1854 Conservative Management of Pneumatosis Cystoides Intestinalis With Pneumoperitoneum in a Scleroderma-Related Illness

Abstract

INTRODUCTION: Pneumatosis Cystoidea Intestinalis with pneumoperitoneum in scleroderma-related illness could mimic an acute abdomen, presenting with pseudo-obstruction and gas within and outside the intestinal wall in the absence of a true perforation of the intestinal wall. The recognition of the clinical presentation of pseudoobstruction with pneumoperitoneum, due to Pneumatosis Cystoidea Intestinalis is paramount for the clinical decision to manage conservatively and avoid a surgical intervention. CASE DESCRIPTION/METHODS: A 30-year-old female with a 3-year history of limited scleroderma and myositis overlap presented with worsening nausea, vomiting, abdominal pain and bloating for about a week. She had a similar episode about a month prior to presentation, with abdominal imaging showing intestinal wall blebs with pneumoperitoneum, and had undergone an exploratory laparotomy. There was no bowel perforation on exploratory laparotomy and biopsy results showed fibrous tissues lined by reactive mesothelial cells and chronic inflammation. On examination, her abdomen was distended with epigastric tenderness, without guarding or rebound tenderness. An abdominal x-ray and CT scan revealed small bowel obstruction with pneumoperitoneum, features characteristic of Pneumatosis Cystoidea Intestinalis. She was evaluated by gastroenterology, surgery and the rheumatology team, and managed conservatively with intravenous fluids, nasogastric tube decompression, and administration of proton pump inhibitor. Her vital signs were normal and abdominal distension subsided with a resolution of her symptoms after 24 hours of conservative management. She was subsequently able to tolerate diet and was discharged on day 2 of admission with a plan for outpatient follow up. DISCUSSION: The presence of pneumoperitoneum portends a sinister finding in a patient with intestinal obstruction, often requiring surgical intervention. Pneumatosis Cystoides Intestinalis is characterized by micro-perforations of intestinal wall blebs which give rise to pneumoperitoneum, without a true perforation of the intestinal wall. The bacterial overgrowth and mechanical theory are both highlighted as mechanisms for the loss of integrity of the intestinal intimal layer, resulting in leakage and formation of gas within the intestinal wall. However, in the absence of acute peritonitis, abdominal abscess, and a worsening clinical status, Pneumatosis Cysotiodea Intestinalis can be managed conservatively in spite of a pseudo-obstruction with pneumoperitoneum.