An Unlikely Cause of Bowel Obstruction in a Woman With Scleroderma

Abstract

Systemic sclerosis is a multisystem disease that is characterized by vasculitis of the small arteries and fibrosis of the skin and other organs. The gastrointestinal system is the most frequently affected organ system in patients with systemic sclerosis, affecting 70-90% of patients. Gastrointestinal manifestations most commonly involve the esophagus but can also affect the entire GI tract from mouth to anus in the form of GERD, gastroparesis, pseudoobstruction, secondary small intestinal bacterial overgrowth (SIBO) and malabsorption. This is a case of a 59 year old woman with scleroderma, gastroparesis, SIBO, pseudoosbtruction. Her symptoms worsened and required a hospitalization and was found to have a cecal volvulus. She was taken to the operating room and the volvulus was reduced and no bowel resection was performed. This volvulus reoccurred four months later but resolved with conservative management. Over the following months, she developed an iron-deficiency anemia and underwent EGD and colonoscopy. Colonoscopy revealed a twist in the ascending colon and could not be traversed to the cecum. Within the twist was a flat polyp diagnosed as being a tubulovillous adenoma with high-grade dysplasia. She was referred to surgery for partial colectomy. In the operating room, she was found to have colonic distension and was found to have a mesenteric defect through which the colon herniated. She underwent an extended right sided colon resection. Large bowel obstruction due to trans-mesenteric internal hernias are exceedingly rare and has a reported incidence between 0.2 - 0.9%. This is a difficult diagnosis to make even with imaging studies and many times is found during the operation. In this case, her first operation did not identify the mesenteric defect and was a presumed volvulus secondary to her scleroderma. Therefore, trans-mesenteric internal hernias are a difficult diagnosis to make and often times require an exploratory laparotomy.