are zones of this surface which are
heavily loaded, and others which bear less
weight. The maximal load affects the central
areas of the femoral and tibial condyles; the
peripheral areas which are covered by the
menisci and the parts next to the intercondylar
eminences are loaded with somewhat less
weight.
The 4-5 -mm-thick hyaline cartilage is un­
changed structure and is an excellent buffer: it
ensures movement with almost no friction for
many decades. On the other hand, if this struc­
ture is damaged, it has a very poor disposition
to heal spontaneously.
29.13. Chondromalacia patellae
This d isorder (involving early degenerative
changes) has long been known, its name char­
acteristica l ly meaning softening of the carti­
lage su rface of the kneeca p.
Etiopathology. This clinical picture is
only the introductory stage to a progressive
degenerative process: the same clinical diag­
nosis applies to the more advanced stage of
patella cartilage damage. For grading of the
cartilage damage, the Outerbridge classifica­
tion is the most popular.
In stage I, the cartilage softens, but its surface
remains smooth; its color may differ from
that of the adjacent.
In stage 11, the surface becomes "hairy", i.e.
covered with small villi, and the involved
area loses its shine. Small superficial fis­
sures may be present.
In stage 111 the degeneration progresses, and
the fissures become numerous and spread
through the entire thickness to the bone.
Stage IV is characterized by large defects ex­
tending deep to the bone, resulting from
the action of shearing forces on the dam­
aged, cracked cartilage surface.
The latter two stages are regarded as the
initial stages of osteoarthritis (see Chapter
1 9 . 8 .).
Biomechanical factors often play a role in
the development of patella chondromalacia.
In a large proportion of the cases, formal de-

Fig. 29.15.
Patellofemoral arthrosis with lateral hyperpression
syndrome.
fects are present: incongruence of the
patellotrochleal junction, anomalies of the
traction route of the quadriceps (increased Q
angle), and a high or low position of the knee­
cap (patella alta or patella baj a), with repeated
luxation or subluxation as a consequence of
the former factors, or patellofemoral hyper­
pressure (Fig. 29.1 5.). Exposure of the knee­
cap to a direct or indirect trauma leads to
hyaline contusion, contusion and hematoma
of the subchondral bone or an occasional pa­
tella fracture. In a large number of cases, none
of these factors can be identified; these cases
are classified as idiopathic. This is often the
situation with young schoolgirls who do not
play any sports, when severe hypotrophy of
the vastus medialis muscle is observed.
Clinical symptoms. Walking up or down
stairs, or standing up from a squatposition
may produce severe retropatellar pain, and
later prolonged flexion without loading may
also cause complaints. A long period of sitting
with the knees flexed can produce complaints
that are known as ' drive-in movie sign ' . Fluid
accumulation is not common. Direct pressure
on the kneecap, tapping or attempted sub­
luxation, either laterally or medially, pro­
vokes pain (the Frond and Zohlen signs). It is
important to assess the angle between the di­
rection of traction of the quadriceps apparatus
and the axis of the patellar ligament
(Q-angle). The mobility and possible ten­
dency of the kneecap to undergo dislocation
must also be assessed.
X-ray signs. X-ray tests play a decisive
role in the identification of etiology. In the lat-
2 9 . D i s o rd e r s of t h e k n e e � 393
era I view, the high o r low position of the knee­
cap, or the relative length of the j oint surface
(the Blackburne or Insall-Salvati indices) may
preconditioning factors. Patella lateralization
too may be visualized in accurate antero­
posterior X-ray images, and the patello­
femoral views taken at 3 0-60 and 90° of
flexion may demonstrate the pathologic
match in the different phases of motion. The
congruency disturbances relating to the shape
anomalies of the patella or the trochlea are of
great importance.
Treatment. Apart from those cases where
there is a severe biomechanical background,
the therapeutic approach is basically conser­
vative. Rest, nonsteroidal anti-inflammatory
drugs, various physiotherapeutic processes
(iontophoresis, ultrasonographic treatment,
selective current stimulation of the vastus
medialis, etc.) and well-selected exercises
play a deciding role. The most important fea­
tures are the strengthening of the vastus
medialis and of the entire quadriceps muscle,
and proprioceptive exercises. Underwater ex­
ercises and bicycling are useful supplements.
If the conservative attempts fail, or if a
well-defined biomechanical cause is proven,
operative treatment may be considered. The
most common procedure is minimally inva­
sive arthroscopy, when the surface changes
exceeding stage 1 1 are smoothed with a
shaver, and the patellofemoral pressure condi­
tions in cases of hyperpression are advanta­
geously affected by incision of the lateral
retinaculum. In stubborn or severe cases, the
tibial tubercle may be ventralized to correct
the direction of patellofemoral traction.
29. 14. Chondropathies,
osteoarthritis (arthrosis)
of the knee
A lengthy series (many yea rs) of pathologic
events usua l ly occur from the mild cartilage
damage (chondro pathy) to the end-stage
(osteoarthritis o r a rthrosis of the knee).
394 � 2 9 . D i s o r d e r s of t h e k n e e
I n itially, macroscopic changes take place i n
t h e carti lage, followed b y deformation a n d
destruction o f t h e joint, involving t h e majority
of the adjacent structu res, resulting in a re­
stricted range of movement and pain.
Etiopathology. Primary osteoarthritis of
the knee is the most common process destroy­
ing the large j oints, followed by hip and
shoulder degeneration. In the age group over
65 years, the radiological prevalence is
20-34%, but only 7- 1 1 % require treatment of
their complaints .
The diagnosis is secondary osteoarthritis
of the knee, when the destruction is due to a
previous illness, trauma or biomechanical
anomalies .
The most common factors are:
- instability (rupture of the collateral or
cruciate ligaments, meniscectomy,
capsule tear, or a change in muscular
balance in neuromuscular disorders)
- overweight
- axial anomalies
- meniscal injuries, or loss
- rheumatologic diseases
- inflammatory or metabolic diseases
- overloading
- a post-traumatic state
i n sta bi lity of the knee
/
path ologi ca l movements
1 1
deteri o ration of the j o i n t ca rtilage
Fig. 29.16.
~ osteoart h ritis of knee
Knee i nstability; a vicious circle leading to arthrosis.
- osteochondritis dissecans, or loose joint
bodies
One of the most common causes is knee
j oint instability, when abnormal, pathologic
joint movements occur leading in a vicious
circle to destruction of the cartilage (Fig.
29.1 6.).
The destruction of the cartilage alters the
biomechanical conditions and causes inflam­
matory and reactive changes; the hyaline sub­
stance may disappear, and subchondral scle­
rosis and cysts may develop accompanied by
osteophyte formation in the non-loaded areas
and contractures of the joint capsule.
Clinical symptoms. When the changes in­
volve only the cartilage and a limited area, the
symptoms are said to be moderate. The most
characteristic symptoms are a decrease in
load-bearing, intermittent fluid accumulation,
pain, tenderness of the involved compart­
ment, click and crepitation. Later, the swell­
ing and fluid accumulation become more
common, pain is present even in the resting
period, the range of movement decreases and
the joint gradually becomes deformed. In the
maj ority of cases, the axial anomalies of the
knee joint give rise to a severe varus defor­
mity with flexion contracture.
� u n e q u a l load
P7XT
ool l ", m l l i " m,"' ' " "' " O)" ''''�
i a l defo rm ity

X-ray signs. Anteroposterior X-ray films
in the loaded position provide indirect data on
the thickness of the cartilage of the
weight-bearing surfaces in extension. The
Rosenberg views at 30° of flexion furnish in­
formation on the quality of the posterior parts
of the joint surface. As the condition pro­
gresses, the joint space diminishes or disap­
pears, the subchondral bone undergoes sclero­
sis and peripheral osteophytes are formed.
Treatment. The process of primary
osteoarthritis of the knee usually lasts for
many years from the first complaints to the
end-stage of the joint destruction. During this
period, the symptoms are present with various
intensity, the painful stages of inflammation
alternating with long symptomless periods.
Initially, conservative treatment is advised.
The aim is to decrease the symptoms and
signs and the contractures. Nonsteroidal
anti-inflammatory drugs, muscle relaxants
and painkillers are prescribed, together with
oral chondroprotective medication (glucose­
amine sulfate and chondroitin sui fate ) and
a b
Fig. 29.17.
Closing-type valgus high tibial osteotomy.
a: Preoperative loaded anteroposterior view: mild
varus deformity.
b: Six months following the closing-type valgus high
tibial osteotomy (lateral based wedge removal and
staple fixation).
2 9 . D i s o r d e rs of t h e knee � 395
intra-articular hyaluronic acid. Adjuvant ther­
apy may include cooling, electrotherapy, vari­
ous iontophoreses, the use of ultrasound and
medical spa treatment. The most significant
therapy is movement of the j oint without a
load, swimming and underwater exercises,
the aim of which is to improve the knee func­
tion and resolve the contractures.
The surgical treatment in prearthrotic
chondropathies can be divided into two cate­
gories. One category involves treatment of the
biomechanical factors presumed as prea­
rthrotic factors, while the other deals with the
existing cartilage damage. In the former
group, the correction of mainly varus and
rarely valgus axial deformities is of great im­
portance.
The varus deformity occurs chiefly on the
tibia. The correction is most often a clos­
ing-type high valgus tibia osteotomy with re­
moval of the laterally based wedge proximal
to the tibial tubercle (Fig. 29.17. a, b); more
rarely, a varus osteotomy is carried out in the
same area with the opening of a medial wedge
(Fig. 29. 1 8. a, b). Apart from these tech-
a b
���:;:;:" valgus high tibial osteotomy.
a. preoperative loaded a nteroposterior view: mild
varus deformity.
b. Six months following the high tibial osteotomy,
the axis is good and the osteotomy has healed.
396 � 29. Diso rders of the k n e e

