Rare Presentation of the Vein of Servelle in a Case of Klippel-Trenaunay Syndrome

TL;DR

A 24-year-old man with Klippel-Trenaunay syndrome showed a rare vein called the vein of Servelle alongside typical symptoms like varicose veins and limb hypertrophy.

Contribution

This case highlights the rare co-occurrence of the vein of Servelle in a patient with Klippel-Trenaunay syndrome.

Findings

The patient exhibited macrodactyly and varicose veins in the right lower limb.

The vein of Servelle was identified as a synchronous presentation in this KTS case.

The case underscores the importance of recognizing embryonic vein persistence in vascular malformations.

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular syndrome involving bone and soft tissue hypertrophy of the involved limb and vascular malformations of the lymphatic, capillary, and venous systems. It is often confused with Parkes-Weber syndrome (PWS). KTS is characterized by a triad of capillary malformation in the form of port wine stains, bone or limb hypertrophy, and varicose veins. The vein of Servelle, also known as the lateral marginal vein, is one of the two persisting embryonic veins of the leg, the persistent sciatic vein being the other. Truncal vascular malformation can be a complication of failure of obliteration of these veins. We present a case of a 24-year-old male of KTS who had varicose veins in his right lower limbs since five years of age and macrodactyly with a synchronous presentation of the vein of Servelle.

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