2. Fibular hemimelia, also known as congenital
absence of the fibula, congenital deficiency of the
fibula, paraxial fibular hemimelia, and aplasia or
hypoplasia of the fibula, is the most common long
bone deficiency
3. Some have recently postulated that interference
with limb-bud development plays an important role
in postaxial hypoplasia of the lower extremity.
During the fetal period, the fibular field of the limb
bud controls development of the proximal femur,
explaining the frequent association of femoral
abnormalities
4. Involved limb is usually shortened, and the foot is in
equinovalgus position
Classically the skin has dimpling seen over the tibia
Clinically, primary problems related to fibular
hemimelia are limb length inequality and foot/ankle
instability
5. Proximal femoral focal deficiency (PFFD)
Coxa vara
Femoral hypoplasia with external rotation
Lateral patella subluxation
Hypoplastic lateral femoral condyle
Genu valgus with lateral mechanical axis
displacement
6. Flattened tibial eminence with absent cruciate and
a positive Lachman sign
Short and/or bowed tibia
Equino valgus at ankle
Ball-and-socket ankle
Absent tarsal bones
Tarsal coalitions
Absent foot rays
7. Type 1 deformity -hypoplasia of the fibula
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
11. The goals of treatment are equalization of limb
length and correction of the foot deformity
Conservative management- No treatment or
the use of heel lifts may be adequate, particularly
in discrepancies less than 2 cm
12. Epiphysiodesis of the normal leg is performed
at the appropriate time so that leg lengths are
equal at the end of skeletal growth
Gruca procedure- Rarely is indicated and
should be used only when the foot is to be
salvaged and the ankle requires stabilization.
13.
14. Syme’s Amputation and prosthetic
rehabilitation- When limb-length discrepancy is
predicted to be more than 12 to 15 cm and the
foot is deformed.
The advantages of early amputation include fewer
hospitalizations and surgical procedures. Children
who undergo amputation at an early age show
excellent emotional adaptation to their disability
and have good functional results
15. Both posterior and lateral releases are required.
The tendo calcaneus, as well as the
fibrocartilaginous anlage of the absent fibula, must
be released
Ankle valgus can be corrected with a dome or
varus supramalleolar osteotomy
