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Upper and lower extremity anomalies.
Day of life 15
AP radiographs of the right and left upper extremities demostrate a cleft hand on the left and only 1 digit on the right with right ulnar hemimelia (partial absence of the distal ulna). Lower extremity radiographs obtained at 15 days of age demonstrate bilateral absence of the tibias, unossified epiphyses of the distal femurs, narrow distal femoral metaphyses of a bifid right femur.
1 year of age
At 1 year of age patient underwent bilateral knee disarticulation and excision of the medial portion of the bifid right femur. Photographs of the gross specimen of the left lower extremity demonstrates 4 digits present. Photograph of the gross specimen of the right lower extremity demonstrates 3 digits with syndactyly of digits #1 and #2. There is bilateral foot equinovarus.
The left distal femur is partially bifid as well, as demonstrated by the appearance of two separate ossification centers in the expected region of the left distal femoral epiphysis at 18 months of age (coned-down radiograph of the left distal femur, arrows).
Gollop-Wolfgang complex is a very rare malformation characterized by a combination of femoral bifurcation with hand ectrodactyly. Approximately 200 cases have been reported in the literature. Bifid femur (most often unilateral), tibial hemimelia (congenital aplasia or hypoplasia of the tibia), clubfeet, oligodactyly or monodactyly of the feet and hand ectrodactyly may be present unilaterally or bilaterally. Both autosomal dominant and recessive inheritance have been suggested, however the precise etiology remains unknown.
- 1. Kaplan-List, K., Sanders, J., Klionsky, N. et al. Systematic Radiographic Evaluation of Patients with Tibial Hemimelia Relevant to the Orthopedic Surgeon. Poster #: EDU-005 (Society of Paediatric Radiology) Pediatr Radiol (2015) 45(1):S174-175.
- 2. Wolfgang G. Complex Congenital Anomalies of the Lower Extremities: Femoral Bifurcation, Tibial Hemimelia, and Diastasis of the Ankle. Case Report and Review of the Literature. J Bone Joint Surg Am. 1984;66(3):453-8. - Pubmed
- 3. Gollop T, Lucchesi E, Martins R, Nione A. Familial Occurrence of Bifid Femur and Monodactylous Ectrodactyly. Am J Med Genet. 1980;7(3):319-22. doi:10.1002/ajmg.1320070313 - Pubmed