Fig. 29.19.
"Microfracture" technique: arthroscopic picture.

niques, dome-shaped or sphere osteotomies

are also performed, allowing minimal rota­
tional correction. For the treatment of carti­
lage damage, arthroscopic debridement and
lavage may be of value, but only for short pe­
riods. The sliding surface reconstruction of
limited, local cartilage defects (hyaline and
osteochondral defects) is attempted by one of Fig. 29.20.
the surface reconstruction techniques, such as Open mosaic plasty on the lateral femoral condyle.
Pridie ' s procedure, abrasive arthroplasty or 2
a: 4 cm bony defect caused by osteochondritis
mosaic plasty. Via small holes made with a b: the defect is filled with grafts 4.5 mm in diameter.
sharp surgical awl, radial fissures are created
in the bone, forming an ideal attachment sur­
face for the regeneratum. The most important
elements of the postsurgical aftercare are 4-8
weeks of movements without weight-bearing
and a further 2-4 weeks of partial weight­
bearing. The popular procedure of ' micro­
fracture' is performed arthroscopically, even
in cases of multiple degenerative cartilage de­
fects (Fig. 29.1 9.). In optimal cases, a satis­
factory gliding surface can be created, rich in
collagen fibers of types I, IX and X.
The other direction of cartilage surface
creation is the treatment of the circumscribed
small and medium-sized full-thickness
hyaline defects. In these cases, the aim is to Fig. 29.21.
produce a hyaline-type and quality sliding Arthroscopic view of mosaic plasty performed 5
surface instead of the above-mentioned fi- years earlier on the medial femoral condyle.

a hyaline cartilage, and a number of technical
b and financial problems limit the application of
Fig. 29.22.
MR view of the mosaic plasty. (29-27 a b)
a: Preoperative sagittal MR view of osteochondritis
dissecans on the lateral femoral condyle.
b: 20 weeks following mosaic plasty.
brous cartilage surface. The ' mosaic plasty'
procedure (introduced by L. Hangody) has
been performed in a large number of cases
since 1 992. Small cylindrical osteochondral
grafts ( 1 -4 cm2 ) replace the damaged surface
of the loading area (usually arthroscopically)
to ensure congruent surface reconstruction
and to decrease the damage to the donor site
(Fig. 29.20. a, b). During the postoperative
period, fibrous cartilage produced between
the transplanted cylinders in the transplanted
area, generating a composite gliding surface,
where the integration of the transplanted
2 9 . D i s o r d e r s of t h e k n e e � 397
hyaline cartilage and the intermediate fibrous
cartilage takes place (Fig. 29.2 1 .) . This is
clearly shown by MRI (Fig. 29.22. a, b). The
most common indications of mosaic plasty
are osteochondritis dissecans, and traumatic
or degenerative defects.
Because of the limits of transplantation
(limited donor sites and technical difficulties),
the future solution may be cartilage formation
via cell proliferation (autologous chondrocyte
transplantation). So far, however, the struc­
tural organization of the resulting surface is
significantly different form that of the joint
this method. The trend of the present technical
development is the integrated use of holding
matrices (scaffolds) and biologically active
hyaline-inducing materials. The best outcome
is expected at present from the transplantation
of fresh osteochondral grafts.
� Surgical solutions in osteoarthritis
If the degenerative processes are severe
and extensive, it is too late for the procedures
listed above and a good result cannot be ex­
pected. Arthrodesis, the stiffening of the de­
stroyed j oint, is not well tolerated nowadays,
even if this procedure eliminates the pain. Re­
moval of the cartilage and lasting fixation of
the joint can fix the bony elements and stop
the pain, but the joint function is lost. The stiff
Fig. 29.23.
I ntraoperative picture of a total bicondylaer knee
endoprosthesis.
398 � 29. D i s o r d e rs of t h e k n e e
Fig. 29.24.
Secondary arthrosis due to rheumatoid arthritis. Pre- and postoperative x-ray pictures of total
knee prosthesis.
j oint changes the basic dynamics of the gait
and exerts an extreme overload on the neigh­
boring j oints, the contralateral hip and knee
j oints, and the lumbar spine, which may lead
to early secondary osteoarthritis. Implantation
of an artificial joint (endoprosthesis) can lead
to a much better quality of life. Depending on
the extent of the damage, a part or the entire
j oint is replaced. When only the surface of one
of the femorotibial compartments is damaged
(usually the medial one), a unicondylar
(sledge) prosthesis may be indicated (Fig.
29.7.). A precondition of this procedure is the
intact state of the ligaments and the other
compartments and normal axial circum­
stances .
In cases of severe cartilage damage in­
volving a number of compartments, the
proper solution is implantation of a total j oint
replacement (Figs. 29.23. and 29.24.). An im­
portant precondition of this procedure is the
intact function of both collateral ligaments.
During surgery, the anterior cruciate ligament
is sacrificed and stability is ensured by the
changed geometry of the replaced surfaces.
The above-mentioned severe destruction
is treated by means of constrained (hinged)
prostheses, which permit flexion and limited
rotation. These can give rise to complications,
as they loosen more often than ordinary pros­
theses.
In a consideration of the indication of an
endoprosthesis, it must be borne in mind that
the implantation will presumably not be the fi­
nal solution, and there may well be complica­
tions. The forces acting at the prosthesis -
bone junction may eventually loosen the im­
plant long run (according to present statistics,
in 1 0- 1 6 years). This will require revision sur­
gery, with even higher risks. The chances of
sterile loosening are higher for younger pa­
tients, who generally demand increased phys­
ical activity. The prostheses mentioned above
are designed both with and without bone ce­
ment fixation. When all the factors are taken
into account, prostheses are implanted as late
as possible, usually after the age of 60 years.
 J a n o s K r a n i cz, Ka l m a n T6t h
30. Disorders of the foot
30. 1. The functional anatomy and
biomechanics of the foot
The function of the foot is to transfer body
. of the short muscles in the foot and the set of
weIght onto the ground, and at the same time
to ensure the mobility necessary for walking :
In orde� to meet this twofold requirement, a
system IS needed that can withstand large im­
pacts of pressure, and guarantee good mobil­
ity and elasticity in response to different dy­
namic effects .
Regarding its anatomic construction ' the
foot resembles an arrangement of pillars con­
nected by a system of arches (Fig. 30. 1 .) .
Five longitudinal arches are distinguished
from the calcaneus to the metatarsus . The
transverse arch is positioned in a medial­
lateral direction. The arches are maintained by
two stabilizing systems : the passive stabiliz­
ing system, comprising the following ele-
Fig. 30.1.
The bony structure of the foot is constructed of
arches.
� ents : the joint capsules to connect the joints
m the foot, and the collection of minuscule
liga�ents to s �pport these j oints; and the dy­
. .
namic stablhzmg system, which is composed
long muscles originating on the tibia and at­
tached to the foot.
By themselves, neither the passive nor the
active stabilizers are sufficient to support the
normal arch system, they operate flawlessly
only when complementing each other. As mo­
tion becomes more and more dynamic, the
arch system gains increasing significance in
each phase of walking. In the heel-strike
phase, for example, the arch system acts as a
sho � k-absorbe� in order to neutralize a pro­
portIOn of the mertial forces originating from
the contact with the ground. In the push-off
phase, the elasticity of the arch increases the
strength of the push-off. Computerized pres­
sure-distribution analyses have unequivocally
proven that higher levels of pressure are ex­
erted under the second, third and fourth
metatarsus heads than under the first and fifth
metatarsals (Fig. 30.2a), even in the standing
phase. The explanation for this is that the
t� ick adipose tissue under the sole plays a sig­
lllficant role m . pressure distribution. This is
also indicated by the clinical experience that
the atrophy of the adipose tissue of the foot in
rheumatoid arthritis patients leads to severe
metatarsalgia.
During the gait cycle, the pressure factors
b�tween �he g�ound and the foot undergo cy­
clIc modificatIOn (Fig. 30.2b). The force in­
creases on every area of the sole, but the load
inc �eases fr0 t? the rear to the front. The expla­
natIOn for thIS IS . that the middle metatarsals
are longer than the first and fifth. With the lift­
ing of the heel, the short metatarsals lift off the
400 � 3 0 . D i s o r d e r s o f t h e foot
16�
� demonstrate an extremely wide range of pre­
a
416 380
314 216
r1 I
49
59
b
Fig. 30.2.
a: Pressu res during standing.
b: Pressures during walking.
ground earlier, and thus the transfer of energy
takes place via the metatarsals maintaining
contact. The more dynamic the movement,
the higher the load on the anterior part of the
foot.
Walking is a harmoniously structured
string of movements resulting from actively
coordinated operations of both the upper and
lower extremities and the axial muscles. An
understanding of each phase of walking is
necessary in order for the physician to recog­
nize deviations, detailed descriptions of
which are to be found in the chapter on patient
examination (Chapter 4).
30.2. Foot deformities in childhood
30.2. 1. Congenital foot deformities,
developmental abnormalities
142 125
Identified congenital foot deformities
sentations.
Depending on the direction of the defor­
mity, joint contractures occurring in the fron­
tal or sagittal plane are identified. These may
occur in an isolated manner, limited to one
j oint, but combined contracture groups are
more common. These are well known as uni­
form diagnoses (e.g. clubfoot or navicular
foot).
312 277
I I Deformities of the foot occurring in the
sagittal plane
� Pes equinus (talipes eq u i nus): the foot is in a
plantar flexion position at the a nkle joint.
� Pes calcaneus: the foot is constantly i n an
u pwa rd, dorsal flexed position.
� Pes cavus (excavated foot): the anterior part
of the foot is in a cu rved position
compared to the tarsus and the
longitudinal a rch is higher than average.
Deformities of the foot occurring in the fron­
tal plane
� Pes varus: the heels i nvert i nward from the
median at the subtalar joint.
� Pes valgus: the heels turn o utward from the
median at the subtalar joint.
� Pes adductus, metatarsus varus (intoe): the
forepart of the foot is bent toward the
medial at Chopart's joint.
� Pes supinatus (inverted foot): the forepart
of the foot supinates at Chopart's joint.
� Pes abductus: the forepart of the foot is
bent latera l ly at Chopart's joint.
Congenital foot deformities that occur
less frequently:
- Cleft foot (the transverse arch is
missing).
- Developmental abnormalities of the
toes : polydactyly, oligodactyly, syndac-
 30. D i s o r d e r s o f the f o o t � 40 1

tyly, digitus varus, valgus, subductus

and superductus.
- Different amnion stricture disorders.

