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General Surgery · Vascular surgery: vascular malformations and tumours
Sample itemA child has a port-wine stain over the trigeminal V1 area, seizures and glaucoma. Which syndrome is this, in contrast to the limb overgrowth syndrome with port-wine stain, varicose veins and hypertrophy?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Sturge-Weber syndrome; the limb syndrome is Klippel-Trenaunay
Sturge-Weber syndrome has a facial capillary malformation with leptomeningeal angiomatosis. Klippel-Trenaunay syndrome has the triad of capillary malformation, venous varicosities and limb hypertrophy. Infantile haemangiomas, unlike malformations, proliferate then involute.
Option by option
A. Von Hippel-Lindau disease; the limb syndrome is Osler-Weber-Rendu
VHL and HHT are different conditions.
B. Kasabach-Merritt syndrome; the limb syndrome is Maffucci
Kasabach-Merritt is a consumption coagulopathy with kaposiform haemangioendothelioma.
C. Sturge-Weber syndrome; the limb syndrome is Klippel-Trenaunay (correct)
Correct. The pairings are accurate.
D. Klippel-Trenaunay syndrome; the limb syndrome is Sturge-Weber
The syndromes are reversed.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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