A case of pneumatosis cystoides intestinalis in a patient with Crohn’s disease complicated by systemic sclerosis

Abstract

Pneumatosis cystoides intestinalis (PCI) is the rare clinical finding of gas accumulation within the gastrointestinal wall.  It can be secondary to a variety of disease processes including Crohn’s disease and systemic sclerosis.  The present case describes PCI associated with pneumoperitoneum and ascites as the initial presentation of systemic sclerosis in a 64-year-old female with a history of Crohn’s disease.  The diagnosis of systemic sclerosis was delayed as PCI was initially attributed Crohn’s disease.  This report reviews the pathogenesis, clinical presentation, and management of PCI in relation to both diseases.