Small Intestinal Scleroderma With Malabsorption and Pneumatosis Cystoides Intestinalis

Abstract

Three patients had scleroderma of the gastrointestinal tract manifested by chronic partial small bowel obstruction and pneumatosis cystoides intestinalis. In two of them rupture of cysts caused pneumoperitoneum. Melabsorption of fat, vitamin B₁₂, and xylose led to rapid wasting. Massive jejunal contamination with bacteria was present in two of the patients, and in these two the steatorrhea was successfully treated with appropriate antibiotics. Repeated courses of antibiotics were necessary because of recurrent diarrhea and steatorrhea. Pneumatosis cystoides intestinalis has not been emphasized previously as a complication of small intestinal scleroderma. The origin of the cyst gas is unknown, but the lumen of the gastrointestinal tract seems the most likely source. Pneumatosis cystoides intestinalis is probably a late complication and its presence suggests a poor prognosis.