30.2.2. Pes equinovarus congenitus

(congenital clubfoot)

Terminology. This is a case of congenital

contractu res affecting all joints of the foot,
which can best be described by the Lati n
term: p e s equ inovarus add uctus et
plantiflexus congenitus (Fig. 30.3).

� Equinus: A flexion contracture is present in

the ankle, the talus in the mal leolar fork is
set in a flexed position, and the m. triceps
surae has shrunk.
� Varus: The calcaneus in the subtalar joint is
tilted toward the medial and the forepart
of the foot, and it is a lso twisted,
supinating toward the medial.
� Supination of the forefoot: As regards the
d irection of deform ity, this corresponds to
the varus position of the heel, but due to
the different position, heel varus and
supination of the forepart of the foot a re
d isti nguished in clinica l practice. The
forefoot may take up a pronated position
relative to the heel.
� Adductus: The forepa rt of the foot bends
toward the medial at the Chopart's and
Lisfranc joints.
� Plantiflexus: The a nterior part of the foot is
in a further cu rved position as compared to
the tarsus at Chopart's joint and the sole is
excavated.
Occurrence. Congenital club foot, which
is present at birth, is more frequent among
males population, the male-female ratio being
approximately 2: 1 . Most cases are bilateral,
but it occurs with Similar frequency on the left
or right side.
The incidence in Europe is between
0, 1 -0,2 %, in Hungary, it is 0, 1 25 %.
Its incidence demonstrates racial charac­
teristics. Occurrence among the Polynesian
Maoris is 0,6- 0,7 %.
Fig. 30.3.
Congenital, severe bilateral clu bfoot, the a nterior
part of the foot displays 90° adduction and
supination.
Etiology. There are only hypothetical the­
ories relating to the etiology of congenital
structural club foot:
� mecha nical reasons,
� endogenous reasons,
mu ltifactorial reasons (genetic +
�
environmental effects)
The multifactorial origin is most widely ac­
cepted, i.e. congenital clubfoot tends to de­
velop as a result of the joint presence of a ge­
netic predisposition and environmental effects.
Familial aggregation. Congenital club­
foot demonstrates a familial aggregation, the
risk of multiple occurrence depending on the
familial incidence. The higher the number of
cases of congenital clubfoot in the index fam­
ily, the higher the familial incidence, and con­
sequently, the higher the expectancy ofthe re­
peated occurrence of congenital clubfoot.
Thus, if two siblings are affected, the ex­
pected incidence among subsequent siblings
is 3-5%.
If one parent has congenital club foot, the
risk is 2%, and if one parent and one sibling
402 � 3 0 . D i s o rd e r s of t h e f o o t

are both affected, the risk of further occur­

rence is approximately 25%.

Pathology. Disease-induced lesions affect

all structures of the foot. Diagnosed alter­
ations of the bones are mostly secondary and
are consequences of adaptation to a chronic
malfunction.
- Changes in shape such as a shortened
calcaneus, an elongated talus neck or a
wedge-shaped deformity of the
navicular bone are most typical.
- Regarding the altered articulation of the Fig. 30.4.
bones, the twisting of the calcaneus Untreated severe bilateral clubfoot. The bursa de­
under the talus is significant, it is veloping on the instep impairs walking.
situated high above the horizontal plane,
the talus is in a flexed position, or the
navicular bone and the os cuboideum ward. A toddler, who is able to stand or walk,
have slipped towards the medial at will position the weight on the outer soles, or
Chopart ' s joint. even stand on the dorsum of the foot (Fig.
- The malleolar fork is rotated toward the 30.4.).
medial. In the course of a joint mobility examina­
- The soft tissue shrinks on the posterior, tion, the degree of contracture and the passive
medial surface and the sole. correction are determined.
- The attachment of the tendons widens, Feet that are short, with an elevated heel
and is often atypical. position and a transverse fold of skin behind
- The tibial muscle mass decreases and the heel are indicative of a poor prognosis.
becomes atrophic. The contractures are rigid, the first toe is
shorter, and there is pronounced muscle atro­
Clinical symptoms. Congenital club foot phy along the tibia.
is easily diagnosable at birth due to character­
istic contractures, it can be said that the foot is Radiological symptoms. Taking an X-ray
"bean-shaped" and turned entirely toward the picture in order to determine the diagnosis is
medial, while the inner sole is concave, the not necessary, but during treatment proce­
outer sole is convex, the heel is upright, and dures, an antero-posterior (a-p) and a lateral
high located, and often only fat tissue is palpa­ X -ray image should be taken in order to evalu­
ble behind the ankle. In severe cases, the sole ate the results of conservative treatment and
establish a surgical plan.

" , ,, "
faces backward, with the instep facing for-

w w w
, ,
...., \ -"', � ..,.. .:' :;
Fig. 30.5.

�. . . W �2 " :'
.... : /
The ang les of the tarsal bones diverge char­

� " J8
\ ... ! ' ... : ' ... '
acteristically in case of foot deformities. A de­
" , ... ... creased angle causes clubfoot and an in­
...
creased angle resu lts in navicular feet. (30-9)

. (2 . . . . /: . . . .

L
a: Clubfoot, angle: 0° .
'
. . .. . !
. . ...
. . . . ..
a b
.
\1 . . . . . . . � e
(j!'3 . ;
d e
.
b: Excavation, angle:
e: Normal, angle: 35°.
d: Flat feet, angle: 50°.
20°.

e: Navicu lar foot, angle: 80°.


The X-ray image depicts the position of
the tarsal bones in relation to each other and
also the shapes and structure of the bones.
In order to describe the position of the tar­
sal bones, the anteroposterior and the lateral
talocalcaneal angles are used, which are
smaller than normal in patients with clubfoot
(Fig. 30.5.).
The relation of the posterior and the ante­
rior parts of the foot can best be described
through the talometatarsal and the naviculo­
metatarsal angles (Fig. 30.6.).
Treatment principles
� Conservative treatment
Treatment is primarily conservative: the
methods applied a re early passive and
active p hysica l exercise, g rad ual
correction with plaster casts, o rthoses
and specia l footwear.
� Surgical treatment
Ea rly soft-tissue surgery, which is
nowadays usually a conseq uence of
unsuccessful conservative treatment.
Reoperation i n recu rrent cases.
Broader surgical i ntervention on soft
tissue and bones at a later age.
� Nursing
Organized care should be offered at
specialized treatment centers.
Regular physiotherapy is carried out
until the p hase of foot g rowth ends,
after which orthopedic technical
support and continuous o rthopedic
specia list fol low- u p is needed.
Detailed description of conservative
treatment implementation. Passive physical
exercises of the foot (unless contraindicated)
and correction of the j oints in the direction op­
posite to the contraction should be com­
menced immediately after birth. This may be
performed by infant ward nurses, physiother­
apists and parents alike. Stimulation of the
skin of the sole (e.g. with a soft toothbrush)
will induce active foot movements. The opti­
mal instance for commencing gradual correc­
tion with plaster casts is on day 5-7.
3 0 . D i s o r d e r s of t h e f o o t � 403
Fig. 30.6.
I ncreased talometatarsal angle in clubfoot.
a: Normal. b: C1u bfoot.
Gradual correction with plaster casts may
be implemented according to the Lorenz prin­
ciples in the following order: inflexion,
adduction, supination of the forefoot, varus of
the heel and finally correction ofthe equinus.
Correction methods aimed at reducing or
eliminating all possible contractures (as long
as the foot can endure them) are also acknowl­
edged.
In both cases, the achieved result is to be
stabilized in a circular plaster cast with the
knee in a 90° position and the cast running
from mid- or upper thigh to the end of the toes,
with the top of the toes left uncovered. Felt
sheets should be attached corresponding to
the bones running directly under the skin, and
the skin itself should be protected with a thin
layer of cotton wool.
It is advisable to inspect the plastered foot
after 20-30 minutes in order to ensure that no
blood circulation disorders have arisen. If any
such problems are perceived, loosening or re­
placement of the cast is necessary.
The plaster should initially be changed
weekly and, after manual exercising, a new
circular plaster cast should be applied.
404 � 3 0 . D i s o r d e r s of the foot

