Abdominal Pain in a 22 Year Old with Cystic Fibrosis

Abstract

Cystic fibrosis is a genetic disorder of the exocrine system resulting in abnormal clearance of mucus and electrolytes effecting multiple organ systems including pulmonary, endocrine, and gastrointestinal. We present an unusual cause of intussusception and ileocecal prolapse in a cystic fibrosis patient. Case: A 22 yo male with PMH of cystic fibrosis, insulin dependent DM, chronic constipation, and biliary cirrhosis presented with worsening RLQ abdominal pain for 1 day associated with nausea and emesis. The patient admitted to chronic RLQ pain for the past one year usually resolved after increasing intake of polyethylene glycol and docusate. He remained afebrile with a normal white blood cell count. Physical exam was significant for bilateral rhonchi, hepatomegaly, and RLQ tenderness. No palpable abdominal mass. A CT demonstrated mid jejunal- ileal intussusception and a probable ileocecal intussusception with marked distortion and edema. Follow up CT one day later showed resolution of small bowel intussusception and residual ileocecal deformity. Colonoscopy revealed rectal varices and ileocecal prolapse with mucosal ischemia. Given the concern for ongoing bowel ischemia the patient underwent exploratory laporotomy which revealed a 6 cm ileocecal prolapse with multiple fecoliths. There was no evidence of true intussusception. Pathology specminen demonstrated acute inflammation and necrosis. The patient did well post operatively and was discharged to home. Discussion: Advances in the pulmonary management of CF patients have increased the median survival of these patients. Many patients with CF exhibit a variety of gastrointestinal complaints most notably constipation. This case demonstrates the importance of a diligent evaluation in the adult CF patient to exclude presence of intussusception, distal intestinal obstruction (DIOS) and even IBD. Frequently endoscopic evaluation can help delineate those cases appropriate for conservative treatment versus operative management. In this case our patient had self limited small bowel intussusception with persistent ileocecal prolapse mimicking intussusception on radiographic studies. This patient continues to be followed in our gastroenterology clinic and requires aggressive bowel regimen in hopes of preventing future GI complications related to underlying CF.