Intestinal pseudo-obstruction and pneumatosis cystoides intestinalis in a scleroderma patient.

A 34-year-old woman with a 3-year history of scleroderma treated with corticosteroids, presented with 2 weeks of abdominal pain and distension and, more recently, nausea. Two months earlier, she had a cesarean section at 29 weeks of gestation for fetal distress. Physical examination showed diffuse abdominal tenderness with signs of peritonitis, and no fever. She had normal white blood cell count, C reactive protein was 45 mg/L (normal range 10 mg/L). X-ray (A) showed pneumoperitoneum with signs of diffuse gas infiltration. CT scans confirmed the pneumoperitoneum (B, arrow a) with free peritoneal exudates and dilated loops of the small bowel (B, arrow b). Retropneumoperitoneum (B, arrow c) with massive gas infiltration of the mesentery (B, arrow d) and two cysts of pneumatosis cystoides intestinalis (B, arrows e) were also seen on the CT scan. At laparotomy, we discovered an incomplete strangulation of a small intestine loop on an incisional hernia of the Pfannenstiel section. The small bowel and the mesentery were massively infiltrated by multiple gas bubbles (C, arrows). Dilated loops of the small bowel were noted above the stricture. Other adhesions to the Pfannenstiel section were divided. Bacteriologic examination of diffuse peritoneal exudates revealed Escherichia coli. The patient’s postoperative course was uneventful, with bowel movement on day 4. Two years later, this young woman had no A B