2605 Distal Intestinal Obstruction Syndrome Resulting in Gastric Perforation in a Patient With Cystic Fibrosis

Abstract

INTRODUCTION: Distal Intestinal Obstruction Syndrome (DIOS) is a unique entity that occurs in patients with cystic fibrosis. DIOS results from the accumulation of mucous and undigested food contents in the small intestine due to decreased luminal water secretion which ultimately leads a proximal obstruction that can cause perforation, shock, and death if not treated. DIOS has an incidence rate of 35 in 1000 adults with cystic fibrosis. Here, we present a unique of DIOS which resulted in gastric perforation. CASE DESCRIPTION/METHODS: A 43-year-old male with cystic fibrosis and double lung transplant was admitted with cramping abdominal pain and decreased oral intake. On admission, he was noted to have abnormal liver function tests with total bilirubin of 9.6 and alkaline phosphatase of 888. A CT scan showed dilation of his common bile duct to 1 cm with ultrasound demonstrating distal common bile duct stones. He underwent endoscopic retrograde cholangiopancreatography with stone extraction and improvement in abdominal symptoms. He received significant narcotics for pain, and his hospital course was then complicated by constipation, worsening abdominal pain, and emesis. Gastroenterology was consulted and recommended a nasogastric tube (NG) and hyperosmolar enema to treat DIOS. An NG tube was unable to be passed and the patient’s abdominal pain suddenly worsened. An upright x-ray was obtained and demonstrated lucency over the right crus with non-contrast CT confirming pneumoperitoneum suggestive of a small bowel perforation. He was emergently taken to the operating room where he was found to have a 2 cm gastric perforation. A gastric wedge resection and omental patch was performed. The patient’s post-operative course involved total parenteral nutrition, scheduled enemas, and strict NPO status. An upper GI series showed no extravasation of contrast. The patient was extubated, started on oral intake, and eventually discharged on an aggressive bowel regimen. DISCUSSION: This case highlights a rare complication of DIOS. Typically, patient with DIOS are at risk for intestinal perforation; however, this patient developed a gastric perforation possibly from prior area of structural weakness. This case also highlights the importance of an aggressive bowel regimen in patients with cystic fibrosis to prevent DIOS. Once DIOS is identified, hyperosmolar enemas such as hypaque enemas and oral laxatives containing polyethylene glycol should be used for aggressive treatment to stymie such complications.