A t later ages, alternating plaster correction residual disorders, dorsal, or dorsa l-inner
should be applied every 2-3 weeks. Special soft-tissue release.
care should be paid not to place the foot in a � In u ntreated, late cases in older children, or
"rocking chair" position, which would mean as repeated su rgery for recu rrence
over-correction of Chopart 's j oint and initiat­ between the ages of 1 and 3, extensive
ing subluxation in the dorsal direction. It is soft-tissue surgery or peritalar a rthrolysis
advisable to verify the situation by means of may be indicated.
an antero-posterior and a lateral X-ray picture. � Between the ages of 3 and 12, the
When untreated clubfoot is encountered in treatment may be as mentioned above,
infants or early childhood, the conservative access being ensured laterally for
treatment applied is identical to that for the repositioning of the os navicu lare:
newborn. In these cases, gradual correction calca neocuboidal wedge resection
with plaster casts is merely capable of reduc­ according to Evans, resection of the
cuboidal su rface of the ca lcaneal joint
ing the contractures and the establishment of
according to Lichtblau or calcaneus
better conditions for surgical treatment.
osteotomy.
In recurrent cases of clubfoot or after re­
Above the age of 3 years, muscle
peated surgery, the application of a long-term
transplantation is possible in order to
plaster cast may be necessary, sometimes with
ensure recovery of the muscle ba lance.
use of the Kite method (following surgical
As soon as the g rowth of the foot has
correction and stabilization of the foot in plas­
ended after pu berty, wedge resection,
ter, a wedge is removed from the cast after
subtalar or Chopart's joint a rthrodesis
which the cast can be further corrected).
may be carried out.
A plastic orthosis may be applied at any
Supramal leolar rotational tibial osteo­
point after removal of the plaster cast.
tomy of the i nverted foot has the aim of
Following the conclusion of conservative
palliative correction.
or surgical treatment, the foot and the muscles
should be exercised and strengthened, primar­
ily by means of physiotherapy.
Surgical methods. Dorsal release. This
Standard shoes are generally adequate for
involves "Z" shaped Achilles tendon length­
walking, but it may be necessary to acquire
ening, dorsal capsulotomy of the ankle and
antivarus shoes with a straight sole-axis and
hindfoot joints, and posterior incision of the
an elongated inner counter reaching to the
talofibular ligament.
front of the shoe.
Dorsal and medial release. Posterior
In recurrent cases, an orthopedic shoe spe­
tenotomy of the m. tibialis is performed, to­
cially constructed for the patient' s foot may
gether with the previous interventions, medial
be necessary, with a straight sole-axis, a fron­
and plantar capsulotomy of the talonavicular
tally extended pronated heel, and a stronger
joint, ad capsulotomy of the frontal and dor­
inner counter.
sal-medial segments of the subtalar joint,
�
capsulotomy of the naviculocuneiform and
Surgical treatment
the metatarsocuneiform joints.
Surgical indication . It is a general princi­
ple, that all structures sustaining contractures
Aspects of physical exercise. These in­
should, as far as possible, be eliminated in the
clude alleviation of contractures, passive cor­
course of surgery.
rective exercise, active exercise, and strength­
ening of the femur and the foot muscles.
Surgery is advised in the fol lowi ng cases: Electric stimulation of weakened muscle
� After 6 months of age, fol lowing groups may be performed and the patient may
conservative therapy, i n o rder to correct be provided with walking instruction, walking

correction, implementation of passive sup­
porting and stabilizing orthoses, and lifestyle
and vocational guidance.
Orthopedic shoes should be worn.
Suggested sports that ensure regular mus­
cle strengthening for the feet are short- and
long-distance running, cycling and ball­
games.
30.2.3. Pes adductus
The forefoot bends medially at Chopart's
joint. The situation is similar to clubfoot, a l­
though it is well distinguishable, since the heel
is not in varus or equ i n us. Although to various
extents, the heel is i n a valgus position, and it
is therefore often referred to as pes adductus
planovalgus.
Occurrence. It is easily diagnosed imme­
diately after birth. Some consider it to be a
genuine developmental abnormality, while
others assume it to be an abnormality due to
the constraint of maintaining the foot in a
varus position while in the womb .
The condition is in most cases bilateral; it
occurs more frequently in the male popula­
tion, the male-female proportion being 3 : 1 .
Diagnosis. Besides the adduction
contracture of Chopart ' s j oint and the valgus
Fig. 30.7.
Pes adductus, the forefoot curves medially at
Chopart's joint.
3 0 . D i s o r d e r s of t h e f o o t � 405
position of the heel, the diagnosis may b e es­
tablished on the basis of the medial
(metatarsus varus) inflexion of the first toe
(Fig. 30.7. and 30.8.). The increase of the dis­
tance between the first and the second toes is
clinically also clearly visible.
Radiological symptoms. The angles of
the talus and the calcaneus are larger than nor­
mal, the navicular bone at the head of the talus
is displaced laterally, directed in a subluxed
position, and the metatarsals deviate toward
the medial side.
Treatment. In mild cases, there is a ten­
dency to spontaneous correction.
Release of the adduction contracture of
Chopart ' s joint is necessary, by conservative
methods if possible.
Manual correction. Axial traction of the
forefoot by fixed calcaneus, and exercising
the forefoot in abduction and pronation is ad­
vised with the application ofBebax redressing
sandals.
a b
Fig. 30.S.
Graphic illustration of a normal foot (a) and a pes
adductus (b).
406 � 3 0 . D i s o rd e r s o f t h e f o o t

Active physical exercise is also recom­

mended with the concurrent application of
skin stimulation.
Surgical treatment. Incision of the m. ab­
ductor hallucis and the medial joint capsules
may be carried out after 2 years of age.
Metatarsus base osteotomy in order to correct
the deformity is possible at a later age.

30.2.4. Pes excavatus

The term hol low foot (pes varus o r pes

cavovarus) describes such foot defo rmities
when t he longitudinal arch is higher than av­
erage, rega rd less of the cause i n itiating the
condition.

Clinical symptoms. This unmistakable

foot deformity takes shape by about the age of
1 0- 1 2 ; the foot is relatively short, the longitu­
dinal arch is higher, the anterior part of the
foot is in adduction, the heel in varus position,
and the 1 st metatarsus head is situated low
down, which appears more explicitly due to
the strongly constrained dorsal-flexed posi­
tion of the big toe and the other toes in the
condition known as hammertoe (Fig. 30.9. a,
b).
The aponeurosis plantaris is stretched like
a taut string, which intensifies during the
dorsiflexion of the forefoot and raises the skin Fig. 30.9.
of the sole. a: Excavated foot, the longitudinal arch is elevated
(X-ray).
Soreness and callosities may emerge on
b: In cases of excavated foot, the patient is unable to
the plantar side of the forefoot over the meta­
rest the heel on the ground because of the exten­
tarsal heads. sive inflexus.

Initiating causes. This deformity gener­

ally develops as a consequence of a mus­
cle-balance disorder. The lumbrical and
interosseus muscles weaken, and therefore the
effects of the long flexors and extensors take
over.
The reason for the muscle-balance disor­
der is generally myelodysplasia or spina
bifida occulta, though other neuromuscular
disorders may also occasionally be diag­
nosed.
Treatment
Conservative treatment. The treatment of
mild cases of pes excavatus is in conservative.
Physical exercise is advised to stretch the
shortened muscles, and the wearing of ortho­
pedic shoes if necessary.
Surgical treatment. In childhood,
Steindler 's operation is carried out: the
aponeurosis plantaris and the short plantar
muscles are detached from the heel . This may

be supplemented with incision of the capsules
on the plantar surface of Chopart 's joint.
Tendon and muscle transplantation may
be needed to correct the arched position of the
toes, especially the big toe.
In the event of a pronounced deformity af­
ter 1 0- 1 2 years of age, osteotomy of the cunei­
form bones with removal of a dorsal-based
wedge will ensure an improvement in appear­
ance, due to the shortening of the longitudinal
arch.
After the foot has finished growing,
arthrodesis of Chopart 's joint and the tarsal
joint may come into consideration.
30.2.5. Pes calcaneovalgus
congenitus
This is a congenital developmental va lgus de­
formity of the foot, with varying extents of
severity.
In line with the appearance of the defor­
mity, the condition is often referred to as rock­
ing chair foot, navicular foot or congenital
flatfoot.
With regard to the main pathogenetic fac­
tor behind these deformities, the disease is of­
ten referred to as vertical talus .
Clinical symptoms. In all cases, the sole
is convex, the instep is slightly concave, the
forefoot is abducted, pronated and extended at
Fig. 30.10.
Oinical depiction of bilateral congenital navicular
foot.
3 0 . D i s o r d e r s of t h e f o o t � 407
Chopart 's joint, furthermore, the tarsus is in
equinus and the heel is in a distinct valgus
(Fig. 30. 1 0.).
Etiology. Foot deformities described as
vertical talus do not form a closed etiological
entity; similarly to clubfoot, several varieties
are defined.
- Structural navicular foot: This is a
distinct developmental deformity of the
foot; its isolated occurrence is very rare.
- Symptomatic navicular foot: This
deformity is present already at birth, as
one symptom of many relating to a
complex syndrome (e.g. Freeman­
Scheldon 's syndrome) . It is often
regarded as a result of arthrogryposis
multiplex congenita. It is generally
bilateral, the contractures are rigid, and
respond very poorly to corrective
treatment.
- Myelodysplasia-related navicular foot.
This too is often already present at birth.
A muscle-balance disorder plays the
main role in its development. It has been
reported by Sharrard, that if the lesion is
present in or above the 3 rd lumbar
vertebra, the contractures are extremely
rigid. In case of lesion between L5 and
S I , looser contractures are perceived.
- Spastic navicular foot. This develops in
most cases after the patient has learned
Fig. 30.11.
Talus positioned vertically, demonstrating the
pathological relation of the talocalcaneal joint and
Chopart's joint.
408 � 3 0 . D i s o r d e rs of the foot

to walk and is a consequence of a

muscle-balance disorder, with the heel
in an equinus position.

