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(Circulation. 2000;101:e82.)
© 2000 American Heart Association, Inc.

Circulation Electronic Pages

Peripheral Vascular Malformation (Servelle-Martorell)

Thomas Weiss, MD; Ulrich Mädler, MD; Heike Oberwittler, MD; Birgit Kahle, MD; Claus Weiss, MD; Wolfgang Kübler, MD

From the Departments of Cardiology/Angiology (T.W., C.W., W.K.), Radiology (U.M., H.O.), and Dermatology (B.K.), University of Heidelberg, Germany.

Correspondence to Thomas Weiss, MD, Medizinische Hochschule Hannover, Department for Cardiology/Angiology, Carl-Neuberg-Straße 1, 30625 Hannover, Germany. E-mail weiss.thomas@mh-hannover.de

	Introduction

 
A38-year-old man from India presented for evaluation of a painful vascular malformation of the right hand and forearm that limited the use of his right hand (Figure 1). The malformation had existed since childhood. Other parts of the body showed no abnormalities. Several operations (details not known) had been performed in India without success. The vascular malformation caused a soft-tissue swelling characteristic of venous ectasias. The right arm and hand were slightly shorter than the left arm. The peripheral pulses were symmetrically palpable, and no bruits or thrills indicative of arteriovenous shunts were found. No temperature difference was observed. Radiographs showed hypotrophy of the bone, soft-tissue swelling, and multiple opacities consistent with phleboliths in the affected hand (Figure 2). Further diagnostic procedures were denied.

View larger version (85K): [in this window] [in a new window]   Figure 1. Right hand: extensive deformity caused by venous ectasias.

View larger version (105K): [in this window] [in a new window]   Figure 2. Right hand: hypotrophy of bone with shortening of the phalanges, cystic deformation, soft tissue swelling corresponding to venous malformations, and multiple phleboliths. Left hand: normal findings.

The history and clinical presentation in this case are diagnostic of an angio-osteohypotrophic syndrome known as Servelle-Martorell angiodysplasia. This syndrome describes the association of venous and rarely, arterial malformations with skeletal abnormalities. In the deep venous system, an abnormal vein location, partial or complete lack of valves, and/or venous hypoplasia or aplasia can be observed. The ectasia and aneurysmal dilatation of the superficial veins may result in a monstrous deformity of the extremity. Intraosseous vascular malformations may lead to hypotrophy of bone with destruction of spongiosa and cortical bone, resulting . . . [Full Text of this Article]