European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease

Augusto Zani, Simon Eaton, Francesco Morini, Prem Puri, Risto Rintala, Ernest Van Heurn, Marija Lukac, Pietro Bagolan, Joachim F. Kuebler, Florian Friedmacher, Rene Wijnen, Juan A Tovar, Michael E. Hoellwarth, Agostino Pierro, EUPSA Network Office

Research output: Contribution to journalArticle

Abstract

Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%).

DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77%), acetylcholinesterase (74%), calretinin (31%), S100 (2%), nicotinamide adenine dinucleotide-tetrazolium reductase (2%), succinate dehydrogenase (1%), and neuron-specific enolase (1%). A total of 85% respondents recognize entities including hypoganglionosis (69%), intestinal neuronal dysplasia (55%), and ultrashort segment HD (50%).

SURGERY: Pull-through (PT) is performed at diagnosis by 33% or delayed by 67% (4 months or > 5 kg). Awaiting definitive surgery, 77% perform rectal irrigations, 22% rectal dilatation/stimulations, and 33% perform a stoma. The preferred type of PT is the Soave approach (65%), performed with transanal technique by 70% respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76%; botulinum toxin = 27%), 30% would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4%), at 1 to 6 months (29%), 6 to 12 months (37%) or older (30%). If required, a stoma is sited in the ileum (31%), according to intraoperative biopsies (54%), macroscopic impression (13%), and radiology (2%). Duhamel PT is performed by 52%, Soave by 31%, and Swenson by 17%. Overall, 31% would perform a J-pouch. Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies.

Original languageEnglish
Pages (from-to)96-101
Number of pages6
JournalEuropean Journal of Pediatric Surgery
Volume27
Issue number1
DOIs
Publication statusPublished - Nov 29 2016

Fingerprint

Hirschsprung Disease
Pediatrics
Biopsy
Enema
Disease Management
Calbindin 2
Colonic Pouches
Laxatives
Succinate Dehydrogenase
Botulinum Toxins
Phosphopyruvate Hydratase
Manometry
Suction
Hematoxylin
Acetylcholinesterase
Eosine Yellowish-(YS)
Ileum
Radiology
NAD
Dilatation

Keywords

  • Journal Article

Cite this

European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease. / Zani, Augusto; Eaton, Simon; Morini, Francesco; Puri, Prem; Rintala, Risto; Van Heurn, Ernest; Lukac, Marija; Bagolan, Pietro; Kuebler, Joachim F.; Friedmacher, Florian; Wijnen, Rene; Tovar, Juan A; Hoellwarth, Michael E.; Pierro, Agostino; EUPSA Network Office.

In: European Journal of Pediatric Surgery, Vol. 27, No. 1, 29.11.2016, p. 96-101.

Research output: Contribution to journalArticle

Zani, A, Eaton, S, Morini, F, Puri, P, Rintala, R, Van Heurn, E, Lukac, M, Bagolan, P, Kuebler, JF, Friedmacher, F, Wijnen, R, Tovar, JA, Hoellwarth, ME, Pierro, A & EUPSA Network Office 2016, 'European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease', European Journal of Pediatric Surgery, vol. 27, no. 1, pp. 96-101. https://doi.org/10.1055/s-0036-1593991
Zani, Augusto ; Eaton, Simon ; Morini, Francesco ; Puri, Prem ; Rintala, Risto ; Van Heurn, Ernest ; Lukac, Marija ; Bagolan, Pietro ; Kuebler, Joachim F. ; Friedmacher, Florian ; Wijnen, Rene ; Tovar, Juan A ; Hoellwarth, Michael E. ; Pierro, Agostino ; EUPSA Network Office. / European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease. In: European Journal of Pediatric Surgery. 2016 ; Vol. 27, No. 1. pp. 96-101.
@article{d5cbab1954584783a24bd1399da09f97,
title = "European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease",
abstract = "Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61{\%}).DIAGNOSIS: All respondents perform rectal biopsies (61{\%} rectal suction [RSBs], 39{\%} open full-thickness), 96{\%} contrast enema, and 31{\%} anorectal manometry. At RSB, 17{\%} take the most distal biopsy 1 cm above the dentate line, 34{\%} take 2 cm, 30{\%} take 3 cm, and 19{\%} take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77{\%}), acetylcholinesterase (74{\%}), calretinin (31{\%}), S100 (2{\%}), nicotinamide adenine dinucleotide-tetrazolium reductase (2{\%}), succinate dehydrogenase (1{\%}), and neuron-specific enolase (1{\%}). A total of 85{\%} respondents recognize entities including hypoganglionosis (69{\%}), intestinal neuronal dysplasia (55{\%}), and ultrashort segment HD (50{\%}).SURGERY: Pull-through (PT) is performed at diagnosis by 33{\%} or delayed by 67{\%} (4 months or > 5 kg). Awaiting definitive surgery, 77{\%} perform rectal irrigations, 22{\%} rectal dilatation/stimulations, and 33{\%} perform a stoma. The preferred type of PT is the Soave approach (65{\%}), performed with transanal technique by 70{\%} respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76{\%}; botulinum toxin = 27{\%}), 30{\%} would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4{\%}), at 1 to 6 months (29{\%}), 6 to 12 months (37{\%}) or older (30{\%}). If required, a stoma is sited in the ileum (31{\%}), according to intraoperative biopsies (54{\%}), macroscopic impression (13{\%}), and radiology (2{\%}). Duhamel PT is performed by 52{\%}, Soave by 31{\%}, and Swenson by 17{\%}. Overall, 31{\%} would perform a J-pouch. Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies.",
keywords = "Journal Article",
author = "Augusto Zani and Simon Eaton and Francesco Morini and Prem Puri and Risto Rintala and {Van Heurn}, Ernest and Marija Lukac and Pietro Bagolan and Kuebler, {Joachim F.} and Florian Friedmacher and Rene Wijnen and Tovar, {Juan A} and Hoellwarth, {Michael E.} and Agostino Pierro and {EUPSA Network Office}",
note = "Georg Thieme Verlag KG Stuttgart · New York.",
year = "2016",
month = "11",
day = "29",
doi = "10.1055/s-0036-1593991",
language = "English",
volume = "27",
pages = "96--101",
journal = "European Journal of Pediatric Surgery",
issn = "0939-7248",
publisher = "Thieme Medical Publishers",
number = "1",

