Bleeding from cavernous angiomatosis of the rectum in Klippel-Trénaunay syndrome: Report of three cases and literature review

Candace L. Wilson, Louis Michel Wong Kee Song, Heidi Chua, Marcello Ferrara, Richard M. Devine, Roger R. Dozois, Vandana Nehra

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60 Scopus citations


Klippel-Trénaunay syndrome (KTS) is a congenital vascular anomaly characterized by limb hypertrophy, cutaneous hemangiomas, and varicosities. GI hemorrhage is a potentially serious complication secondary to diffuse hemangiomatous involvement of the gut. We report on three patients with KTS who presented with transfusion-dependent anemia and life-threatening bleeding due to extensive cavernous hemangiomas involving the rectum. Two patients were treated by proctocolectomy and coloanal anastomosis, which preserved anal function while controlling bleeding. The third patient required an abdominoperineal resection because of extensive rectal, perianal, and perineal angiomatosis. The literature on the evaluation and management of GI hemorrhage in KTS, particularly of colorectal origin, is reviewed.

Original languageEnglish (US)
Pages (from-to)2783-2788
Number of pages6
JournalAmerican Journal of Gastroenterology
Issue number9 SUPPL.
StatePublished - 2001


ASJC Scopus subject areas

  • Gastroenterology

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