Radiological symptoms. The steep posi­

tion of the talus, the pathological correlation
of the tibiotalaris and the talocalcaneal is, and
the dislocated or dorsolaterally subluxed posi­
tion of the os naviculare on the talus-neck
(Fig. 30. 1 1 .) are characteristic of all above
listed foot deformities .

Treatment. The obj ective is repositioning

of the talus and the os naviculare, normaliza­
tion of the position of the tarsus and the ante­
rior part of the foot and preservation of the
corrected position.
Conservative treatment. If any type of
navicular foot is noticed at birth, passive
physical exercising should be commenced
immediately, followed by gradual correction
with plaster casts.
In case of navicular foot developing as a
result of spastic or flaccid paralysis, gradual
correction with plaster casts is rarely imple­
mented, treatment is primarily surgical.
Surgical treatment. From the age of 6
months to 3 years, posterolateral or, if neces­
sary, medial release is performed.
30.2.6. Accessory ossicles
on the foot
Generally, no complaints are caused by
this condition, although its occurrence is fre­
quent, most often involving the os tibiale
externum ( 1 0%), the os trigonum (8%), and
the os peroneum (5%).
� Os tibiale externum
This is found on the inner side of the foot,
beside the os naviculare. It is associated via
either fibrous tissue or cartilage with the
navicular bone, together forming a horn
shape, which is why it is also referred to as os
naviculare cornutum.
Its clinical significance is that it protrudes
in the medial part of the foot, on the inner side,
and consequently, the wearing of shoes will
Fig. 30.12.
Accessory bones on the medial side of both
navicular bones: os tibiale externum.
eventually cause painful inflammation and
hyperkeratosis.
On an X-ray, it is easily detectable, espe­
cially in an antero-posterior position beside
the os naviculare as a separate bone center
(Fig. 30.1 2.).
Treatment. The use of insoles may reduce
discomfort, but only surgical removal will re­
sult in complete elimination of the irritation.
Special attention should be paid to maintain­
ing the posterior attachment of the m. tibialis.
30.2.7. Tarsal coalition
This is a pathologic connection(s) between
different tarsal bones.
When stiff flat feet are detected in adoles­
cence, possible pathologic bindings between
different tarsal bones should be taken into
consideration.
Depending on which bones are connected,
the following forms are identified: calcaneo­
navicular coalition, talonavicular coalition
and calcaneocuboid coalition.
In most cases, calcaneonavicular coalition
is diagnosed. The binding tissue may be fi­
brous, cartilaginous or bony. The possibility
of this disorder is often suspected on a lateral
 3 0 . D i s o r d e r s of t h e foot � 409

X-ray picture, but a cartilaginous or bony co­

alition is weII perceivable on an oblique im­
age (Fig. 30.13.).

Treatment. The disorder is not curable by

conservative treatment.
In childhood, resection of the bony bar,
and in adulthood, subtalar and Chopart ' s j oint
arthrodesis is performed in order to terminate
the complaints.
Fig. 30.13.
30.2.8. Osteochondrosis calcanei The bony fusion between the os naviculare and the
(apophysitis calcanei) calca neus can be observed in an oblique image.

This mostly develops between the ages of 7

and 13 in male patients, often bilatera l ly. It is
believed that besides ossification d isorders, an
i ncreased functional load and d isorders of the
blood supply of the apophysis a lso p lay a part
in its development.

Clinical symptoms. Children complain of

rapid exhaustion, and of tarsal pain and sensi­
tivity around the adherence of the Achilles
tendon, which escalates when subjected to ex­
tensive strain. The pain is more intense in re­
sponse to the dorsiflexion of the foot, it de­
creases upon plantiflexion. Walking up and
down stairs is especiaIIy difficult. The heel is
slightly thickened.
Radiological symptoms. The apophysis
of the calcaneus is indistinct, with an unclear
contour and an irregular shape. The ossifying
center is fragmented (Fig. 30.1 4.).
Treatment. Treatment of the disorder is
conservative. Complaints should cease upon
relaxation and lifting of shoe-heels.
30.2.9. Osteochondrosis ossis
navicu laris pedis (Kohler's I
disease)
The essence of this d isorder is aseptic necrosis
occu rring in the os navicu lare pedis. The d iag­
nosis was described by Kohler i n 1908. It ge-
Fig. 30.14.
The apophysis of the calcaneus is shattered and
sclerotic.
nera l ly occurs between the ages of 3 and 10.
The d isorder is more freq uent in males and
approximately 30% of the cases a re bilateral.
Etiology. The etiology has not yet been
clarified; the most probable cause is an ossifi­
cation disorder. Some associate the iIIness
with trauma.
Clinical symptoms. The condition often
develops without causing any particular com­
plaints and only an X-ray image taken for
some other reason will shed light on its exis­
tence. The complaints are manifested in rapid
exhaustion, limping, and local pain and swell­
ing in the area of the navicular bone in the in­
step. The limping intensifies after extensive
walking. Passive movement of Chopart ' s
j oint is limited, and may even be painful.
4 10 � 3 0 . D i s o r d e r s o f t h e foot

Radiological symptoms. The structure of

the navicular bone is irregular, it is porotic.
During the progression of the process, the
bone decreases in size, gradually forms a disk
shape, becomes sclerotic and diminishes. At­
rophy may be observed on other bones of the
foot. In the process of healing, the os
naviculare regains its original form and shape
(Fig. 30. 1 5).
Fig. 30.15.
Treatment. Shoe inserts are recom­
Kohler's I disease: the ossifying center of the os
mended, through which the navicular bone naviculare is cracked and the bone is flattened.
can be partially relieved of body weight.
There is no need for surgical treatment.

30.2. 10. Ostechondrosis capitis

metatarsi 11. (Kohler's 11 disease)

This disease denotes aseptic necrosis occur­

ring in the 2nd, (rarely the 3rd or 4th) meta­
tarsa l head. It genera l ly develops between the
age of 10 and 18 and is three times as fre­
q uent i n female popu lation as in males, rarely,
it is bi latera l.

Etiology. It generally occurs in cases of

pes transversoplanus. Its development is ex­
plained by the fact that since the arch of the
foot is descended, the 2nd metatarsal head is
set deeper and is exposed to increased weight
pressure. An inadequate blood supply of the
epiphysis may also play a role in its develop­
ment. Fig. 30.16.
Kohler's 11 disease: deformation of the metatarsal
Clinical symptoms. The symptoms vary head.
in accordance with the 3 stages of the disor­
der.
tensifies during the roll and when standing
a) In the early stage, the dorsal surface of the on tiptoe (plantarflexion of the foot).
metatarsal head is sensitive to pressure. c) In the final stage, arthrotic symptoms pre­
Rapid exhaustion occurs, which intensi­ dominate. Exostosis is palpable on the
fies upon exertion, and the patient there­ metatarsal head, and the toes sub luxate.
fore tends to walk on the edge ofthe sole. Callosity develops in the corresponding
spot on the sole.
b) In the middle or compression stage, swell­
ing and painful disability arise in the fore­ Radiological symptoms. No changes are
foot. The patient tries to evade the regular perceivable in the initial period. In the com­
rolling phase of the feet, since the pain in- pression stage, the first sign is the sub-
 3 0 . D i s o r d e r s of t h e f o o t � 411

chondral, transverse radiolucency in the meta­

tarsal head; later, the joint surface becomes
flattened and deformed, but it remains well­
defined. Arthrosis and deformed joint sur­
faces are characteristic in the final stage; the
metatarsal head is cylindrically deformed
(Fig. 30.1 6.).

Treatment. Treatment of this deformity, a

if possible, is conservative, the chief objective
being to relieve the load on the forefoot and
the metatarsal head. Suitable insoles and spe­
cially designed rocking bottom shoes will
eliminate the discomfort. During the develop­
ment of normal bone structure, the deformity
will heal in 1 -2 years .
Resection of the head will result in total re­
cuperation. Excochleation of the necrotic seg­
ment and spongiosa plasty may also be at­
tempted.

30.3. Foot deformities in adu lthood

30.3. 1. Pes planus (pes

planovalgus, flat foot, fallen arch)

This is the most frequent foot d isorder of

static orig in. The background of the condition
is the collapse of the a rched system of the
foot, since the active and passive stabilizing
systems supporting the arch a re insufficient. c
The d isorder is cal led pes pla novalgus if the
d
predomi nant factor is collapse of the longitu­
dinal arch together with the va lgus heel. If the
transverse arch has fa llen with the calcaneus
i n the normal position, the condition is ca l led
pes transversoplanus.

