Purpose or Case Report: Klippel-Trenaunay Syndrome (KTS) is a rare cutaneous vascular malformation syndrome involving capillary, venous, and lymphatic malformations often involving a unilateral lower limb. Patients with KTS can also have disease involvement of the gastrointestinal (GI) tract, predisoposing patients to GI bleeding. Here, we present a case of a 15-year-old male with known congenital Klippel-Trenaunay Syndrome involving the left lower extremity presenting with acutely worsening rectal bleeding. The patient presented with two weeks of rectal bleeding, fatigue, and weakness. On initial evaluation, the patient was found to have acute drop in hemoglobin concerning for active GI bleeding. Initial imaging included a tagged red blood cell scan, which demonstrated diffuse increased radiotracer activity along the left hemiabdomen, consistent with active bleed. However, the etiology/source of bleeding at this time could not be determined due to the extensive abdominal involvement. Further evaluation included CT angiography of the abdomen and pelvis, which revealed a large, extensive venous malformation extending throughout the wall of the descending and rectosigmoid colon, with venous malformation extending throughout the left lateral pelvic musculature. Arterial phase imaging demonstrated abnormal enhancement of the mucosa of the descending and rectosigmoid colon, and venous phase imaging demonstrated contrast blushing throughout the descending colon concerning for active bleeding, without clear identifiable source. Due to decline in clinical status and refractory anemia requiring massive transfusion, the patient was evaluated surgically and subsequently underwent emergent colectomy and left lower quadrant ostomy placement. Intraoperative findings demonstrated a massively dilated sigmoid colon with complex tangle of cavernous venous malformations involving approximately two feet of bowel. In setting of a technically challenging operation, the patient’s post-operative course has been complicated by rectal stump dehiscence, multiple pelvic abscesses, and guarded prognosis. In summary, KTS is a rare syndrome involving extensive capillary, lymphatic, and venous malformations, with gastrointestinal involvement predisposing patients to potential life-threating GI bleeding. Here we review a case of a 15-year-old male with KTS presenting with refractory GI bleeding in an extensive colonic vascular malformation necessitating emergent colectomy.
Methods & Materials:
Results:
Conclusions: