Circulation. 2000;101:e82-e83
(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{at}mh-hannover.de
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Introduction
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 Top
 Introduction
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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.
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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.
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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 in shortening of the limb.
Intraosseous vascular ectasias can induce cystic deformation of the
medullary cavity and thinning of the cortex, which may result in joint
destruction. Multiple phleboliths can be found in venous ectasias on
the radiograph. The prognosis of this disorder is uncertain. Therapy is
predominantly conservative. In the presence of aneurysmal
complications or severe shunting, surgery may be
indicated.
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Footnotes
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The editor of Images in Cardiovascular Medicine is Hugh A. McAllister,
Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal
Hospital and Texas Heart Institute, and Clinical Professor of
Pathology, University of Texas Medical School and Baylor College
of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.
