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Question 4#

Which is not a feature of Klippel-Trenaunay syndrome (KTS)?

A) Limb overgrowth
B) Venous varicosities
C) Limb undergrowth
D) Cerebriform palmar hyperplasia
E) Lymphatic malformation

Correct Answer is D

Comment:

Cerebriform palmar hyperplasia. This is not a feature of Klippel-Trenaunay syndrome (KTS). KTS is a sporadic disorder characterised by a triad of vascular malformation, venous varicosity and growth disturbance of soft tissue or bone. Capillary malformation of the port wine type is the most common vascular malformation but can be present with underlying lymphatic malformation. Overgrowth (hyperplasia) is most common but undergrowth can also occur in the affected limb. Cerebriform palmar hyperplasia is a feature of Proteus syndrome, another overgrowth syndrome.