Discuss the Orthopaedic

Manifestations of SCD &

their Management
Dr. Arojuraye S.A
National Orthopaedic Hospital
Dala-Kano
 Outline

 Introduction
 Pathophysiology
 Orthopaedic manifestations
 Management
 Conclusion
 Introduction

 SCD = Inherited disorder due to HbS

 Substitution of valine for glutamic acid at 6th a.a of the
ß globin chain
 Inheritance = autosomal recessive
 Major genotypes = SS, SC & sickle-beta thalassemia
 HbS is poorly soluble
 It assumes sickle shape when deoxygenated
 Introduction…

Incidence
 Black American

 AS = 8 – 10%
 SS = 0.2 - 0.5%

 West African
 AS = 20%
 Introduction…

Diagnosis
 Peripheral blood smear = sickle rbc

 Hb electrophoresis = abnormal haemoglobins

 Diagnosis of the major genotypes = simple

 Differentiation of various subtypes = complex

 Introduction…
 1910: First description of SCD was made in Chicago
by Herrick.

 1927: Hahn & Gillespie showed that sickling of the

rbc was induced by deoxygenation & reversed with
reoxygenation.

 1949: Electrophoretic abnormalities of Hb were

demonstrated by Pauling et al
 Pathophysiology
 Hypoxia & dehydration
 Sickling
 Membrane distortion
 Rbc stickiness

Haemolysis &
Vaso-occlusion
 Pathophysiology…

 Haemolysis results from

destruction of the sickled
rbc by monocytes &
macrophages.

 Vaso-occlusion is due to
entrapment of sickled cells
in the microcirculation =
ischaemia.
Pathophysiology…
 Clinical manifestations

 CNS: CVA, meningitis

 Ocular: Retinopathy

 Pulmonary: Acute chest syndrome, pneumonia,

restrictive lung disease
 Clinical manifestations…
 Renal: Haematuria, nephropathy

 Spleen: Massive splenomegaly, autosplenectomy

 Biliary: Cholelithiasis, cholecystitis

 CVS: HF, MI, cor pulmonale

 Orthopaedic manifestations

Acute Chronic
 Vaso-occlusive crisis  Osteonecrosis

 Osteomyelitis  Chronic arthritis

 Septic arthritis  Chronic osteomyelitis

 Stress fracture  Osteoporosis

 Vertebral collapse  Impaired growth

 Vaso-occlusive crisis
VOC
 Affects virtually all patients

 Begins in late infancy & recurs throughout life

 Can occur in any organ

 Particularly common in bone marrow

 Vaso-occlusive crisis…

Clinical presentation
 Intense pain

 Localized tenderness

 Swelling & erythema

 Febrile episodes

 Juxta-articlar area = joint effusion

 Leucocytosis are also common

 Most patients recover with no complications

 Vaso-occlusive crisis…

Plain radiograph
 Diagnosis is clinical

 Not useful in acute phase

 Later: subchondral &

intramedullary lucency &
patchy sclerosis
 Vaso-occlusive crisis…

MRI
Radioisotope scan
 Very sensitive

 Not specific (Infarction vs OM)

 Not routinely used

 Vaso-occlusive crisis…

Dactylitis (hand-foot syndrome)

 Described by Danford

 Subsequently Xterized by Smith

 Under 5yrs (typically 6 – 18months)

 Small bones of hands & feet

 Vaso-occlusive crisis…

Dactylitis
 Clinically; acute, painful swelling

 Erythema & warmth

 Most episodes resolve within 2 weeks

 Epiphyses = premature fusion & shortened fingers

 Vaso-occlusive crisis…

Dactylitis
 Radiological evidence of
new bone (2weeks)

 ‘Moth-eaten’ appearance
bcs of cortical thinning &
irregular attenuation of
the medullary spaces
 Vaso-occlusive crisis…

Dactylitis
 Histology:

 Infarction of the marrow, medullary trabeculae &

inner layer of the cortex

 Subperiosteal new bone formation

 Vaso-occlusive crisis…

Treatment
 Infection, dehydration, acidosis, hypoxia, cold,

 IVF & analgesia

 Paracetamol
 NSAIDs
 Opioid derivatives
 Antibiotics
 Osteomyelitis
 90% of SCD develop OM before 10yrs
 No bone is exempted
 Multifocal
 Precipitated by VOC

Risk factors in SCD

 Hyposplenism

 Impaired complement activity

 Infarcted or necrotic bone

 Osteomyelitis

Common organisms
 Staphylococcus aureus

 Salmonella (Hodges & Holt, 1951)

