The Klippel-Trenaunay Syndrome: Clinical and Radiological Aspect

Abstract

Klippel-Trenaunay syndrome (KTS) is characterized by port-wine hemangiomas, deep venous system abnormalities, superficial varicosities, and bony and soft-tissue hypertrophy. When associated with an arteriovenous fistula, it has been termed Klippel-Trenaunay-Parkes-Weber syndrome. It is imperative that both the radiologist and surgeon be aware of this entity, as incomplete evaluation and inappropriate surgery may be devastating. Radiological workup includes phlebography, angiography, and conventional radiography of the involved extremities. Surgery should be performed only to relieve deep venous obstruction (if present) or to correct inequality in the lengths of legs. Removal of superficial varicosities is contraindicated because it will worsen existing symptoms. Five cases of KTS are presented and the literature reviewed.

Spinal Extradural Meningeal Cyst in Klippel-Trenaunay Syndrome

“…It is also expressed in the developing heart, vessels, and limbs and is essential for normal development of the aortic arch and axial skeleton. KTS consists of a triad of cutaneous capillary hemangiomas, bone and soft tissue hypertrophy, and venous varicosities and these triad was present in the present case 13,16,21) . The manifestations of KTS are variable 21) .…”

“…KTS consists of a triad of cutaneous capillary hemangiomas, bone and soft tissue hypertrophy, and venous varicosities and these triad was present in the present case 13,16,21) . The manifestations of KTS are variable 21) . The venous abnormalities usually involve the affected extremity and present as superficial varicose veins.…”

Cavernous Lymphangioma of the Spleen in a Patient with Klippel-Trenaunay-Weber Syndrome.

“…The Klippel-Trenaunay-Weber syndrome (K-T-W syndrome) is characterized by cutaneous port wine hemangiomas, superficial venous varicosities, and soft tissue and bony hypertrophy (or hypotrophy) of an extremity (1)(2)(3). This constellation of abnormalities is thought to give arise to hypoplasia or atresia of the deep venous system (4).…”

Klippel-Trenaunay syndrome: a very infrequent cause of microcalcifications in mammography

“…Klippel-Trenaunay syndrome (KTS) is a rare disorder whose major manifestations are (a) cutaneous hemangioma, (b) varicosities, and (c) soft tissues and bone hypertrophy [1][2][3]. The presence of hemodynamically significant arteriovenous fistulae excludes a KTS, because that abnormality is characteristic of Parkes-Weber syndrome [1].…”

“…The presence of hemodynamically significant arteriovenous fistulae excludes a KTS, because that abnormality is characteristic of Parkes-Weber syndrome [1]. Usually, one leg is affected, but even involvement of one upper extremity has been reported.…”