Femoral bifurcation and bilateral tibial hemimelia: case report

Abstract

Femoral bifurcation and tibial hemimelia are rare anomalies described as a variant of Gollop-Wolfgang complex. This article presents a case of Gollop-Wolfgang complex without hand ectrodactyly. A 5-year old patient presented with bilateral tibial hemimelia and left femoral bifurcation. The patient's left limb lacked knee extensor mechanism, disarticulation was done. The right leg which had Jones type 2 tibia hemimelia was treated with tibiofibular synostosis. Currently patient is ambulant with prosthesis on the left limb and ankle foot orthosis on the right. In the absence of proximal tibial anlage, especially in patients with femoral bifurcation, the knee should be disarticulated. Tibiofibular synostosis is a good choice in the presence of a proximal tibial anlage with good quadriceps function.

Deformity Reconstruction Surgery for Tibial Hemimelia

“…Ipsilateral deformities at the thigh and knee include congenital femoral deficiency, a missing patella or quadriceps extensor mechanism, knee hyperextension or flexion, and a bifid femur. Foot deformities include clubfeet, syndactyly, missing or duplicated toes, diplopodia, ectrodactyly, micromelia, and a mirror foot deformity [35][36][37][38][39][40][41][42][43]. Other associated deformities include coxa valga, hip dysplasia or dislocation, radial dysplasia, lobster claw deformity, hand syndactyly and polydactyly, triphalagism, mirror hand, missing fingers or toes, hemivertebrae, and myelomeningocele [44][45][46][47].…”

Early Outcome After Limb Salvage Surgery of a Rare Case of Gollop-Wolfgang Complex in a Limited Resource Setting in Africa

“…Several associations between GWC and maternal use of antiepileptic drugs, most notably that of sodium valproate and cabamazepines, have been documented previously [7,8]. The best treatment option for patients with GWC is early knee disarticulation and resection of the protruded bifurcated femur followed by fitting of a modern prosthesis [9][10][11].…”

“…Few cases of GWC have been reported in sub Saharan Africa and most of them were treated by amputation and prosthetic replacement [9,11,12]. Amputation is not sometimes accepted by parents for many reasons; therefore, alternatives to amputation should be considered [13].…”

“…However, there were no other malformations recorded on examination. Gollop Wolfgang complex is listed as a "rare disease" by the United States Office of Rare Diseases (ORD) of the National Institute of Health (NIH) [1], and there are only few cases reported in Africa [9,11,12].…”

“…Surgical correction of GWC is still very difficult and does not have a predefined protocol or procedure [12][13][14]. The best treatment option for patients with GWC is early knee disarticulation and resection of the protruded bifurcated femur followed by fitting of a modern prosthesis [9][10][11]. Those with complete tibial hemimelia like in this case have an uncertain longterm outcome and require intensive post-operative rehabilitation.…”