Diseases title

Ulnar hypoplasia - split foot )

Chrom mut
Orpha Number: 1122
Synonym(s):
Ulnar hypoplasia - lobster-claw deformity of feet, Van den Berghe-Dequecker syndrome

This syndrome is characterised by the association of severe ulnar hypoplasia, absence of fingers two to five, and split-foot. It has been described in four males belonging to two generations of the same family. X-linked recessive inheritance is suggested, but autosomal dominant transmission cannot be excluded.
source: Orphanet