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Congenital Limb

Length Discrepancy

By
Dr Frazand Ali
PG-Trainee Orthopedics
LGH Lahore
Limb Length Discrepancy
 Difference between the
length of upper and
lower arm / upper and
lower leg is called “Limb
Length Discrepancy”
(LLD)
Causes
Congenital : Infections
 Femoral deficiency  Poliomyelitis
 Coxa vara  Septic arthritis
 Fibular hemimelia  Osteomylitis leading
 Tibial hemimelia to growth plate
 Psuedarthrosis of tibia damage
 Neurofibromatosis Neurological
 Cerebral palsy
Trauma
 Over riding fracture Tumour
 Multiple exostosis
 Epiphyseal injuries
leading to shortening Inflamatory
 Rheumatoid arthritis
Signs and Symptoms
 Limping gait
 Unappealing shoe lift
 Low back ache
 Compensatory scoliosis
 Degenerative arthrosis of lumbar and
sacral region
Compensation and
Tolerance
 Different Patients respond differently
to LLD depending upon age, height
and body weight e.t.c.
 children tolerate discrepancies better
than adults because of their inherent
flexibility
 a 6-foot-tall patient tolerates a 2-cm
discrepancy with little or no trouble,
whereas a 5-foot-tall patient would
be less tolerant of the same
discrepancy.
Clinical Examination

 Limb shortening
 The relative knee heights are measured
with the hips and knees flexed.

Femoral length Tibial length


 Neuromuscular:( muscle wastage on
effected side and contractures )

 Joint examination is necessary


Analyzing Gait
The patient's gait is
evaluated for the
compensatory
mechanisms i.e.
 Pelvic tilting
 Long knee flexion
 Pelvic internal
rotation (less
frequently)
 Equines foot
Radiologic
Assessment
 History and clinical
examination may not
always allow for an
accurate diagnosis
 scanogram, aids in
making a complete
diagnosis
 In growing children, a
scanogram should be
accompanied by a bone
age film of the wrist to
Tibial hemimelia
 First described by Otto in 1941

 congenital longitudinal
deficiency of the tibia,
congenital dysplasia of the tibia,
paraxial tibial hemimelia, tibial
dysplasia, and congenital
deficiency or absence of the
tibia.
Causes
 Exact cause is unknown,
 Sweet and Lane described a
murine model for tibial hemimelia in
which the dominant mutation resides
on the X chromosome.
Classification
 Jones, Barnes, and Lloyd-Roberts
classification which is based on
the early roentgenographic
presentation;

 In type 1A deformity there is a


complete roentgenographic
absence of the tibia and a
hypoplastic distal femoral
epiphysis

 In type 1B deformity there also


is no roentgenographic evidence
of a tibia, but the distal femoral
epiphysis appears more normal
in size and shape.
 In type 2 deformity a proximal
tibia of varying size is present at
birth. The fibula usually is normal
in size, but the head is proximally
dislocated

 Type 3 deformity, in which the


proximal tibia is not
roentgenographically visible, is
rare. The distal femoral epiphysis
usually iswell formed, but the
upper end of the fibula is
proximally dislocated

 Type 4 (rare) deformity, the tibia


is shortened and there is a
proximal migration of the fibula
with distal tibial fibular diastasis
Treatment
 Goal of treatment is a
functional limb equal in
length to the normal limb
 Type 1A deformities are
most frequently treated
with knee disarticulation
and sometime
reconstruction.
 Brown described
reconstruction of type 1A
tibial hemimelia by
surgically transferred of
fibula into the
intercondylar notch to
create a tibia
 Success of Brown
procedure depends upon
presence of quadriceps
mechanism and absence of
knee contractures
 In type 1B and type
2 deformities a
functional knee joint
exists, and knee
disarticulation is not
required if the
quadriceps
mechanism intact
 A proximal tibiofibular
synostosis combined
with a Syme
amputation or distal
reconstruction is the
treatment of choice
 Type 3 patients function
well as below-knee
amputees
 Type 4 deficiencies,
treatment must be
individualized. Syme
amputation provides
excellent function
 Most patients can be
treatedwith combinations
of distal tibiofibular
synostosis and distal
fibular epiphysiodesis.
 Equinovarus deformities of
the foot, if present, require
soft tissue releases.
Fibular Hemimelia
 Also known as
– congenital absence of the fibula,
– congenital deficiency of the fibula,
– paraxial fibular hemimelia,
– aplasia
– hypoplasia of the fibula.

