When the Benign Pneumatosis Intestinalis Becomes No Longer Benign: A Rare Case of Bowel Perforation in a Patient with Systemic Sclerosis

Abstract

Systemic sclerosis is a multisystem disease featured with autoimmunity and organ fibrosis. Although gastrointestinal (GI) tract involvement is common in patients with systemic sclerosis, colonic perforation is extremely rare. Benign pneumatosis intestinalis, a phenomenon more frequently seen in rheumatologic conditions, makes the diagnosis of colonic perforation even more challenging. We report a unique case of colonic perforation in a patient with chronic systemic sclerosis. This patient initially presented with mild abdominal pain and hematemesis. Urgent upper endoscopy was unremarkable and radiology showed stable pneumatosis intestinalis. Due to worsening abdominal pain, laparotomy exploration was performed and colonic perforation with transmural ischemic necrosis was found.

Spontaneous bowel perforation in the setting of colonic involvement with scleroderma

“…About 20%–50% of those patients will have some degree of colonic involvement 1. Life-threatening visceral perforations, including colonic perforations, have been described in the setting of scleroderma,5–11 although the exact incidence of these serious complications remains unknown with most evidence coming from single case reports or case series.…”

Pneumatosis intestinalis with delayed onset small bowel perforation: a case report

“…True intestinal perforation is uncommon but life-threatening in a PI patient. In addition, timely detection of intestinal perforation is difficult but necessary for treatment of PI patient [5,6,10]. The occurrence of delayed onset perforation is very rare but it could be.…”