}

TY - JOUR

T1 - European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease

AU - Zani, Augusto

AU - Eaton, Simon

AU - Morini, Francesco

AU - Puri, Prem

AU - Rintala, Risto

AU - Van Heurn, Ernest

AU - Lukac, Marija

AU - Bagolan, Pietro

AU - Kuebler, Joachim F.

AU - Friedmacher, Florian

AU - Wijnen, Rene

AU - Tovar, Juan A

AU - Hoellwarth, Michael E.

AU - Pierro, Agostino

AU - EUPSA Network Office

N1 - Georg Thieme Verlag KG Stuttgart · New York.

PY - 2016/11/29

Y1 - 2016/11/29

N2 - Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%).DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77%), acetylcholinesterase (74%), calretinin (31%), S100 (2%), nicotinamide adenine dinucleotide-tetrazolium reductase (2%), succinate dehydrogenase (1%), and neuron-specific enolase (1%). A total of 85% respondents recognize entities including hypoganglionosis (69%), intestinal neuronal dysplasia (55%), and ultrashort segment HD (50%).SURGERY: Pull-through (PT) is performed at diagnosis by 33% or delayed by 67% (4 months or > 5 kg). Awaiting definitive surgery, 77% perform rectal irrigations, 22% rectal dilatation/stimulations, and 33% perform a stoma. The preferred type of PT is the Soave approach (65%), performed with transanal technique by 70% respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76%; botulinum toxin = 27%), 30% would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4%), at 1 to 6 months (29%), 6 to 12 months (37%) or older (30%). If required, a stoma is sited in the ileum (31%), according to intraoperative biopsies (54%), macroscopic impression (13%), and radiology (2%). Duhamel PT is performed by 52%, Soave by 31%, and Swenson by 17%. Overall, 31% would perform a J-pouch. Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies.

AB - Aim This study aims to define patterns of Hirschsprung disease (HD) management. Methods An online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members. Results A total of 294 members (61 countries) answered (response rate: 61%).DIAGNOSIS: All respondents perform rectal biopsies (61% rectal suction [RSBs], 39% open full-thickness), 96% contrast enema, and 31% anorectal manometry. At RSB, 17% take the most distal biopsy 1 cm above the dentate line, 34% take 2 cm, 30% take 3 cm, and 19% take > 3 cm. Rectal biopsy staining's are hematoxylin/eosin (77%), acetylcholinesterase (74%), calretinin (31%), S100 (2%), nicotinamide adenine dinucleotide-tetrazolium reductase (2%), succinate dehydrogenase (1%), and neuron-specific enolase (1%). A total of 85% respondents recognize entities including hypoganglionosis (69%), intestinal neuronal dysplasia (55%), and ultrashort segment HD (50%).SURGERY: Pull-through (PT) is performed at diagnosis by 33% or delayed by 67% (4 months or > 5 kg). Awaiting definitive surgery, 77% perform rectal irrigations, 22% rectal dilatation/stimulations, and 33% perform a stoma. The preferred type of PT is the Soave approach (65%), performed with transanal technique by 70% respondents. If symptoms persist after PT, most opt for conservative approach (enemas/laxatives = 76%; botulinum toxin = 27%), 30% would redo the PT. Total colonic aganglionosis: PT is performed in neonates (4%), at 1 to 6 months (29%), 6 to 12 months (37%) or older (30%). If required, a stoma is sited in the ileum (31%), according to intraoperative biopsies (54%), macroscopic impression (13%), and radiology (2%). Duhamel PT is performed by 52%, Soave by 31%, and Swenson by 17%. Overall, 31% would perform a J-pouch. Conclusions Most aspects of HD management lack consensus with wide variations in obtaining a diagnosis. Transanal Soave PT is the most common technique in standard segment HD. Guidelines should be developed to avoid such variability in management and to facilitate research studies.

KW - Journal Article

U2 - 10.1055/s-0036-1593991

DO - 10.1055/s-0036-1593991

M3 - Article

C2 - 27898990

VL - 27

SP - 96

EP - 101

JO - European Journal of Pediatric Surgery

JF - European Journal of Pediatric Surgery

SN - 0939-7248

IS - 1

ER -