Chronic Pneumoperitoneum in Systemic Sclerosis

A 71-year-old female patient with a known diagnosis of scleroderma attended the emergency department with a subacute history of minor weight loss, abdominal distension, and prominent bowel sounds, without a change in bowel habit. Scleroderma in this patient had been complicated by chronic intestinal pseudo-obstruction and bacterial overgrowth. Regular medications included rifaximin 400 mg three times daily in 4-week pulses, interrupted by 2-week rest periods, when an oral probiotic was taken. Other medications included laxatives, promotility agents, calcium supplements, and a bisphosphonate. She had a history, 5 years previously, of partial small bowel volvulus, which required laparotomy with a fibrous band division. Examination revealed a soft, nontender tympanitic abdomen, with marked distension and an uncomplicated incisional hernia at the site of a previous laparotomy. She had a plain abdominal radiograph, after which she had a computed tomography (CT) of the abdomen and pelvis (Figure 1). The CT demonstrated a large volume of free intraperitoneal gas consistent with extensive pneumoperitoneum. There also was extensive retroperitoneal free air but no signs of visceral perforation, pneumatosis intestinalis, or pneumatosis coli. The large bowel was dilated but without evidence of a transition point to suggest mechanical obstruction. Hematologic and biochemical investigations were unremarkable. Because the patient was systemically well without signs of acute peritonitis, she was managed conservatively. At outpatient follow-up 3 months later, she had persisting abdominal distension, she remained clinically well. A repeat