Surgical management and multidisciplinary care of Dieulafoy’s lesion in Klippel-Trenaunay-Weber Syndrome

ABSTRACT

A man in his early 40’s with Klippel-Trenaunay-Weber syndrome (KTWS) was referred to the surgical team for treatment of massive upper gastrointestinal bleeding. This syndrome is a rare congenital condition that leads to abnormal development of blood vessels, bones, and soft tissues. Although venous malformations are common in KTWS, gastrointestinal involvement is rare but can cause life-threatening bleeding. In this case, a series of diagnostic tests, including endoscopic and angiographic examinations, failed to identify the etiology of the bleeding. Surgical intervention was required after conservative and interventional radiologic therapies proved unsuccessful. A Dieulafoy’s lesion was identified in the proximal stomach and later confirmed histologically. The patient required a multidisciplinary care involving gastroenterology, interventional radiology, and general surgery for successful management.

The pathogenesis of Dieulafoy’s lesion is not well understood, and interventions vary from endoscopic to surgical management. The treatment of Dieulafoy’s lesion is not well- described in patients with KTWS, especially in cases in which endoscopic treatments have failed.

Keywords: Klippel-Trenaunay Syndrome; Dieulafoy’s lesion; gastrointestinal bleeding; radiology, interventional; endoscopy; surgical treatment