Outcome of Total Colonic Aganglionosis Involving the Small Bowel Depends on Bowel Length, Liver Disease, and Enterocolitis.
Journal
Journal of pediatric gastroenterology and nutrition
ISSN: 1536-4801
Titre abrégé: J Pediatr Gastroenterol Nutr
Pays: United States
ID NLM: 8211545
Informations de publication
Date de publication:
01 05 2022
01 05 2022
Historique:
pubmed:
18
2
2022
medline:
20
5
2022
entrez:
17
2
2022
Statut:
ppublish
Résumé
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. 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. Twenty-five patients were followed for a median of 10.9 years. Fifteen patients had less than 80 cm of ganglionic small bowel (SB) with a median of 20 cm. Ten patients had more than 80 cm of ganglionic sB with a median of 115 cm. The median PN duration was significantly shorter for patients with more than 80 cm: 0.9 versus 7.5 years in those with less than 80 cm (P < 0.001). No patient with less than 80 cm was weaned off PN, except 1 who underwent intestinal transplantation. Ten patients with less than 80 cm develop enterocolitis on the excluded segment, leading to emergency entero-colectomy in 5. Liver disease was more frequent in patients with less than 80 cm (11 vs 0). Three patients required combined liver-intestine transplantation; 2 underwent an isolated intestinal transplantation. Digestive autonomy was possible in most patients with more than 80 cm 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.
Identifiants
pubmed: 35175997
doi: 10.1097/MPG.0000000000003415
pii: 00005176-202205000-00006
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
582-587Informations de copyright
Copyright © 2022 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.
Déclaration de conflit d'intérêts
The authors report no conflicts of interest.
Références
Moore SW. Total colonic aganglionosis in Hirschsprung disease. Semin Pediatr Surg 2012; 21:302–309.
Goulet O, Nader EA, Pigneur B, et al. Short bowel syndrome as the leading cause of intestinal failure in early life: some insights into the management. Pediatr Gastroenterol Hepatol Nutr 2019; 22:303–329.
Frykman PK, Short SS. Hirschsprung-associated enterocolitis: prevention and therapy. Semin Pediatr Surg 2012; 21:328–335.
Soh HJ, Nataraja RM, Pacilli M. Prevention and management of recurrent postoperative Hirschsprung's disease obstructive symptoms and enterocolitis: Systematic review and meta-analysis. J Pediatr Surg 2018; 53:2423–2429.
Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 2017; 33:517–521.
Goulet O, Joly F, Corriol O, et al. Some new insights in intestinal failure- associated liver disease. Curr Opin Organ Transplant 2009; 14:256–261.
Lacaille F, Irtan S, Dupic L, et al. Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center. Transpl Int 2017; 30:178–186.
Fusaro F, Morini F, Mutanen A, et al. Autologous intestinal reconstructive surgery in the management of total intestinal aganglionosis. J Pediatr Gastroenterol Nutr 2019; 68:635–641.
Ruttenstock E, Puri P. A meta-analysis of clinical outcome in patients with total intestinal aganglionosis. Pediatr Surg Int 2009; 25:833–839.
Saxton ML, Ein SH, Hoehner J, et al. Near-total intestinal aganglio- nosis: long-term follow-up of a morbid condition. J Pediatr Surg 2000; 35:669–672.
Fouquet V, De Lagausie P, Faure C, et al. Do prognostic factors exist for total colonic aganglionosis with ileal involvement? J Pediatr Surg 2002; 37:71–75.
Yeh YT, Tsai HL, Chen CY, et al. Surgical outcomes of total colonic aganglionosis in children: a 26-year experience in a single institute. J Chinese Med Assoc 2014; 77:519–523.
Ieiri S, Suita S, Nakatsuji T, et al. Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan. J Pediatr Surg 2008; 43:2226–2230.
Goulet O, Baglin-Gobet S, Talbotec C, et al. Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children. Eur J Pediatr Surg 2005; 15:95–101.
Goulet O, Colomb-Jung V, Joly F. Role of the colon in short bowel syndrome and intestinal transplantation. J Pediatr Gastroenterol Nutr 2009; 48: (Suppl 2): S66–71.
