ABSTRACT
Klippel-Trenaunay syndrome is a rare but well-documented congenital malformation. Klippel-Trenaunay syndrome has sometimes been used interchangeably with Klippel-Trenaunay-Weber syndrome. However, Klippel-Trenaunay syndrome is the correct term used for the triad of congenital anomalies. Klippel-Trenaunay-Weber syndrome or Parkes-Weber syndrome is accepted as a separate entity consisting of the triad of Klippel-Trenaunay syndrome accompanied by a clinically apparent arteriovenous fistula. Hemodynamically insignificant arteriovenous malformations do not preclude a diagnosis of Klippel-Trenaunay syndrome. It is important to differentiate between the 2 syndromes because treatment and prognosis are so different. Parkes-Weber syndrome has a poor prognosis for limb viability. This article describes a case study of an infant presenting with Klippel-Trenaunay including a review of the syndrome and treatment recommendations.