Cause ------------unknown
Presenting Complaints
 leg-length discrepancy with
equinovalgus deformity of the foot
 flexion contracture of the knee
 femoral shortening
 instability of the knee and ankle
 a stiff hindfoot with absent lateral
rays
Classification

 Achterman and Kalamchi

– type 1 deformity (hypoplasia


of the fibula)

– type 2 deformity (complete


absence of the fibula).
 Type 1 deformities are
further subdivided into
types 1A and 1B.
 type 1A, the proximal
fibular epiphysis is distal to
the proximal tibial
epiphysis and the distal
fibular epiphysis is
proximal to the talar dome.
type 1B, the deficiency of
the fibula is more severe,
with 30% to 50% of the
length missing and no
distal support for the ankle
joint
Treatment
 Aims
– Equalize the limb length
– Correction of foot deformity
– Shoe lift and epiphysiodesis of normal
leg
– Syme amputation and prosthetic
rehabilitation when limb length
discrepancy is predicted more then 12-
15 cm and foot is deformed.
Proximal Femoral Focal
Deficiency
 PFFD consists of a partial skeletal defect in
the proximal femur with a variably
unstable hip joint, shortening, and
associated other anomalies.e.g
– Fibular hemimelia and agenesis of the cruciate
ligaments of the knee
– clubfoot,
– congenital heart anomalies,
– spinal dysplasia,
– facial dysplasias
Classification
 Aitken's four-part
classification
scheme
– A,B,C,D

– Class A there is a
normal acetabulum
and femoral head
with shortening of
the femur and
absence of the
femoral neck on
early
 Class B there is no
bony connection
between the
proximal femur
and the femoral
head, and a
pseudarthrosis is
present
 Class C there is
further degradation
in the formation of
the hip,
characterized by a
dysplastic
acetabulum,
absent femoral
head, and short
femur
 Class D the
acetabulum,
femoral head, and
proximal femur are
totally absent
unlike in class C,
there is no ossified
tuft capping the
proximal femur.
.
Kalamchi et al developed a simplified
classification scheme for congenital
deficiency of the femur that included five
groups:
 group I, short femur and intact hip joint;
 group II, short femur and coxa vara of the
hip
 group III, short femur but well-developed
acetabulum and femoral head;
 group IV, absent hip joint and dysplastic
femoral segment
 group V, total absence of the femur.
Nine Pappas Classification of
Congenital Abnormalities of the
Femur
 Class I Femur absent
Ischiopubic bone structures
Underdeveloped and
deficient Lack of Acetabular
development
 Class II(Aitken D) Femoral
head absent Ischiopubic
bone structures delayed in
ossification
 Class III(Aitken B) No
osseous connection between
femoral shaft and head
Femoral head ossification
 Class IV(Aitken A) Femoral
head and shaft joined by
irregular calcification in
Fibrocartilaginous matrix

 Class V (AitkenA) Femur


Incompletely ossified,
hypoplastic, and irregular
midshaft of femur abnormal

 Class VI Distal femur short,


irregular, and hypoplastic
irregular distal femoral
diaphysis
 Class VII Coxa vara
hypoplastic femur proximal
femoral diaphysis irregular
with thickened cortex lateral

 Class VIII Coxa valga


hypoplastic femur femoral
head and neck smaller
proximal femoral physis
horizontal abnormality

 Class IX Hypoplastic femur


Treatment

 Many methods of limb equalization


are available for treating LLD
a) Surgical
b) Nonsurgical)
Nonsurgical Treatment
 A 1-cm lift can fit
comfortably inside of
the shoe;.
 Lifts placed on the
sole of the shoe
function well up to
approximately 3 cm.
 Beyond this, the shoe
becomes heavy and
awkward
Surgical Shortening
Accomplished in one of two ways:
 (1) the physeal growth center can be
retarded or arrested prematurely by
epiphysiodesis (growth plate arrest),
or
 (2) the long bone can be shortened
by resecting a segment of the bone.
Epiphysiodesis
 In 1933, Phemister (9)
described
epiphysiodesis as a
technique to equalize
discrepancies of 2 cm
to 5 cm;
 In recent years,
percutaneous
epiphsiodesis using
transphyseal screw
(PETS)techniques
have replaced
Phemister's method
Epiphysiodesis
Bone Shortening
(Resection)
Reserved for patients
 not candidates for limb
lengthening,
 do not wish to undergo
lengthening
 are skeletally too mature
for epiphysiodesis
 In femur 5-6 cm
shortening can be done
without seriously effecting
soft tissues.
Tibial shortening osteotomy

In tibia 2-3cm shortening can be perform


Surgical Lengthening
Increasing bone length has been attempted
by a variety of methods, including
 creating arteriovenous shunts,
 implanting foreign material under the
epiphysis,
 stripping the adjacent periosteum,
 ganglionectomy,
 mechanical distraction.

Of these, only mechanical distraction is


a practical method of limb
lengthening
Mechanical Bone
Lengthening
 Mechanical bone lengthening
was first reported by Codivilla
in 1905.
 The lengthening site was
held in place with plaster and
a fraction pin
 In recent years, technical
advances in limb lengthening
have focused on the
development of external
fixators that allow for weight
bearing and maintenance of
joint function during gradual
 In the past 15
years, the primary
advancement in
limb lengthening
has been the
method described
by Ilizarov, whose
biologic principle of
distraction
osteogenesis has
revolutionized limb
lengthening.
Rotational osteotomy
 Van-nes described
below knee
rotational 180
degree osteotomy
Precautions
 If shortening is of 12-14 cm lengthening
can be perform in stages that is first at 4-5
years and second 8-9 years age
 Should done before contracture developed
 Not recommended before 5 years of age
due to small bone size
 Before treatment Features including
scoliosis, pelvic obliquity, contractures,
dysplasias, and angular deformities must
be identified
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