Ulnar aplasia - split foot syndrome

[MIM 314 600]

(Van den Berghe-Dequeker syndrome, ulnar hypoplasia with lobster-claw deformity of feet)

Only a few cases have been reported. Association of a severe ulnar hypoplasia with absence of fingers 2 to 5 and a lobster-claw deformity of the feet (split foot). Probably X-linked transmission as the women carrying the mutation present with syndactyly and ulnar hypoplasia.


Anesthetic implications: 

difficult access to peripheral veins.


References : 

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Updated: June 2019