 Osteomyelitis …

Diagnosis
 Common mgt dilemma

 Failure = life threatening infection

 Erroneous diagnosis = unnecessary 6wks of antibiotics

 Pain, swelling, tenderness & pseudoparalysis

 Most common sites: Femur, tibia & humerus
 Often affect diaphysis
 Features are similar to those of VOC
 Osteomyelitis …

Plain radiograph
 Always first step

 Not diagnostic

 May exclude other dxs

 Bony changes in 2-3wks

 Osteomyelitis …

USS
 Soft tissue disturbance

 Subperiosteal collection

 Useful for aspiration

 Osteomyelitis …

 Bone scan
 MRI
 Osteomyelitis …
Treatment
 Based on organism

 3rd generation cephalosporin

 Ciprofloxacin in older children

 Drainage

 Bone drilling

 Once AO COM
 Conservative Rx, until involucrum is formed
 Sequestrectomy
 Septic arthritis

 Not common
 Tends to occur with painful VOC
 Aetiology as in OM
 Salmonella arthropathy is very rare
 Septic arthritis…

Rx
 Early aggressive drainage, debridement & splintage

 A two-week course of IV antibiotic

 Osteonecrosis

AVN
 Most common complication

 More common in SC

 > 50% of SCD > 30yrs

 M:F = 1
 Osteonecrosis…

Presentation
 There may be hx of trauma

 Pain, limited motion

 Occasionally with pain at rest

 May be asymptomatic (shoulder)

 Osteonecrosis…

Common sites
 Femoral heads

 Head of the humerus

 Knee joint

 Small joints of the hands & feet

 Common to have multiple joints affected

 Hip: bilateral in >50%

 Shoulder : 74% have AVN of HOF

 Osteonecrosis…

Plain radiographs
 Mottled attenuation of
the epiphysis
 Subchondral lucency

 Flattening/collapse of
the articular surfaces
 Narrow joint space

 Articular sclerosis &

osteophyte formation
 Osteonecrosis…

MRI
 Best for early disease

 Double line sign

 Osteonecrosis…

 Untreated, 87% of HOF will collapse within 5 years

 Bed rest & symptomatic Rx: unacceptable
 Early dx: Core decompression & osteotomy
 Late dx: Arthroplasty

SCD patients must be cared for in specialized centres

with expertise in SCD as they have a very high
incidence of perioperative complications
 Osteopaenia & Osteoporosis

 Reduced bone mineral density (BM hyperplasia)

 Common in the vertebrae
 Vertebral collapse
 Osteopaenia & Osteoporosis…

Radiograph
 Increased radiolucency

 Prominence of vertebral trabeculae

 Smooth, biconcave vertebrae

 ‘Fish-mouth’ vertebrae
 Growth disturbance

BM hyperplasia
 Vertebrae

 Ischaemia of the
vertebral growth plate
 Disturbance of
vertebral growth
 Characteristic ‘H’
shaped vertebrae
 Some develop ‘tower’
vertebra
 Growth disturbance…

 Long bones
 Premature closure of
epiphyses
 Impaired growth of the
long bones
 Conclusion
 SCD prognosis is still poor
 Orthopaedic manifestations: major cause of morbidity
 Mgt of its orthopaedic complications is challenging
 Necrosis & infections are responsible for major
functional impairment
 Surgery is the mainstay of Rx of these complications
While waiting for new genetic therapy for SCD, the
surgeon will treat the complications but should keep
in mind that anaesthesia is more risky for these
patients .
 References
 Dgere A, Ndjoko R, Docquier P, Mousny M, Rombouts J. Orthopaedic
complications associated with sickle-cell disease. Acta Orthop. Belg., 2006, 72,
741-747.
 Antonio A, Irene R. Bone involvement in sickle cell disease. British J Haemat,
2005; 129:482–490
 Michael HH, Gary EF, James SM. Orthopeedic Manifestations of Sickle-Cell
Disease. J Bio Med, 1990; 63:195-207
 Louis S. Osteonecrosis and related disorders. Apley’s system of orthopaedics
and fractures, 9th ed; Hodder Arnold, 2010; 6: 103 – 15.
 Archampong EQ, Addo AO. Surgical aspects of the hemoglobinopathies.
Principles and practice of surgery including pathology in the tropics, 4th ed;
51: 1015 – 25.
 Geraldo B, Elizabeth D, Francisco A. Osteoarticular involvement in sickle cell
disease. Review article. Rev Bras Hematol Hemoter. 2012;34(2):156-64