It is worthwhile to reflect on the different

types concurrently, since they are often asso­
ciated and the triggering causes are mostly the
same.
Etiopathology. When the active and pas­
sive stabilizing systems of the foot are not ca­
pable of equalizing the pressure of body
weight placed on the feet, the valgus position
of the calcaneus will develop and the longitu-
Fig. 30.17.
Normal longitudinal arch (al and pes planus
b: The descent of the lon.gitudinal arch.
c: This image of the patient clearly reveals how the
medial side of the foot almost touches the g round;
calluses are also well observable.
d: X-ray picture of a flattened longitudinal arch in a
standing patient.
412 � 3 0 . D i s o r d e rs o f t h e foot

dinal arch will descend (Fig. 30. 1 7. a-c). This more, medially from the tip of the medial an­
is generally followed by the collapse of the kle, distally and slightly anterioriy, the con­
transverse arch as well. tour of the talus head protrudes. Under normal
The reasons for the disruption of the bal­ conditions, body weight is transferred onto
ance: the trochlea of the talus, and from here is dis­
- overloading of the foot (body weight or tributed toward the calcaneus and the
pregnancy) metatarsus. With weakening of the m. tibialis
- the decreased weight-carrying capacity posterior, the m. extensor hallucis longus, the
of the foot m. extensor digitorum longus and the pero­
- insufficient functioning of the passive neus muscle group, the talus head will tilt
stabilizers (congenital weakness of
connective tissue, Ehlers-Dahnlos
syndrome, hormonal effects or preg­
nancy)
- insufficient functioning of the active
stabilizers muscles (paralysis,
�1
Heine-Medin disease or myelodys­
plasia)
� 0 O D-D 2
- improper loading of the foot: com­
pensatory (infantile cerebral paresis)
- following trauma or inflammation, or in
case a tumor can damage the bony
structure, tendons or ligaments of the
foot arch 3

Clinical symptoms. The height of the nor­

mal longitudinal arch decreases, or in extreme
cases it may disappear totally. Posterior ob­
servation of the patient reveals the position of
the calcaneus valgus (Fig. 30.1 8.). Further-
a

Fig. 30. 19.

Fig. 30.18.
Pes calcaneovalgus. The axes of the leg and the
calca neus are at a n angle of approximately 20°.
a: Graphic image of a normal (1) and a collapsed
transverse arch (2) and an expanded forefoot (3).
b: Calluses developed on the sole below the
2nd-4th metatarsal heads (as a sign of increased
pressure on the sole) in the case of a fallen trans­
verse arch.

downward, toward the medial. The medial
contour of the foot will therefore become con­
vex and the forefoot will assume a pronated
position in relation to the heel.
Collapse of the transverse arch is also a
static deformity, its triggering causes corre­
spond to those of pes planovalgus, with which
it is often associated, generally in the mid­
dle-aged. Because of the increased weight af­
fecting the metatarsal heads, the transverse
arch drops (Fig. 30.19. a), and the foot
spreads out. Calluses may develop under the
2nd, 3rd and 4th metatarsal heads on the sur­
face of the sole (Fig. 30.19. b). In cases of
rheumatic diseases, the decay of the ligaments
and the inflammated bursa on the sole of the
foot intensify the deformity and the pain.
In case of neonates, the arch system of the
foot is not yet developed, and similarly, their
physiological curves are not yet perceptible
on the spine. These features develop later, in
response to the appropriate stimuli . Parents
often worry that the transverse and longitudi­
nal arches have not yet developed on the foot
of their infant, who is about to start walking.
The development of the arch system is
time-consuming, and furthermore, the muscu­
lar system of the foot has to grow used to the
muscle exertion necessary for walking. The
final development of the arch system is con­
sidered normal up to the age of two years .
Throughout growth (bearing in mind that
growth is not a linear process) there are peri­
ods when the foot is exposed to increased ex­
ertion. One of these periods is the commenc­
ing of school, when the wearing of a backpack
and walking to school may trigger an over­
loading of the feet. Adolescence is similarly a
critical stage, owing to the significant increase
in body weight and the changing circum­
stances (intensive sporting activity and per­
haps the commencing of employment) on the
other.
In cases of fixed, muscularly affixed flat­
foot, both the ligamental system and the dy­
namic stabilizing system are insufficient, but
the disorder can still be corrected: in this con­
dition, the arch may be restored under anes­
thesia. When ligamental fixation has devel-
3 0 . D i s o r d e r s of t h e foot � 413
oped, the arches can not be restored, not even
under narcosis. This condition is referred to as
bony rigidity.
Naturally, there are certain circumstances
or occupations that may bring about an insuf­
ficiency of the arch system by straining it ex­
cessively. Such occupations include those of
waiters, hairdressers and surgeons. Physio­
logical changes too may cause the develop­
ment of flat feet, as in pregnancy, when the
enhanced loosening of tissue results in a de­
crease in the passive stabilizing effect of the
ligamental system. With a normal ligament
and muscular system, obesity will lead to the
collapse of the arches because of the excess
weight load.
Treatment. One of the most important ob­
j ectives is prevention.
In childhood, the child should not be
forced to walk and should be carried when
tired. Strengthening of the foot muscles is also
very important. One technique is to encourage
walking on uneven surfaces. It is very impor­
tant, that walking barefoot on artificially flat
surfaces such as wooden floors or boarding is
explicitly harmful, since in these cases there is
no need for the active operation of the foot
muscles, and thus the muscles will wither. In
contrast, on pebbly, bumpy, grassy or sandy
surfaces, the foot is forced to adapt to the
given surface owing to its uneven nature,
which results in the strengthening of the dy­
namic stabilizing system. At the same time, it
is possible to actively fortify the foot muscles
by means of playful activities, e.g. picking up
pens and pencils from the floor with the toes.
It is possible to encourage the strengthening
of the muscles of the sole through such exer­
clses.
Insoles are not prescribed automatically
for children aged 6-7 with flatfoot. In a pes
calcaneovalgus condition, the valgus position
of the heel is compensated by the lifting of the
medial part of the shoe heels. A 5 -mm wedge
is positioned medially under the heel, sloping
toward the lateral. Thus, the elevation corrects
the axis of the valgus positioned calcaneus.
Physical exercising of the foot is especially
4 14 � 3 0 . D i s o r d e r s of t h e f o o t

important for these children. Insoles are not part of the 1 st metatarsal in the medial direc­
recommended, since relieving of the foot tion (Fig. 30.20. a, b). One of the conse­
muscles and passive support of the arch will quences of this displacement is that the m. ab­
lead to further weakening of the muscles. ductor hallucis slides onto the surface of the
When fixed flatfoot develops in older chil­ sole and pronates the hallux. The flexors and
dren, loosening of the rigidity may be extensors with the sesamoid bones lateralize
achieved conservatively through application relative to head of the I st metatarsal, pulling
of a cold compress, and after loosening, the the hallux into adduction (valgus) (Fig. 30.2 1 .
corrected position should in certain cases be a ) . Hyperactivity of the m. adductor hallucis
maintained with a plaster cast. and the position of the flexors results in a vi­
If no results can be attained through appli­ cious circle that constantly amplifies the de­
cation of a cold compress, manipulation formity.
should be performed under narcosis and a
plaster cast should possibly be applied.
In adolescence and adulthood, patients
with flatfoot are recommended insoles. In­
soles correct the valgus position of the
calcaneus, thereby establishing the longitudi­
nal arch and they may also support the trans­
verse arch. In case conservative treatment
does not bring the desired results, surgical so­
lutions may also come into question. Instead
of the former practice of positioning a bony
wedge into the sinus tarsi in order to achieve
an extra-articular arthrodesis, corrections are
nowadays executed through the utilization of
screws. Another possibility is calcaneus
osteotomy, in the course of which the patho­
logic axis is corrected through wedge resec­
tion and/or shifting. When marked arthrosis is a
associated with axis deviation, fusion of the
b
talocalcaneal and Chopart 's j oints may be
considered.

30.3.2. Hallux valgus, metatarsus I.

varus

This is a valgus deformity (subluxation in the

metatarsop halangeal joint) of the ha llux of
static origin with a variable extent of varus
position of the 1st metatarsal.

Etiopathology. Congenital, constitutional

(loose ligaments or falling of the transversal
arch) and external factors (tight, fashionable
or pointed shoes) can all play a role in the de­ Fig. 30.20.
velopment of a bunion. The most common a: Severe hallux valgus.
cause of this disorder is a shift of the distal b: X- ray image of the above patient.
 3 0 . D i s o r d e r s of t h e f o o t � 415

b
i a

+---- 1
i i __-1-1---- 4
�....b,J_...I__l_--- 5

Fig. 30.2 1.
Hallux valgus.
a: The pathological pulling direction of the tendons
intensifying the valgus
(1: m. extensor hallucis longus, 2: m. flexor hallucis
brevis, 3: m. add uctor hallucis, 4: m. flexor hallucis
longus, 5: m. abductor hallucis).
b: Graphic illustration of an orthotic device to cor­
rect hallux valg us.

