Courtesy Michael O. Murphy, MD
Klippel-Trenaunay Syndrome

Arterio-venous malformations (AVM) are high-flow vascular malformations. AVM are composed of abnormal vessels with many direct arterio-venous link (without capillary). Arteriovenous fistulae in contrast, can be acquired after external trauma. Various syndromes may be associated e.g. Parkes-Weber syndrome. About half are congenital, and the rest arise usually in the first 3 decades.

Stage Classification according to Schobinger

Stage Clinical
I Flat macule at birth. Asymptomatic stable. AV shunt may be detected by Doppler ultrasound
II Growing, pulsatile, with local hyperthermia, thrill and wheeze. Tortuous vessels at puberty
III Complicated AVM. E.g. may have ulceration, bleeding, continuous pain, and possibly necrosis and lytic bone lesions
IV Associated with heart failure


Intra-vascular embolization of afferent arteries as well as surgery may be done.


Actas Dermosifiliogr.2014;105:347-58 - Vol. 105 Num.4


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