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Fibular hemimelia

Fibular hemimelia is a congenital absence or deficiency of the fibula that is associated with other lower limb abnormalities. It results from interference with limb bud development during fetal development. Clinically, it presents with a shortened limb and foot deformities like equinovalgus, as well as leg length discrepancy and foot/ankle instability. Treatment options range from conservative management with heel lifts for small discrepancies, to epiphysiodesis or amputation for larger discrepancies. Surgical treatment may involve osteotomies, tendon releases, or procedures like a Gruca or Syme's amputation.

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 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
 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
 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
 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
 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
 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
Type 2 deformity -Complete absence of the fibula.
 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
 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.
 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
 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
Fibular hemimelia
Fibular hemimelia