A Case of Pneumatosis Cystoides Intestinalis in a Patient with COPD

Abstract

Pneumatosis cystoides intestinalis (PCI) is a rare disease characterized by the presence of gaseous cysts containing nitrogen, hydrogen, and carbon dioxide in the intestinal wall. The exact etiology is unknown, however various theories have linked the occurrence of PCI to mechanical, bacterial, and pulmonary causes. Most patients with PCI remain asymptomatic, however some may present with a constellation of relatively non-specific symptoms including abdominal pain, abdominal distention, hematochezia, and constipation. Left untreated, severe complications including volvulus, adhesions, pneumoperitoneum, and bleeding can occur. A 70 year old male with a past medical history of iron deficiency anemia and COPD was evaluated for worsening microcytic anemia. A prior colonoscopy in 2014 showed nodular mucosa with normal biopsies. An esophagogastroduodenoscopy (EGD) showed only mild duodenitis. A drop in hemoglobin from 11.3 to 9.2 g/dl over 1 year prompted repeat colonoscopy. This revealed numerous submucosal cysts along the ileocecal valve, cecum, ascending colon, hepatic flexure, and transverse colon with overlying hemorrhagic mucosa which was friable and bled on contact. Biopsy of these firm submucosal cysts demonstrated an underlying air filled cavity with collapsibility, a finding consistent with pneumatosis cystoides intestinalis. He then underwent a CT of his chest, abdomen, and pelvis which showed colonic pneumatosis with small amount of pneumoperitoneum likely due to ruptured bleb in the setting of COPD. The etiology of PCI remains unknown, but is thought to be multifactorial. This finding can represent a wide variety of pathologies which range from life threatening to benign. Our patient had a longstanding history of COPD, it was felt that bleb rupture caused the gas to breach the bowel wall leading to formation of cysts. Asymptomatic patients can be observed because the cysts resolve spontaneously in about 50% of patients. In patients with significant symptoms, treatment consists of diet, antibiotics, or the use of hyperbaric oxygen. Urgent surgical intervention would be required for signs of hypotension, sepsis, elevated lactate, and imaging demonstrating gas within the vasculature, linear/cresenteric gas pattern, or small bowel gas. Our case highlights the importance of keeping a broad differential in evaluation of PCI and also to ensure that the patient does not have findings warranting urgent surgical evaluation.