Spontaneous pneumoperitoneum with Pneumatosis cystoides intestinalis in a patient with mixed connective tissue disease

Abstract

Pneumatosis cystoides intestinalis (PCI), a disorder in which gas filled cysts develop within the bowel wall, has been associated with collagen vascular disease, usually progressive systemic sclerosis (PSS). We have extended the spectrum of collagen vascular disease associated with PCI by this report of a patient with mixed connective tissue disease (MCTD) who developed PCI and recurrent spontaneous pneumoperitoneum (SP). Possible pathogenetic mechanisms are reviewed and the management of the asymptomatic patient with pneumoperitoneum, PCI and collagen vascular disease is discussed.