Klippel–Trenaunay syndrome in combination with congenital dislocation of the hip

Abstract

Klippel–Trenaunay syndrome (KTS) is a rare and sporadic disorder characterized by the triad of capillary malformations, venous varicosities, and limb hypertrophy. The clinical manifestations of KTS are heterogeneous. In this report, we present a unique case of KTS in combination with congenital dislocation of the hip (CDH) in a 4-day-old female neonate. The patient had a widespread port-wine stain surrounded by regions of unaffected skin in a mosaic pattern, cutaneous hemangioma on the upper lip, left-sided hemihypertrophy involving the entire body, and also evidence of left CDH (based on the results of a physical examination and radiographic interpretation). We present this case for the rarity of presentation, discuss the relationship between KTS and CDH, and the treatment options available with a brief review of the literature.