When Surgery meets Autoimmunity: A Case of Pneumatosis Cystoides Intestinalis mimicking mesenteric infarct in systemic sclerosis

Pneumatosis intestinalis was first identified by Johann Georg Du Vernoy in 1783, at which was later renamed to pneumatosis cystoides intestinalis (PCI) by Mayer in 1825 (1). With the advancement of computed tomography (CT) imaging, PCI is now frequently detected incidentally. However, its management remains a clinical challenge, often requiring individualized decision-making. Here, we present the case of a 42-year-old woman with no known medical history who presented with acute abdominal pain and significant distension. Contrast-enhanced CT of the abdomen and pelvis (CECT AP) revealed pneumoperitoneum with features suggestive of a perforated viscus, dilated small bowel loops, diffuse pneumatosis intestinalis, and reduced mural enhancement in the lower abdomen—raising concern for possible mesenteric infarct. She underwent exploratory laparotomy and peritoneal washout, which revealed minimal clear ascites and grossly dilated small bowels, but no evidence of perforation or necrosis. Postoperatively, she was initiated on corticosteroid therapy. Subsequent investigations confirmed a new diagnosis of diffuse systemic sclerosis. The patient responded well to treatment by the rheumatology team and was discharged in stable condition. This case highlights the diagnostic and therapeutic dilemmas faced by surgeons in managing pneumatosis intestinalis, particularly when it mimics surgical emergencies.