Abstract

FERNANDES, Carlos et al. Klippel-Trenaunay-Weber Syndrome: Gastrointestinal hemorrhage in a young patient. J Port Gastrenterol. [online]. 2013, vol.20, n.3, pp.128-131. ISSN 0872-8178.  https://doi.org/10.1016/j.jpg.2012.07.005.

Klippel-Trenaunay-Weber syndrome is a rare pathology characterized by the occurrence of capillary cutaneous malformations, venous and/or lymphatic anomalies, bony and/or soft tissue hypertrophy associated with arteriovenous malformations. Gastrointestinal involvement occurs in about 1/5 of affected patients and usually presents as lower gastrointestinal bleeding. Treatment is mainly symptomatic. The authors present a clinical case of a 19 year-old woman with known Klippel-Trenaunay-Weber syndrome who was referred to our Proctology outpatient clinic due to sporadic rectal bleeding since early childhood. She had venous insufficiency of her right lower limb, capillary cutaneous hemangiomas in both lower limbs and hypertrophy of the left lower limb. Proctologic examination revealed grade II haemorrhoids, which were congested but not friable. On flexible sigmoidoscopy, several varicose veins with polypoid appearance and endoscopically normal mucosa were apparent. Management with laxatives and dietary fiber increase was initiated with symptomatic improvement.

Keywords : Klippel-Trenaunay-Weber Syndrome; Gastrointestinal hemorrhage; Proctology.

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