Extensive pure venous malformations in the upper or lower limb: A review of 27 cases - 11/09/11
From the Interdisciplinary Study Group for Vascular Anomalies,a the Department of Dermatology of Pr Drouet,c Department of Interventional Radiology of Pr Merland,e Hôpital Lariboisière, the Department of Dermatology of Pr Escande, Hôpital Cochin, Pavillon Tarnier,b and the Department of Vascular Surgery, Hôpital Saint Joseph.d
Abstract |
Background: Extensive pure venous malformations (VMs) involving the entire lower or upper limb and adjacent trunk form a particular group of rare vascular malformations.
Objective: Our purpose was to review 27 cases of extensive limb VMs and describe their characteristics and management.
Methods: Cases of extensive limb VMs were investigated, treated, and observed for a mean of 7 years.
Results: Eleven cases involved the upper limbs and 16 involved the lower limbs. All involved skin and muscle. In 81% of cases in the lower limb there was also knee joint involvement that created severe functional impairment. Ultrasonography with Doppler (duplex scans), computed tomographic scans, and magnetic resonance imaging were helpful noninvasive diagnostic procedures in these patients, whereas arteriography and phlebography were less informative. Muscle involvement was present in 100% of patients and bone abnormalities in 63%. Leg length was either normal or there was slight limb undergrowth, except in three patients who had minor overgrowth of the affected limb. Coagulation profiles demonstrated localized intravascular coagulation in 88% of patients. The majority of patients had conservative management (elastic stockings). In a few, percutaneous sclerotherapy or partial excision of skin and muscle VMs was beneficial. Knee joint involvement required synovectomy and VM excision during childhood in 7 of 16 patients.
Conclusion: Extensive limb VMs are characterized by diffuse involvement of the skin, muscle, and joints, and by a specific localized intravascular coagulopathy with general consequences. This group of vascular malformations should be separated from the Klippel-Trenaunay and Parkes Weber syndromes. 1997;36:219-25.
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Reprint requests: O. Enjolras, MD, Department of Dermatology, Hôpital Cochin, Pavillon Tarnier 89 rue d'Assas, Paris 75006, France. |
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0190-9622/97/$5.00 + 0 16/1/77700 |
Vol 36 - N° 2
P. 219-225 - février 1997 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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