ABSTRACT
Introduction: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal wall. Although rare, it may also involve other regions, such as the mesentery. PCI is classified as primary or secondary and is associated with multiple predisposing factors. It may be associated with either a benign condition or a potentially fatal condition, such as mesenteric ischemia. The objective of this study was to review the medical literature on the rare benign presentations of PCI, excluding cases associated with intestinal ischemia.Methods: We conducted a systematic literature review according to the PRISMA statement. We searched PubMed and LILACS databases for articles published between January 2015 and December 2020 using the following Medical Subject Headings: "pneumatosis cystoides intestinalis" and "pneumoperitoneum," "pneumatosis intestinalis," and "pneumoperitoneum" or "mesenteric pneumatosis."Results: We included 51 articles comprising 58 patients with PCI and pneumoperitoneum. Most patients were men, and mean patient age was 64.9 years. We identified an idiopathic etiology in 29.31% of cases, and the most common predisposing factor was immune dysfunction (29.31%). A total of 24.13% of patients were asymptomatic. The most commons symptoms were abdominal pain (43.10%), nausea and vomiting (41.37%), and abdominal distention (37.93%). Diagnostic surgery was conducted in 26 patients (44.82%). Only 1 patient underwent surgical treatment.Conclusions: PCI is a clinical condition that may have a benign etiology and not require surgery. Treatment of the benign etiology is conservative. Thus, life-threatening conditions should be excluded in all cases.