Orthopedics > OCOSH Classification > Musculoskeletal Abnormalities > Congenital Limb Deformities > Unilateral bifid femur with monodactylous ectrodactyly OMIM

< Previous

Unilateral bifid femur with monodactylous ectrodactyly OMIM

Visit Resource Review It Rate It Bookmark It

Location: http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=228250

Description: %228250 FEMUR, UNILATERAL BIFID, WITH MONODACTYLOUS ECTRODACTYLY
Alternative titles;
GOLLOP-WOLFGANG COMPLEX; GWC
Gollop et al. (1980) described 2 brothers, each with 1 normal upper limb; 1 had tridactylous ectrodactyly of 1 hand with normal radius and ulna, and the other had monodactyly of 1 hand with absent ulna. Both had monodactyly of the feet, absence of the tibias, and unilateral bifurcation of the femur. A sister of the paternal grandfather was purportedly similarly affected. The mode of inheritance was considered unclear. Kohn et al. (1989) reported 2 unrelated families, each with 2 affected offspring with bifid femur, absent tibia, and ectrodactyly. In each family, the healthy parents were consanguineous.

Type: Reference Material
Author/Contact: Victor A. McKusick
Institution: OMIM
Primary Subject/Category:

Language: English

Submitted by: admin
Hits: 645
Added: Sat Aug 08 2009