Ikenna K. Emelogu, MD, Cynthia N. Tran, MD, Joshua D. Novak, MD, Wendy R. Greene, MD Emory University School of Medicine, Atlanta, GA
Introduction: Cystic fibrosis (CF) can lead to obstructive gastrointestinal disease, such as meconium ileus in children and distal intestinal obstruction syndrome (DIOS) in adults. DIOS is characterized by the accumulation of inspissated stool and viscous secretions in the terminal ileum and proximal colon, leading to complete or incomplete obstruction. Treatment strategies for DIOS are still essentially empirical as there are no randomized controlled trials to guide therapy. We describe a case of a CF patient with DIOS successfully treated with 4% N-acetylcysteine (NAC) and polyethylene glycol (PEG) administered via colonoscopy.
Case Description/Methods: A 46-year-old woman with cystic fibrosis and prior episodes of DIOS presented with several days of abdominal pain and constipation. On exam, her abdomen was distended and diffusely tender to palpation. Abdominal CT showed extensive debris in the distal small bowel with resultant dilation proximally, thickening at the terminal ileum, and a decompressed colon (Fig. 1A). She was initially managed with frequent enemas and oral laxatives including Lubiprostone and PEG via nasogastric tube. Her symptoms worsened and she was taken for urgent colonoscopy. With the patient in supine position, the colonoscope was meticulously advanced to the cecum, traversing several matted, mucoid-adherent folds (Fig. 1B). A mucoid plug was seen extending from the ileocecal (IC) valve to the walls of the cecum (Fig. 1C). The mucoid plug was injected with 4% NAC and PEG. The scope was then advanced into the terminal ileum approximately 20 cm to the proximal end of a large, actively dissolving fecalith with administration of NAC and PEG. A CRE 8-9-10 mm balloon was used to sweep the stool from the distal ileum. Following this maneuver, peristalsis was observed with inspissated stool emanating from the distal ileum towards the IC valve.
Discussion: Optimal management of DIOS in CF patients remains a challenge. Although most cases are relieved with conservative treatments, select patients will need surgery, which is associated with a higher peri- and postoperative morbidity. However, the administration of 4% N-acetylcysteine and polyethylene glycol via colonoscopy offers a viable remedy that may obviate the need for surgery.
Figure: Figure 1A. Abdominal CT showing extensive debris in the distal small bowel. Figure 1B. Matted, mucoid-adherent folds in the colon. Figure 1C. A mucoid plug extending from the ileocecal valve to the walls of the cecum.
Disclosures:
Ikenna Emelogu indicated no relevant financial relationships.
Cynthia Tran indicated no relevant financial relationships.
Joshua Novak indicated no relevant financial relationships.
Wendy Greene indicated no relevant financial relationships.
Ikenna K. Emelogu, MD, Cynthia N. Tran, MD, Joshua D. Novak, MD, Wendy R. Greene, MD. C0199 - Successful Treatment of Distal Intestinal Obstruction Syndrome With N-acetylcysteine and Polyethylene Glycol via Colonoscopy, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.
