Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis

Fabio Fusaro, Francesco Morini, Annika Mutanen, Paola De Angelis, Roberto Tambucci, Teresa Capriati, Dominique Hermans, Manila Candusso, Antonella Diamanti, Pietro Bagolan, Mikko Pakarinen

Research output: Contribution to journalArticle

Abstract

OBJECTIVES: Total/Near Total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.

METHODS: Records from children affected by total intestinal aganglionosis and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry (PIRAT) were retrospectively reviewed.

RESULTS: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, four patients had satisfactory stoma output with enteral tolerance without additional procedures, eight underwent ten AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 ml/kg/day (p = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long-term in five of eight patients (63%), and temporarily in one, leading to a reduction of PN requirement from 100% to 70% (p = 0.0231).

CONCLUSION: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce PN requirements. AIR and Intestinal transplantation (ITx) are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.

Original languageEnglish
JournalJournal of Pediatric Gastroenterology and Nutrition
DOIs
Publication statusE-pub ahead of print - Jan 2 2019

Fingerprint

Reconstructive Surgical Procedures
Small Intestine
Transplantation
Jejunostomy
Hirschsprung Disease
Total intestinal Aganglionosis
Ileum
Registries
Rehabilitation
Pediatrics

Cite this

@article{1d2ad52572d94e269314da49bb5f7444,
title = "Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis",
abstract = "OBJECTIVES: Total/Near Total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.METHODS: Records from children affected by total intestinal aganglionosis and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry (PIRAT) were retrospectively reviewed.RESULTS: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, four patients had satisfactory stoma output with enteral tolerance without additional procedures, eight underwent ten AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 ml/kg/day (p = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long-term in five of eight patients (63{\%}), and temporarily in one, leading to a reduction of PN requirement from 100{\%} to 70{\%} (p = 0.0231).CONCLUSION: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce PN requirements. AIR and Intestinal transplantation (ITx) are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.",
author = "Fabio Fusaro and Francesco Morini and Annika Mutanen and {De Angelis}, Paola and Roberto Tambucci and Teresa Capriati and Dominique Hermans and Manila Candusso and Antonella Diamanti and Pietro Bagolan and Mikko Pakarinen",
year = "2019",
month = "1",
day = "2",
doi = "10.1097/MPG.0000000000002260",
language = "English",
journal = "Journal of Pediatric Gastroenterology and Nutrition",
issn = "0277-2116",
publisher = "Lippincott Williams and Wilkins",

}

TY - JOUR

T1 - Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis

AU - Fusaro, Fabio

AU - Morini, Francesco

AU - Mutanen, Annika

AU - De Angelis, Paola

AU - Tambucci, Roberto

AU - Capriati, Teresa

AU - Hermans, Dominique

AU - Candusso, Manila

AU - Diamanti, Antonella

AU - Bagolan, Pietro

AU - Pakarinen, Mikko

PY - 2019/1/2

Y1 - 2019/1/2

N2 - OBJECTIVES: Total/Near Total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.METHODS: Records from children affected by total intestinal aganglionosis and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry (PIRAT) were retrospectively reviewed.RESULTS: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, four patients had satisfactory stoma output with enteral tolerance without additional procedures, eight underwent ten AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 ml/kg/day (p = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long-term in five of eight patients (63%), and temporarily in one, leading to a reduction of PN requirement from 100% to 70% (p = 0.0231).CONCLUSION: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce PN requirements. AIR and Intestinal transplantation (ITx) are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.

AB - OBJECTIVES: Total/Near Total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.METHODS: Records from children affected by total intestinal aganglionosis and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry (PIRAT) were retrospectively reviewed.RESULTS: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, four patients had satisfactory stoma output with enteral tolerance without additional procedures, eight underwent ten AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 ml/kg/day (p = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long-term in five of eight patients (63%), and temporarily in one, leading to a reduction of PN requirement from 100% to 70% (p = 0.0231).CONCLUSION: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce PN requirements. AIR and Intestinal transplantation (ITx) are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.

U2 - 10.1097/MPG.0000000000002260

DO - 10.1097/MPG.0000000000002260

M3 - Article

C2 - 30614951

JO - Journal of Pediatric Gastroenterology and Nutrition

JF - Journal of Pediatric Gastroenterology and Nutrition

SN - 0277-2116

ER -