Research Day

Title

Lower Extremity Surgical Treatment to Improve Function in a Patient with Gollop Wolfgang Complex

Document Type

Abstract

Date

2017

Abstract

Introduction: Gollop-Wolfgang complex is a very rare limb malformation with a bifurcation of the distal femur associated with ipsilateral tibial aplasia. Case Description: Patient was an 18 month old male with Gollop-Wolfgang complex and VACTERL association. Orthopaedic manifestations included: left bifid femur with ipsilateral complete tibial hemimelia, hypoplastic bilateral thumbs, and right tarsal-type pre-axial polydactyly of the foot. No extensor mechanism was present at the left knee. The left foot had a varus and supination deformity. He had not started walking, crawling, or pulling to stand secondary to his deformity. The goal of treatment was to create a functional left leg. Given the absent tibia and extensor mechanism, bifid femur, and foot deformity the most predictable outcome was expected with a through-knee amputation with subsequent prosthetic fitting. The knee was disarticulated to allow continued growth of the femur. The non-functional branch of the bifid femur was resected. An osteotomy was performed to correct alignment of the remaining femur. An adductor myodesis was performed to allow for proper muscle balance at the hip. The pre-axial polydactyly was also excised from the contralateral foot. Tendon transfers to improve thumb function will be performed in the future. The patient was placed in a cast to allow for soft tissue and bone healing. Unfortunately, the patient developed a wound infection of his left leg. Multiple irrigation and debridement procedures with negative pressure wound therapy were performed. After the infection was cleared, a split thickness skin graft was performed without complication. A prosthesis was manufactured and he has been participating in physical therapy. He is currently 31 months old and is walking with a walker using a left prosthesis and right supramalleolar orthosis. Discussion: Gollop-Wolfgang complex was first described in 1980. It is classically treated with a knee disarticulation and prosthetic fitting. There are some reported cases of limb salvage secondary to absence of parental approval for amputation. Results have not been as predictable with salvage; especially if there is complete tibial aplasia and no extensor mechanism. This case reinforces the use of amputation as a viable treatment option for patients with Gollop-Wolfgang complex. Conclusion: Through-knee amputation and prosthetic fitting can provide a functional limb for patients with a bifid distal femur, complete tibial aplasia, and no extensor mechanism.

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