Lipomatosis of the Ileocecal Valve Masquerading as Crohnʼs Disease

Abstract

Purpose: Case: A 27 year old male with a five year history of Crohn's disease maintained on long term steroids complained of right lower quadrant crampy abdominal pain associated with ten loose bowel movements per day with scant blood. On examination, he had a depressed affect, Cushingoid features, and mild right-sided abdominal tenderness. No abdominal or pelvic mass lesions were appreciated. His hemoglobin was 9.5 g/dL with microcytic indices, his white blood cell count was 11 K/ul, and erythrocyte sedimentation rate was 20 mm/hr. Colonoscopy revealed a confluence of pseudopolyps along the ileocecal valve. Intubation of his terminal ileum could not be achieved. Biopsies from the pseudopolyps and cecum showed mild chronic inflammation and lymphoid aggregates. Several months later the patient presented with worsening nausea, abdominal cramping, and constipation. He had an abdominal CT scan that revealed infiltrative changes of the fat in the ileocecal region, and terminal ileum wall thickening. A small bowel follow-through was ordered and demonstrated a 5 cm stricture in the distal segment of his small bowel. Azathioprine was started as a steroid-sparing agent and he was referred to colorectal surgery for resection of the stricture. The gross pathology report from his ileocecectomy revealed polypoid lipomatous hyperplasia of the ileocecal valve with associated diverticular outpouching arising from the base of the polyps. Acute diverticulitis with focal perforation was also identified, as were small ulcerations/erosions in the ileum possibly due to intermittent intussusception. Following the operation, the patient remained symptom-free. Discussion: Lipomatosis is a benign focal proliferation of submucosal adipose tissue. When involving the ileocecal valve, it is usually a smooth symmetric enlargement of the valve, though has been reported in the literature as a “grape-like cluster”, as was seen in our patient. Our patient's symptoms were felt to be due to both the polypoid lesions along his ileocecal valve causing intussusception, and the adhesions and stricturing from diverticular disease leading to small bowel obstruction. Though most cases of lipomatosis are asymptomatic, clinical manifestations that may occur include abdominal pain, nausea, vomiting, diarrhea, constipation, and rarely bleeding due to intermittent intussusception. Surgical resection with ileo-colic anastomosis is the definitive treatment for lipomatosis in symptomatic individuals. This case reminds us as clinicians to be wary of established diagnoses, especially in those patients failing to respond to conventional therapy.