李育軒 醫師 於 2013-10-07 19:31:18 回覆

Klippel-Trenaunay-Weber Syndrome
http://reference.medscape.com/article/1084257-overview
Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by a triad of port-wine stain, varicose veins, and bony and soft tissue hypertrophy involving an extremity. Note the image below.

In 1900, noted French physicians Klippel and Trenaunay first described a syndrome in 2 patients presenting with a port-wine stain and varicosities of an extremity associated with hypertrophy of the affected limb's bony and soft tissue. They termed the syndrome "naevus vasculosus osteohypertrophicus." In 1907, Parkes Weber, unaware of Klippel and Trenaunay's report, described a patient with the 3 aforementioned symptoms as well as an arteriovenous malformation of the affected extremity. He termed the process hemangiectatic hypertrophy.

Today, conflicting opinion exists in the literature as whether to separately designate the original triad as Klippel-Trenaunay syndrome and the triad with the addition of arteriovenous malformation as Parkes Weber syndrome. Making the distinction is probably wise given the increased morbidity associated with arteriovenous malformations. For this discussion, the 2 types are considered together.

In 1900, noted French physicians Klippel and Trenaunay first described a syndrome in 2 patients presenting with a port-wine stain and varicosities of an extremity associated with hypertrophy of the affected limb's bony and soft tissue. They termed the syndrome "naevus vasculosus osteohypertrophicus." In 1907, Parkes Weber, unaware of Klippel and Trenaunay's report, described a patient with the 3 aforementioned symptoms as well as an arteriovenous malformation of the affected extremity. He termed the process hemangiectatic hypertrophy.

Today, conflicting opinion exists in the literature as whether to separately designate the original triad as Klippel-Trenaunay syndrome and the triad with the addition of arteriovenous malformation as Parkes Weber syndrome. Making the distinction is probably wise given the increased morbidity associated with arteriovenous malformations. For this discussion, the 2 types are considered together.

以上摘自Medscape
http://reference.medscape.com/article/1084257-overview