ABSTRACT
The Kippel Trenaunay Weber syndrome is a rare entity. It is characterized by osteohypertrophic angiodysplasia. The vascular abnormalities touch especially the lymphatic and venous systems. However the existence of superficial or deep arteriovenous fistula is not rare. The deep one can have multiple visceral seats and put a strain on vital prognosis of patients in the event of a rupture. The authors report the case of a 9 year-old girl who presented a Klippel Trenaunay Weber Syndrome diagnosed on the occasion of a recurrent rectorrhagia which lead to severe anemia with 4,4g/100 ml of haemoglobin. The osteohypertrophic angiodysplasia touch the two inferior limbs. It is more marqued on the right side and extends to the lumbus. The colonoscopy found a rectosigmoidal fistula associated with a rectal prlapsus. The abdominal angioscan found multiple parietointestinal calcifications. It shows that vascular abnormalities in digestif tractus are diffuse. In the light of this case and the review of literature, the authors remind the clinical, para clinical aspects and difficulties of treatment of this rare entity