Treatment. Pain killers, insoles, the wear­ 1 ---+�

ing of wide or orthopedic shoes and perhaps a
hallux valgus-correcting appliance (Fig.
30-2 1 . b) at night may come into consider­
---=="r"F';;,./
2
ation as forms of conservative therapy. A sat­ 3

isfactorily improved condition may be ex­

pected only after surgical treatment.
Radiological parameters, which play a
role in the surgical indication are demon­
strated in Fig. 30.22.
There are more than 1 50 possible surgical
4 --+--+
interventions for the correction of hallux
valgus. These may be divided into three basic
groups: operations involving only soft tissue,
operations of only bony tissue, and a combi­
nation of the two. One factor that significantly
influences the surgical indication is whether
arthrosis is present in the 1 st metatarso­
phalangeal joint. If it is, correctional osteo­ Fig. 30.22.
tomy can not come into question. Soft-tissue
Radiological parameters playing a part in the indica­
operations are generally carried out before the tion: 1: interphalangeal angle, 2: hallux valgus angle,
closure of the growth plates. In adulthood, the 3: the angle formed by the metatarsus axis and the
indication of combined surgery depends on joint su rface, 4: the angle formed by the axes of the
the following factors : 1st and 2nd metatarsi.
4 16 � 3 0 . D i s o r d e r s of t h e f o o t
Fig. 30.23.
Osteotomy of the 1st metatarsus base and Akin osteotomy of the proximal phalanx. a: Clinica l image before
su rgery. b: Antero-posterior X-ray image of the foot before surgery. c: Antero-posterior X-ray image of the
foot after surgery. d: Clinical image after su rgery.
Phase 1 : The hallux valgus angle is smaller
than 2 5° , the intermetatarsal angle is
smaller than 1 2° , the mobility of the 1 st
metatarsophalangeal j oint is complete, and
the dislocation of the sesamoid bone is
smaller than 25%. In this case, Schede ' s
operation and/or distal metatarsal and/or
distal phalanx osteotomy may come into
question concurrently with a soft-tissue
correction (which generally involves
shortening on the medial side and release
on the lateral side), supplemented with
tenotomy of the adductor hallucis.
Schede 's operation: chiseling away the
exostosis that has developed on the medial
side of the 1 st metatarsal, together with re-
moval of the irritative bursa formed be­
tween the exostosis and the skin.
Phase 2: The hallux valgus angle is 25-3 5°
(possibly even 40°), the intermetatarsal
angle is 1 2 - 1 8°, there may be subluxation
in the 1 st metatarsophalangeal joint, and
the dislocation of the sesamoid bones is
25-50%. The interventions applied in this
phase: besides removal of the exostoses
and soft-tissue interventions, distal (possi­
bly diaphyseal) or proximal 1 st metatarsal
osteotomy and if necessary, proximal pha­
lanx osteotomy.
Phase 3: The hallux valgus angle is larger
than 40°, the intermetatarsal angle is larger
than 1 8°, the 1 st metatarsophalangeal joint

is subluxed and/or the 1 sI ray is hyper­
mobi le. Surgical interventions imple­
mented in this phase (at a young age, if no
arthrosis is present in the metatarso­
phalangeal joint) are proximal 1 st metatar­
sal osteotomy (Fig. 30.23. a-d) or meta­
tarsocuneiform arthrodesis and if neces­
sary, proximal phalanx osteotomy.
30.3.3. Hallux rigidus
Hallux rigid us is the term used to describe
pain-ind ucing, isolated limitation of move­
ment of the 1st metata rsophalangeal joint, it
is genera l ly caused by arthrosis. I n these cases,
valgus deformity is minimal or not p resent at
all. The ROM may be minimal i n the functional
aspect, but enough is left to sustai n the pain
(Fig. 30.24.).
The clinical symptoms of osteoarthritis of
the 1 st metatarsophalangeal j oint: pain, crepi­
tation and a significantly decreased ROM.
The condition may develop as a result of
trauma or inflammation, or a preceding bun­
ion operation.
The patient ' s gait is disturbed by the pain
induced and the lift-off phase of the patient' s
gait i s perturbed because of the absence o f the
1 st metatarsophalangeal j oint extension.
Rocking bottom orthopedic shoes are recom­
mended as conservative treatment. Besides
Fig 30.24.
X- ray image of hallux rigid us. The joint space of the
metatarsophalangeal joint is significantly narrowed,
and osteophytes may be observed on the edge of
the bones.
3 0 . D i s o r d e r s of t h e f o o t � 417
Fig. 30.25.
Schematic illustration of cheilectomy. 113 oblique
dorsal resection of the distal joint surface of the 1st
metatarsus, and 113 dorsal resection of the proximal
joint su rface of the proximal phalanx.
the radiological and clinical condition, the in­
dication of surgical treatment is strongly de­
pendent on the patient' s age and the level of
his/her requirements .
The surgical treatment of hallux rigidus
may be categorized as follows :
a) An operation retaining the 1 sI metatarso­
phalangeal joint, which consists in remov­
ing with a chisel the bony osteophytes that
have developed on the metatarso­
phalangeal joint area of the metatarsus and
the proximal phalanx dorsally, in this way
re-enabling dorsiflexion of the j oint
(cheilectomy) (Fig. 30.25.).
b) Operations sacrificing the 1 st metatarso­
phalangeal j oint, consisting in either re­
section of the base of the proximal phalanx
of the hallux or arthrodesis of the meta­
tarsophalangeal joint.
30.3.4. Diseases of the 5th ray
Bony irregularities and axia l deformities may
develop on the 5th ray on the latera l side of
the metata rsus (Fig. 30.26. a, b).
These disorders are essentially equivalent
to lateral deviation or increase of the 4th-5th
intermetatarsal angle. They may cause dis­
comfort and/or problems even when normal
shoes are worn.
4 18 � 3 0 . D i s o r d e r s of t h e f o o t

a
b

1 2 3 4
Fig. 30.26.
a: Categorization of deformities of the 5th metatarsus:
Type 1: Exostosis on the lateral side of the 5th metatarsus head; no lateral deviation on the metatarsus, the
4th-5th intermetatarsal angle is normal.
Type 2: I ntense lateral deviation of the 5th metatarsus. No real hypertrophy can be observed on the lateral
side of the metatarsus head; the intermetata rsal angle is within the normal range.
Type 3: I ncreased i ntermetatarsal ang le; no hypertrophy and the extent of deviation is normal.
Type 4: Combined variation: hypertrophy and/or increased deviation is present with an increased
intermetatarsal ang le.
b: Schematic drawing of a correctional osteotomy performed on$ a type 4 deformity as described above.

Treatment. In cases of exostosis, if no 30.3.6. Metatarsalgia

other disorder is observed (an increased
4th-5th intermetatarsal angle or enlarged
curve of the 5th metatarsus), removal of the
exostosis is recommended. When deformities
of the 5th metatarsus are observed, osteotomy
may be considered. If the 5th toe is super­
posed over the 4th toe (digitus V varus super­
ductus, which may be present congenitally),
the above solutions may be supplemented
with plastic surgical interventions carried out
on the joint capsule, the tendon or the skin,
through the formation of artificial syndactyly.
30.3.5. Diseases of the sesamoid
bone
The most frequent pathological disorders
are fractures, avascular necrosis and arthrosis.
Metatarsalgia is the term used to describe the
pain ind uced by load ing of the metata rsals.
Hyperkeratosis is a lso generally present under
the metatarsal heads concerned. The d isorder
usua l ly develops as a resu lt of pes
transversoplanus.
Conservative treatment may prove to be
successful with well-adapted insoles, other­
wise, surgical treatment is recommended.
Metatarsalgia of static origin generally affects
the central (2nd, 3rd and 4th) metatarsi; in
these cases, it is advisable to carry out surgery
on the 2nd-4th rays concurrently (e.g. sub­
capital osteotomy according to Helal), since
osteotomy involving only one ray may lead to
metatarsalgia of the other rays.

