Introduction: Management of intestinal failure secondary to severe CIPO is based on parenteral nutrition associated to decompression or diversion enterostomies +/- segmental resections (mainly colectomy). Early prophylactic subtotal enterectomy is not recommended, since small bowel remains partially functional in CIPO. This study evaluates the risk linked to the excluded bowel in such patients.

Methods: Charts of all patients with severe CIPO requiring parenteral nutrition and enterostomies, managed from 1986 to 2017 in our institution, were reviewed. Data about initial management, bowel exclusion, incidence and treatment of enterocolitis and, eventually, transplantation were analyzed.

Results: Forty-two patients were included. Initial management consisted of a complete diverting jejunostomy in 8 patients, ileostomy in 12 and both in 22, leaving an excluded segment of small bowel and colon in 30 and colon in 12 patients.

9 patients used to have daily irrigation and 21 had prophylactic antibiotic therapy.

11 CIPO patients presented at least one episode of enterocolitis (EC). Median age at first EC was 3.6 years [0.9 -12]. EC episodes occurred in the excluded bowel in 4 patients, in the non-excluded intestine in 2, and both excluded and non-excluded intestine were involved in 5 patients.

None of the patient underwent subtotal enterectomy. All patients were treated conservatively, except 3 who underwent 2 ileo-colectomies and 1 diversion, at a median age of 4.6 years [3.6- 9.6].

Finally, 3 patients underwent a liver and intestinal transplantation and 4 underwent an intestinal transplantation.

Overall mortality was 7% (n=3).

Conclusion: CIPO patients have a risk of enterocolitis but mainly mild episodes that are managed conservatively as compared to long segment Hirschsprung disease patients for whom subtotal enterectomy is frequently required. The main goals of CIPO management remain based on nutritional support, fluid/electrolyte restoration, antibiotics for bacterial overgrowth/infections, and control of particularly bothersome symptoms.