S4066 Just Kidding Around: Unusual Adult Manifestation of Cystic Fibrosis With Distal Intestinal Obstruction Syndrome (DIOS), Appendiceal Disease, Biliary Pathology, and Pancreatic Manifestations

Introduction: As cystic fibrosis (CF) survival extends into adulthood, gastroenterologists increasingly encounter gastrointestinal and hepatobiliary complications requiring new management strategies. This case introduces 2 treatment principles: (1) the necessity of early multimodal imaging to differentiate CF-related pathology from surgical emergencies, and (2) the importance of conservative management that avoid unnecessary interventions. Case Description/Methods: A 41-year-old man with CF (ΔF508 mutation), pancreatic insufficiency, and CF-related diabetes presented with progressive abdominal distension and emesis despite optimized bowel regimens. Initial computed tomography (CT) revealed long-segment colo-colonic intussusception extending to the mid-transverse colon with moderate stool burden, complete pancreatic lipomatous pseudohypertrophy, and dilated left intrahepatic biliary tree. Follow-up magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) demonstrated persistent intussusception, left intrahepatic biliary ductal dilation with confluence narrowing, and multiple biliary calculi. Subsequent presentation with abdominal pain and hematochezia prompted repeat imaging, showing resolution of intussusception but persistent colonic wall thickening and an enlarged appendix with hyperdense secretions. The following colonoscopy revealed distended appendix with ulcerated appendiceal orifice mucosa with purulent discharge, initially concerning for appendicitis or malignancy, though biopsy was unremarkable. Distended appendix from accumulation of thick mucinous is thought to be the pathologic lead point for colo-colonic intussusception. The patient was managed conservatively with an aggressive bowel regimen and plans for outpatient endoscopic ultrasound and endoscopic retrograde cholangiopancreatography, avoiding unnecessary surgical intervention. Discussion: This case illustrates essential treatment strategies for aging CF patients: early implementation of comprehensive imaging protocols (CT, MRI/MRCP, endoscopy) enables accurate differentiation of CF-related complications from acute surgical conditions, while conservative management approaches prevent unnecessary procedures. Gastroenterologists must recognize that CF-related GI pathology often mimics surgical emergencies but frequently responds to medical management and targeted endoscopic interventions. A multidisciplinary approach emphasizes careful observation, serial imaging, and selective intervention, which can improve outcomes while minimizing procedural risks in the aging CF population.