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Gardner syndrome (GS) is a rare autosomal dominant inherited disorder with a high degree of penetrance characterized by intestinal polyposis, bone and soft-tissue tumors, including osteoma, epidermal inclusion cyst, lipoma, fibroma, gastric and duodenal polyposis and desmoid fibromatosis. In 100% of patients with GS untreated, cancer develops in the large intestine before the age of 40 years. Hence, prophylactic colectomy is indicated (1). The restorative proctocolectomy in combination with ileal pouch-anal anastomosis (RPC/IPAA) is the best operation for GS patients because it not only resects all large intestine mucous membranes to avoid carcinogenesis, but also preserves the intestine function and sex ability and avoids colostomy, thus improving the quality of life of patients (2). We present the case of a patient with GS treated with restorative proctocolectomy with ileal pouch-anal anastomosis with poor post-operative outcome, who developed severe ulcerative chronic pouchitis refractory to treatment. Case report

Refractory ulcerative chronic pouchitis in a patient with Gardner syndrome following restorative proctocolectomy with ileal pouch-anal anastomosis