What is Klippel Trenaunay Syndrome?
Klippel-Trénaunay-Weber syndrome, occasionally termed as angioosteohypertrophy syndrome is an uncommon heritable circulatory condition caused by the failure of blood vessels to develop properly. It is characterized by a localized or dispersed vascular mark which spreads over superficial and deep dilated capillaries with related hypertrophy of soft and bone tissues. It was first documented by Klippel and Trenaunay after observing two patients with distinctive symptoms, calling the syndrome as naevus vasculosus osteohypertrophicus.
The primary pathophysiology still needs to be elucidated. Existing theories suggest intrauterine damage to sympathetic ganglia, deep vein abnormalities, mesodermal defect at some point in fetal development and finally underlying mixed mesodermal and ectodermal dysplasia.
The disorder can be diagnosed by the existence of a multiplicity of symptoms including varicose veins, one or more distinctive port-wine stains, bony and soft tissue hypertrophy, blood in the urine, bleeding from the affected limb and rectum, skin infection, anemia, seizure, limb pain and an improperly developed lymphatic system.
The treatment for this syndrome is usually asymptomatic. To alleviate pain and swelling, medications and compression stockings are given. If patient becomes anemic, iron supplements may be recommended. To reduce the threat of further serious complications, surgical and non-surgical treatments are performed including ligation and stripping, sclerotherapy, laser therapy and endovenous thermal ablation.
Pictures of Klippel Trenaunay Syndrome
Images, Photos and Pictures of Klippel Trenaunay Syndrome…