Pneumatosis cystoides intestinalis as a complication of celiac disease

Pneumatosis cystoides intestinalis (PCI) is an uncommon but important condition characterized by the presence of gas-filled cysts in the submucosa and subserosa of the gastrointestinal tract. PCI has been associated with several clinical settings. We report a case where PCI developed in a patient with known celiac disease. To our knowledge, this type of coincidence has been described in seven prior cases. Since PCI often results in pneumoperitoneum through rupture of the cysts, it is important to differentiate the benign form of pneumoperitoneum, in which no intervention is indicated, from the life-threatening form with intestinal infarction and/or perforation, in which immediate surgery is mandatory. Differentiating between them can be difficult; this explains the high rate of negative laparotomy, which, in the past, occurred in 27% of cases. By performing diagnostic laparoscopy, we were able to establish the diagnosis of PCI and exclude intestinal perforation or infarction, thus permitting the patient to avoid an unnecessary laparotomy. Although diagnostic laparoscopy is not routinely indicated as a diagnostic tool for the detection of PCI, it proved to be a safe and accurate method to differentiate between the benign form of PCI and the life-threatening form. Whenever this differentiation has to be made, we recommend that diagnostic laparoscopy be performed first rather than primary laparotomy. Herein, the laparoscopic features of PCI are described and the coincidence of PCI with celiac disease is reviewed.