Intestinal necrosis associated with hemolytic uremic syndrome: A case series

Abstract

IntroductionHemolytic uremic syndrome (HUS) usually presents with mild gastrointestinal symptoms, typically managed medically. We describe 3 patients with challenging clinical presentations diagnosed with colonic perforation, review the literature, and discuss lessons learned. Case presentationCase 1: A 5-year-old girl presented with symptoms of HUS. Three weeks later, with new fevers and tachycardia, computerized tomography (CT) revealed pneumoperitoneum. She underwent resection of necrotic small bowel and sigmoid, with a second look resection and creation of multiple stomas. After 5 months, she was discharged on total parenteral nutrition (TPN) and hemodialysis. She later had restoration of intestinal continuity and renal transplantation. Case 2A 2-year-old boy with HUS underwent, over 3-weeks, 9 ultrasounds, percutaneous ascites aspiration, and 2 CT scans before diagnosis of colonic necrosis and perforation. A laparotomy revealed necrotic colon with a thick rind encasing the bowel, rendering full exploration and stoma creation impossible. Drains were placed, and he was discharged on TPN after 2 months. He underwent colo-colonic anastomosis and a TPN wean after one year with full renal recovery. Case 3A 3-year-old boy with HUS developed worsening distension. After one week, he suffered cardiopulmonary arrest and CT showed bowel hypoenhancement. He underwent resection of necrotic colon with transverse colostomy creation. After 4 months, he was discharged on hemodialysis. He underwent subsequent colostomy closure and renal transplantation. ConclusionBowel ischemia is difficult to diagnose in HUS. CT with intravenous contrast is critical for diagnostic accuracy. Frequent re-evaluation by pediatric surgery is important to avoid missing a surgical emergency.