Stenström P, Brautigam M, Borg H, et al. Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis. J Pediatr Surg 2017; 52:1302–1307.
Laughlin DM, Friedmacher F, Puri P. Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome. Pediatr Surg Int 2012; 28:773–779.
Menezes M, Prato AP, Jasonni V, et al. Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review. J Pediatr Surg 2008; 43:1696–1699.
Li Q, Li L, Jiang Q, et al. The mid-term outcomes of TRM-PIAS, proctocolectomy and ileoanal anastomosis for total colonic aganglio- nosis. Pediatr Surg Int 2016; 32:477–482.
Langer JC, Rollins MD, Levitt M, et al. American Pediatric Surgical Association Hirschsprung Disease Interest Group. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 2017; 33:523–526.
Menezes M, Puri P. Long-term outcome of patients with enterocolitis complicating Hirschsprung's disease. Pediatr Surg Int 2006; 22:316–318.
Khen-Dunlop N, Sarnacki S, Victor A, et al. Prenatal intestinal obstruction affects the myenteric plexus and causes functional bowel impairment in fetal rat experimental model of intestinal atresia. PLoS One 2013; 8:e62292.
Yildiz HM, Carlson TL, Goldstein AM, et al. Mucus barriers to microparticles and microbes are altered in Hirschprung's disease. Macromol Biosci 2015; 15:712–718.
Gosain A, Brinkman AS. Hirschsprung's associated enterocolitis. Curr Opin Pediatr 2015; 27:364–369.
Pierre JF, Barlow-Anacker AJ, Erickson CS, et al. Intestinal dysbiosis and bacterial enteroinvasion in a murine model of Hirschsprung's disease. J Pediatr Surg 2014; 49:1242–1251.
Han-Geurts IJM, Hendrix VC, De Blaauw I, et al. Outcome after anal intrasphincteric Botox injection in children with surgically treated Hirschsprung disease. J Pediatr Gastroenterol Nutr 2014; 59:604–607.
Hukkinen M, Koivusalo A, Merras-Salmio L, et al. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J Pediatr Surg 2015; 50:1859–1864.
Sharif K, Beath SV, Kelly DA, et al. New perspective for the management of near-total or total intestinal aganglionosis in infants. J Pediatr Surg 2003; 38:25–28.
Escobar MA, Grosfeld JL, West KW, et al. Long-term outcomes in total colonic aganglionosis: a 32-year experience. J Pediatr Surg 2005; 40:955–961.
Goulet OJ, Cai W, Seo JM. Lipid emulsion use in pediatric patients requiring long-term parenteral nutrition. J ParenterEnter Nutr 2020; 44 (S1):S55–67.
Goulet O, Lamaziere A, Abi Nader E, et al. Erythrocyte fatty acid membrane composition in children on long-term parenteral nutrition enriched with omega-3 fatty acids. Am J Clin Nutr 2021; 115:422–431.
Sauvat F, Grimaldi C, Lacaille F, et al. Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children. J Pediatr Surg 2008; 43:1833–1838.
Courbage S, Canioni D, Talbotec C, et al. Beyond 10 years, with or without an intestinal graft: Present and future? Am J Transplant 2020; 20:2802–2812.
Carter BA, Cohran VC, Cole CR, et al. Outcomes from a 12-week, openlabel, multicenter clinical trial of teduglutide in pediatric short bowel syndrome. J Pediatr 2017; 181: 102.e5–111.e5.
Sigalet DL. Advances in glucagon like peptide-2 therapy. physiology, current indications and future directions. Semin Pediatr Surg 2018; 27:237–241.
Sigalet DL, Lam V, Brindle M, et al. The glucagon like peptide-2 ‘axis’: capacity for production and response following intestinal resection or repair of gastroschisis in infants. J Pediatr Surg 2018; 53:898–904.
Kocoshis SA, Merritt RJ, Hill S, et al. Safety and efficacy of teduglutide in pediatric patients with intestinal failure due to short bowel syndrome: a 24-week, phase III study. J Parenter Enter Nutr 2020; 44:621–631.