30.3.7. Digitus malleus
(hammertoe)
The flexor-extensor balance on the forefoot is
disrupted as a resu lt of the dropping of the
transverse arch, and a characteristic condition
develops. The metatarsophalangeal joint takes
up a hyperextended position, the proximal
interphalangeal (PIP) joint fixes in a flexed po­
sition and the distal interphalangeal (DIP) joint
assumes a n extended position.
A clavus forms on the dorsal side of the
toe, corresponding to the proximal inter­
phalangeal joint as a result of the pressure in­
duced by wearing shoes. A sensitive, irritable
bursa develops between the clavus and the
skin (Fig. 30.27. a, b).
Treatment. In the early stage, the process
may be reversible by the wearing of insoles
and regular toe exercises; later, after the de­
formity becomes rigid, only surgical interven­
tion may yield a good result. Of the several
Fig. 30.27.
a: Schematic illustration of a hammertoe deformity.
b: Hammertoe.
3 0 . D i s o r d e r s of t h e f o o t � 419
possible surgical solutions, the most common
is surgical resection of the proximal phalanx
head.
In neglected cases, when subluxation or
luxation has developed in the metatarso­
phalangeal joint, arthrodesis of the proximal
interphalangeal joint, or possibly capsul­
ectomy in the metatarsophalangeal joint, re­
section of the base of the proximal phalanx or
temporary wire fixation may be necessary.
30.3.8. Morton's neuroma
The essence o f t h i s d isorder is a typica l spin­
d le-shaped neuroma on t he digita l nerve be­
tween the 3rd and 4th, less freq uently be­
tween the 2nd and 3rd metatarsi, located d i­
rectly p roximally from the partition of the
nerve.
Clinically, a sharp, flashing pain occurs in
the supply area of the nerve, after which a per­
sistent pain of lower intensity remains. It is
important to distinguish the disorder from
metatarsalgia, which may be achieved
through a simple physical examination. In
metatarsalgia, the metatarsals are sensitive to
direct pressure from underneath, while the
pain is not induced upon transverse pressure
of the anterior part of the foot. In contrast, in
Morton' s neuralgia, the foot is not sensitive to
pressing of the metatarsals from below, but
the pain may be provoked by transverse com­
pression of the metatarsus heads .
Treatment. Lidocaine infiltration admin­
istered in the area of the neuroma eliminates
the pain immediately, this may partially be
utilized for diagnostic purposes. A local ste­
roid inj ection may be attempted once, or pos­
sibly twice. If no improvement is experi­
enced, removal of the thickened nerve seg­
ment may be considered.
420 � 3 0 . Disorders of t h e foot
30.3.9. March fracture (stress
fracture, fatigue fractures
on the metatarsals)
The term "ma rch fracture" also implies the es­
sence of the i l l ness. It was first described
among soldiers, as a syndrome occu rring after
persistent strain, marching.
The fundamentals of the disorder are stress
fractures which develop most commonly on
the 2nd and 3rd metatarsi, as a result of per­
sisting, repeated strain; these fractures can not
be spotted immediately in the X-ray picture.
A few days later, upon osteolysis of the edges
of the fracture, it can be well distinguished,
and after a few weeks, a large mass of callus
may be observed (See Chapter 3 and Fig. 3 . 5).
The significance of this diagnosis is
chiefly the differential-diagnostic aspect: if it
is not taken into consideration, a turnor may
be suspected. Later, metatarsalgic complaints
may arise subsequent to march fractures.
Treatment. The condition heals spontane­
ously if the legs are rested.
30.3. 10. Achillodynia (paratenonitis
of the Achilles tendon)
This d isorder is cha racterized by pain in the
Achilles tendon and the surrounding
paratenon, possibly accompanied by its thick­
ening.
The condition is characteristic at a young
age, or in male patients who earlier exercised
regularly and resume physical exercise after a
long intermission. The increased strain on the
Achilles tendon causes pain, pressure sensi­
tivity and a palpable, reel-shaped swelling 1 -2
cm above the point where the tendon is at­
tached to the calcaneus. In certain cases, crep­
itation is also observed above the tendon upon
movement of the feet. When further physical
exercise is forced, the total rupture of the
damaged Achilles tendon often occurs.
Differential diagnosis. This disorder
must be distinguished from partial or total
rupture of the Achilles tendon. If a rupture has
occurred, an indent is palpable in the affected
area, and the patient can not support him/her­
self on tiptoe on one foot. The Thomson test
may be of assistance in establishing the cor­
rect diagnosis: with the patient lying in prone
position, in normal cases, compression of the
flexing muscles of the leg results in
plantarflexion of the foot; with a ruptured
Achilles tendon, the foot does not move.
Treatment. This comprises resting, eleva­
tion of the leg and the administration of
nonsteroidal anti-rheumatic drugs. After the
temporary discontinuance of physical exer­
cise, it is very important to recommence and
build up the exercising program gradually,
corresponding to the load-bearing capacity of
the tendon.
Locally administered steroid products
may reduce the discomfort, but they are not
recommended, for they increase the danger of
rupture of the Achilles tendon.
30.3. 1 1. Haglund heel
This disorder involves a bony enlargement
developing in adolescence, after the end of the
growth phase, on the upper part of the dorsal
surface of the calcaneus, in the area where the
Achilles tendon adheres to the bone.
The upper rim of the shoe presses on the
bony enlargement, causing severe discomfort.
This is not genuine exostosis, the disorder is
rather a variation of form, or excessive bone
mass.
Treatment: by means of periodic change of
the shoes and conservative treatment (cold
compresses and nonsteroidal anti-inflamma­
tory cream) the complaints usually cease.
Rarely, in stubborn cases, resection of the
bone excess may be carried out.
30.3. 12. Calcaneus spur
The essence of this disorder is that a small,
spur-like protrusion develops at the origin of

Fig. 30.28.
X-ray picture of a calcaneal spur.
the plantar fascia, originating from the tuber
of the calcaneus, which is sensitive to strain or
direct pressure.
Patients generally complain of a stinging
pain in the heel area, which they compare to
stepping on a sharp pebble.
The sensitive area is well palpable, and the
pain (usually originating at the medial perim­
eter of the calcaneus tuber) can be provoked
by applying pressure. In a lateral X-ray pic­
ture, a spike-shaped protuberance can be ob­
served at the origin of the aponeurosis
plantaris in the maj ority of cases (Fig. 30.28).
Considering that in many cases, a calcaneus
spur is not always seen in spite of the intense
pain, and as a spur may develop without caus­
ing the patient any discomfort, it may be pre­
sumed, that the pain is not induced by the
exostosis, but by the overstraining of the plan­
tar structures and periostitis.
Treatment is generally conservative: in
most cases, relaxation, the wearing of insoles
to relieve the foot and the administration of
anti-inflammatory agents will improve the pa­
tient's condition. Also, local steroid inj ections
often have a good effect. If conservative treat­
ment does not produce acceptable results in
3-6 months, incision of the aponeurosis
plantaris adjacent to the calcaneal origin may
be considered. Recently, by means of an ar­
throscopic intervention, the aponeurosis can
be detached through a minimally invasive
procedure as an outpatient operation.
3 0 . D i s o r d e r s o f t h e foot � 421
30.3.13. Arthrosis of the foot joints
This d isorder develops most frequently in the
subtalar joint and Chopart's joint. Primary
a rthrosis occurs in elderly patients. The sig nifi­
cance of secondary a rthrosis is higher. These
cases a re genera l ly recognised as end stages
of progressive prearthrotic conditions (pes
excavatus, clu bfoot, etc.) o r as a conseq uence
of some other d isease (inflammation, aseptic
bone necrosis, or trauma).
Clinical symptoms. Pain localized in the
arthrotic area causes discomfort on movement
of the affected joint. The area is sensitive to
pressure, and osteophyte formation is often
palpable upon external examination.
Radiologically, narrowing of the joint
space is characteristic in osteoarthritis, with
subchondral sclerosis, subchondral cysts and
osteophytes.
Treatment. In mild cases, non steroidal
anti-rheumatic agents, symptomatic treatment
and therapeutic X-ray irradiation may be
highly effective. Orthopedic shoes may be
recommended later. In case conservative
treatment does not prove successful, surgical
treatment may be considered, with arthrodesis
of the arthrotic joint. When the entire tarsus is
affected, combined arthrodesis (triple
arthrodesis) of the talocalcaneal j oint and
Chopart ' s j oint is performed. It is important to
distinguish arthrosis from diabetes-related
arthropathy, which in its early stage may dis­
play a picture similar to that of conventional
osteoarthritis, but demonstrates only a few of
the clinical symptoms of the disorder.
30.3. 14. Tarsal tunnel syndrome
This involves compression of the tibial
nerve on the inside of the medial ankle at the
entrance of the tarsal tunnel . The mechanism
of its development corresponds to that of the
carpal tunnel syndrome. The causes of the dis­
order may be any of the following : inflamma­
tion, trauma, a venous circulation disorder, or
422 � 3 0 . D i s o rders of the foot
functional reasons. Patients describe nightly
paresthesia on the sole of the foot, around
their toes. The symptoms can be provoked by
applying direct pressure to the tarsal tunnel.
An EMG examination may help establish the
diagnosis.
Treatment. Conservative treatment, shoe
inserts to support the medial side of the sole,
and the administration of non steroidal
anti-inflammatory drugs lead to an improve­
ment. Rarely, surgical decompression may be
indicated.
30.3. 15. Diabetic foot
Diabetic foot is a collective term for foot
p roblems relating to diabetes: macro and
microangiopathy and foot deformities devel­
oping as a conseq uence of neuro pathy, ac­
companied by u lcers o r necrosis in severe
cases.
The nutrition and oxygenization of the tis­
sues is diminished because of the impaired
blood circulation, and wounds therefore heal
with greater difficulty, than under normal
conditions. Because of the sensation disorders
developing as an accessory symptom of the
neuropathy, minor injuries of the foot do not
Fig. 30.29.
Characteristic symptoms of a diabetic foot: the skin
is d ry and cracked, the toenails are dry and are
mycosis-infected, and a circulation disorder in an
early stage may be perceived u nder the 1st
metatarsus head.
attract attention, until more severe complaints
occur, resulting from the disorder not having
been treated (Fig. 30.29.). Clinical experience
shows that certain diabetic ulcers develop not
on the sole (under pressure), but on the dor­
sum ofthe foot, this proving that ulcers are not
caused merely by pressure. It is most impor­
tant to diagnose the neuropathy and also to
prevent it. The risk factors of ulcers develop­
ing on the diabetic foot are as follows :
- retinopathy,
- old age,
- previous foot ulcers,
- alcohol consumption,
- smoking,
- known cardiac disease,
- foot deformity (hallux valgus,
hammertoe, etc.),
- limited joint mobility.
The following measures may be recom­
mended as forms of prevention. The diabetic
patient should wash hislher feet regularly with
warm water. The temperature of the water
should be checked before stepping into it. The
patient should examine hislher soles every
day with the help of a mirror. Drying-out of
the skin should be avoided by the application
of skin cream. Before putting on shoes, the pa­
tient should ascertain that there are no wrin­
kles in the socks and no other pressure-induc­
ing obj ects are present. The patient should not
walk barefoot or wear new shoes for long pe­
riods. If the patient perceives any problems,
he/she should turn to a physician immedi­
ately, and should not treat the complaint at
home. The development of ulcers and rapid
progression of the infection is a serious risk in
case of diabetic feet and in certain cases may
lead to amputation of the foot. It is most im­
portant, therefore, to control the patient's
blood sugar level. From an orthopedic aspect,
total contact shoe inserts and the provision of
diabetic orthopedic shoes are of great signifi­
cance as concerns the avoidance of the devel­
opment of ulcers and other complications .
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