Intraoperative intraluminal injection of N-acetylcysteine allowing treatment of distal intestinal obstruction syndrome without the need for enterotomy

Abstract

We describe a case of an 18-year-old man who suffers from cystic fibrosis and developed distal intestinal obstruction syndrome while being treated as an inpatient. Following failed medical management, we proceeded to laparotomy where the small bowel was decompressed with retrograde milking into the stomach, leaving a section of impacted stool in the distal ileum. N-acetylcysteine was injected into the bowel lumen proximal to the obstruction. This resulted in dissolution of the stool without the need for enterotomy and is, to our knowledge, the first successful example of this technique in the literature.

Distal intestinal obstruction syndrome: an important differential diagnosis for abdominal pain in patients with cystic fibrosis

“…DIOS can develop at any age; however, it occurs almost exclusively in patients over the age of 15 and peaks in young adults aged 20–25 years . There is a varying incidence observed in the literature that ranges from 2.3 to 11.3 episodes/1000 patient‐years internationally, and is higher in those aged 20–25 years at 35.5 episodes/1000 patient‐years . A gradual increase in prevalence is being observed, initially less than 7% and now greater then 40%, likely reflecting the increasing life expectancy of CF patients …”

“…In patients with a severe CF genotype, or class I–III mutations where there is no functional CFTR protein, there is an association between the development of DIOS and the degree of impairment in chloride secretion . However, it is not an absolute association as patients with a mild genotype may still suffer from DIOS …”

“…Risk and/or precipitating factors can include: A history of previous episodes of DIOS (10‐fold increase in risk) A history of meconium ileus at birth (almost 50% of cases) Alteration in pancreatic enzyme intake (up to 20% of cases) Dehydration or dietary change CF‐related diabetes. …”

“…Prokinetics have been suggested; however, there remains a lack of evidence base to support their use in DIOS . In mild–moderate cases, the above approach can be trialled and an intestinal lavage with a balanced electrolyte osmotic solution can be given orally or via nasogastric tube …”

“…It can be ‘milked’ carefully into the colon while warm saline mixed with mineral oil is administered through the nasogastric tube and advanced manually to the point of obstruction . If this fails, then an appendicectomy with appendicostomy tube placement for intestinal lavage can be performed with warm saline, diatrizoate meglumine and diatrizoate sodium, 50% pancreatic enzymes or N‐acetylcysteine solution . The softened stool is then gently ‘milked’ into the distal colon or evacuated via the appendicostomy .…”