Cronkhite-Canada Syndrome: A Diagnostic Challenge and Management With Vedolizumab

BACKGROUND Cronkhite-Canada syndrome (CCS) is a rare, nonhereditary gastrointestinal polyposis syndrome characterized by diffuse polyps, diarrhea, malnutrition, and ectodermal changes. It can mimic inflammatory bowel disease (IBD), leading to misdiagnosis and delayed treatment. Here, we present a case of CCS that was initially thought to be Crohn's disease, managed with vedolizumab, and ultimately required total parenteral nutrition (TPN) due to severe malnutrition. CASE REPORT A 65-year-old man presented with a history of 2 months of watery diarrhea, weight loss, postprandial abdominal pain, dysgeusia, and nail dystrophy. He was initially diagnosed with Crohn's disease based on colonoscopy and biopsies. His symptoms were initially managed with prednisone and vedolizumab. However, 8 months later, he developed worsening dysgeusia, anorexia, and further weight loss. A follow-up esophagogastroduodenoscopy (EGD) showed large nodular polyps in the stomach and duodenum, with chronic inflammation, epithelial hyperplasia, and mucosal edema on biopsy. In a multidisciplinary review, the clinical picture was deemed more consistent with CCS rather than Crohn's disease. Vedolizumab was continued due to partial symptomatic relief. Endoscopic or surgical gastrostomy was deferred due to severe malnutrition and extensive gastric involvement, and TPN was initiated. CONCLUSIONS This case highlights the diagnostic complexity and clinical severity of CCS, which can closely mimic IBD. Although the patient had a partial response to vedolizumab, the progression of disease led to severe malnutrition requiring TPN. Early recognition and aggressive nutritional and medical management are essential for managing this debilitating condition.