P-124 YI Severe Colonic Stricture in Immunosuppressed Patient with Chronic Cytomegalovirus Colitis

Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency due to a defect in phagocyte NADPH oxidase resulting in recurrent life-threatening bacterial and fungal infections, commonly with catalase positive organisms. These patients may present with an inflammatory bowel disease phenotype and frequently have severe intestinal disease. Bone marrow transplantation is curative for CGD, but chronic immunosuppression places patients at higher risk for disseminated infections including cytomegalovirus (CMV). We present a case of CMV colitis resulting in severe stricture formation in a post-transplant patient. A 6 year old boy with history of CGD diagnosed at age 21 months after presenting with multiple infections and colitis underwent 2 autologous bone marrow transplantations. He later developed several months of bloody diarrhea, intermittent fever, and severe abdominal pain. Infectious stool studies were negative. EGD and flexible sigmoidoscopy revealed epithelial damage in his antrum, duodenum, and colon with increased apoptosis and cytopathic changes in his colon with positive CMV tissue PCR and immunostain. Initial differential diagnosis included Graft versus Host disease and CMV colitis. Imaging at the time revealed diffuse circumferential bowel wall thickening with pericolonic fat. He continued to have profuse bloody diarrhea as well as diffuse abdominal pain. Subsequently, he was treated with IV Ganciclovir and Foscarnet, as well as bowel rest. Follow up flexible sigmoidoscopy biopsies remained CMV tissue PCR positive. With antiviral treatment, his symptoms resolved, and he tolerated enteral feeds with a normal stooling pattern again. Two months following therapy, he returned with progressive, severe distinct episodes of diffuse abdominal pain. The pain was worse with defecation and passing flatus and pain improved on Miralax. CT scan demonstrated a long segment of submucosal bowel wall thickening involving the descending colon. Subsequent barium enema reproduced the patient's episodic pain symptoms and revealed a pinpoint focal colonic stricture at the level of the splenic flexure. Ultimately, surgical resection of 20cm of colon revealed ulceration with chronic inflammation and granulation tissue with scattered capillary endothelial cells with viral cytopathic changes consistent with CMV and highlighted by immunohistochemical stains. CMV is an opportunistic pathogen that results in systemic disease through blood circulation and subsequent invasion of endothelial cells of several organ tissues. CMV infection typically remains latent in immunocompetent individuals, however, it is critical to evaluate for the virus in the immunosuppressed patient, where the infection can be reactivated. The GI tract is a common site for CMV disease, often resulting in esophagitis or colitis. Typical symptoms include watery diarrhea that progresses to rectal bleeding, abdominal pain, fatigue, fever, or even toxic megacolon. While CMV has been previously implicated in complicating severe steroid-refractory inflammatory bowel disease, this is the first report of colonic stricture secondary to chronic CMV infection in an immunosuppressed patient.