Klippel‐Trenaunay syndrome with anorectum involvement and portal hypertension

Gastrointestinal involvement in Klippel-Trénaunay syndrome: pathophysiology, evaluation, and management

“…Its incidence is underestimated, may be as high as over 30% in patients with KTS [ 2 , 4 ]. Gastrointestinal involvement is typically presented with edematous and thickened colorectum by the venous malformation (VM) network around and intra-wall [ 3 , 5 , 6 ]. These VMs can cause recurrent rectal bleeding, ranging from occult to massive, and life-threatening hemorrhages [ 1 , 2 ].…”

“…There are no current recommendations for managing of gastrointestinal VM in KTS. In some instance, some seemingly adequate treatments may be contraindicated [ 3 , 6 ]. For example, a popular treatment, sclerotherapy can be preferred for anorectal VM managing [ 2 , 3 , 6 ], but primary sclerotherapy is not suitable for anorectosigmoid VM in KTS, since norectosigmoid VM has distinct pathophysiologies from anorectal VM [ 3 ].…”

“…Sclerotherapy is becoming a popular management recently. Since anorectal VM mainly drains into the IIV [ 2 , 3 , 6 , 39 ], and rarely into the median sacral vein [ 39 ] (Figs. 1 and 2 ), adequate sclerotherapy carries little complication [ 3 , 12 , 14 ].…”

“…It is a safe choice and recommended by clinical practice guideline [ 1 ]. We prefer ethanol-based sclerotherapy via direct puncture and/or trans-IIV approaches [ 2 , 3 , 6 ] (Figs. 2 and 3 ).…”

“…2 ). Primary VM along, around and within the rectosigmoid wall serves as a spongy blood pool and the source of thrombus [ 3 , 6 ] (Fig. 2 ).…”