Superior Mesenteric Artery Pseudoaneurysms in Patients With Familial Adenomatous Polyposis-Associated Intra-abdominal Desmoids: Case Series

BACKGROUND: Rupture of a superior mesenteric artery pseudoaneurysm is a rare but potentially lethal complication in patients with familial adenomatous polyposis and desmoid disease. OBJECTIVE: We report our experience in the management of such patients with a rare but significant and life-threatening condition. DESIGN: This is a descriptive study of a small series of patients. SETTINGS: Data were obtained from their medical charts and from the Cologene Database of the David G. Jagelman Polyposis Registry in the Sanford R. Weiss, M.D., Center for Hereditary Colorectal Cancer at the Cleveland Clinic Foundation. PATIENTS: Of 227 patients with mesenteric desmoid disease, there were 4 cases of superior mesenteric artery pseudoaneurysm rupture. MAIN OUTCOMES MEASURES: We reviewed the patients with mesenteric desmoid tumors in our desmoid registry. The registry is approved by the institutional review board of the Cleveland Clinic. RESULTS: The patients were young (aged from 22 to 28 y at presentation), with otherwise minimal comorbidities. Two patients had a previous proctocolectomy and J-pouch, and 2 had a total colectomy and ileorectal anastomosis. Two patients had preemptive endoluminal stenting and fared better than the 2 who had damage control embolization. One patient died and, in the others, recovery was prolonged and complicated. Two of the 4 patients have ended up with a reasonable quality of life. LIMITATIONS: We acknowledge that this is a rare complication of an uncommon disease and, as such, any case series will be limited by small numbers; therefore, a tailored approach is warranted when managing such complex patients. CONCLUSIONS: We advocate an increased awareness of the possibility of visceral pseudoaneurysms in patients with familial adenomatous polyposis who have desmoid disease encasing the superior mesenteric artery.