Gollop-Wolfgang complex

Case contributed by Dr Katia Kaplan-List

Presentation

15-day-old male born full term via cesarean-section with upper and lower extremity anomalies.

Patient Data

Age: 15 day old male
Gender: Male

Day of life 15

Modality: X-ray

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

Modality: Pathology

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.

18 months

Modality: X-ray

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).

Case Discussion

GOLLOP-WOLFGANG COMPLEX; OMIM entry 228250.

Gollop-Wolfgang complex (GWC) is 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 bilterally. The etiology remains unknown. Both autosomal dominant and autosomal recessive inheritance have been suggested, however the etiology still remains unknown.

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Case Information

rID: 40932
Case created: 8th Nov 2015
Last edited: 8th Nov 2015
Inclusion in quiz mode: Included

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