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Congenital Limb

Length Discrepancy

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”
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
 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
 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
 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
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
mechanisms i.e.
 Pelvic tilting
 Long knee flexion
 Pelvic internal
rotation (less
 Equines foot
 History and clinical
examination may not
always allow for an
accurate diagnosis
 scanogram, aids in
making a complete
 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
 Exact cause is unknown,
 Sweet and Lane described a
murine model for tibial hemimelia in
which the dominant mutation resides
on the X chromosome.
 Jones, Barnes, and Lloyd-Roberts
classification which is based on
the early roentgenographic

 In type 1A deformity there is a

complete roentgenographic
absence of the tibia and a
hypoplastic distal femoral

 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

 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
 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
 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
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
 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

 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
 Aims
– Equalize the limb length
– Correction of foot deformity
– Shoe lift and epiphysiodesis of normal
– Syme amputation and prosthetic
rehabilitation when limb length
discrepancy is predicted more then 12-
15 cm and foot is deformed.
Proximal Femoral Focal
 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
 Aitken's four-part
– 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
 Class B there is no
bony connection
between the
proximal femur
and the femoral
head, and a
pseudarthrosis is
 Class C there is
further degradation
in the formation of
the hip,
characterized by a
absent femoral
head, and short
 Class D the
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
 group I, short femur and intact hip joint;
 group II, short femur and coxa vara of the
 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
 Class I Femur absent
Ischiopubic bone structures
Underdeveloped and
deficient Lack of Acetabular
 Class II(Aitken D) Femoral
head absent Ischiopubic
bone structures delayed in
 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
 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


 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
Surgical Shortening
Accomplished in one of two ways:
 (1) the physeal growth center can be
retarded or arrested prematurely by
epiphysiodesis (growth plate arrest),
 (2) the long bone can be shortened
by resecting a segment of the bone.
 In 1933, Phemister (9)
epiphysiodesis as a
technique to equalize
discrepancies of 2 cm
to 5 cm;
 In recent years,
epiphsiodesis using
transphyseal screw
have replaced
Phemister's method
Bone Shortening
Reserved for patients
 not candidates for limb
 do not wish to undergo
 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
 stripping the adjacent periosteum,
 ganglionectomy,
 mechanical distraction.

Of these, only mechanical distraction is

a practical method of limb
Mechanical Bone
 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
osteogenesis has
revolutionized limb
Rotational osteotomy
 Van-nes described
below knee
rotational 180
degree osteotomy
 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