Intestinal necrosis associated with hemolytic uremic syndrome: A case series

Introduction: Hemolytic 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 presentation: Case 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 2: A 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 3: A 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. Conclusion: Bowel 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.