[Spontaneous pneumoperitoneum in scleroderma]

A spontaneous and asymptomatic pneumoperitoneum was observed in two women presenting with pseudoileus resulting from severe gastrointestinal involvement in progressive systemic sclerosis. In the first case, pneumoperitoneum was associated with pneumatosis cystoides intestinalis and remained unchanged during 3 years. As obstruction resisted to medical management, surgery was performed; however at laparotomy neither perforation nor obstruction of the bowel could be found; the patient died during the postoperative course. In the second case, pneumoperitoneum disappeared after medical management of low-grade intestinal ileus but the patient died 6 months later because of cardiac failure. Autopsy revealed major distention of the bowel but failed to show any signs of perforation. These findings show that, in progressive systemic sclerosis, pneumoperitoneum can occur in the absence of digestive perforation and that surgery is not required. However this complication seems to carry a very poor prognosis.