Outcome of Total Colonic Aganglionosis Involving the Small Bowel Depends on Bowel Length, Liver Disease, and Enterocolitis.
J Pediatr Gastroenterol Nutr
; 74(5): 582-587, 2022 05 01.
Article
in En
| MEDLINE
| ID: mdl-35175997
ABSTRACT
OBJECTIVES:
Total colonic aganglionosis involving the small bowel is a rare form of Hirschsprung disease. We aim to analyse the long-term outcomes, digestive autonomy, and complications, to suggest recommendations for prevention and treatment.METHODS:
All patients born between 2000 and 2015 followed in our centre were retrospectively included. We analysed the length of aganglionosis, surgical procedures, growth, duration of parenteral nutrition (PN), enterocolitis, liver disease, intestinal transplantation.RESULTS:
Twenty-five patients were followed for a median of 10.9âyears. Fifteen patients had less than 80âcm of ganglionic small bowel (SB) with a median of 20âcm. Ten patients had more than 80âcm of ganglionic sB with a median of 115âcm. The median PN duration was significantly shorter for patients with more than 80âcm 0.9 versus 7.5âyears in those with less than 80âcm (Pâ <â0.001). No patient with less than 80âcm was weaned off PN, except 1 who underwent intestinal transplantation. Ten patients with less than 80âcm develop enterocolitis on the excluded segment, leading to emergency entero-colectomy in 5. Liver disease was more frequent in patients with less than 80âcm (11 vs 0). Three patients required combined liver-intestine transplantation; 2 underwent an isolated intestinal transplantation.CONCLUSIONS:
Digestive autonomy was possible in most patients with more than 80âcm of ganglionic SB. The more severe complication was enterocolitis. Liver disease compromised long-term survival without transplantation. Both complications should be prevented by early diversion and enterectomy of the whole aganglionic segment. Follow-up in or together with a multidisciplinary intestinal rehabilitation centre is suggested.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Short Bowel Syndrome
/
Enterocolitis
/
Hirschsprung Disease
/
Liver Diseases
Type of study:
Etiology_studies
/
Guideline
/
Observational_studies
Limits:
Humans
/
Infant
Language:
En
Journal:
J Pediatr Gastroenterol Nutr
Year:
2022
Document type:
Article