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; on behalf of the EUPSA Network Office

doi:10.1055/s-0036-1593991

European Journal of Pediatric Surgery, Table of Contents

Eur J Pediatr Surg 2017; 27(01): 096-101
DOI: 10.1055/s-0036-1593991

Original Article

Georg Thieme Verlag KG Stuttgart · New York

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

Augusto Zani

¹Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

²Department of Paediatric Surgery, Sapienza University of Rome, Rome, Italy

,

Simon Eaton

³Department of Pediatric Surgery, University College London Institute of Child Health, London, United Kingdom

,

Francesco Morini

⁴Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy

,

Prem Puri

⁵Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland

,

Risto Rintala

⁶Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland

,

Ernest van Heurn

⁷Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands

,

Marija Lukac

⁸Department of Pediatric Surgery, Faculty of Medicine, Belgrade, Serbia

⁹Department of Neonatal Surgery, University Children's Hospital, Belgrade, Serbia

,

Pietro Bagolan

⁴Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy

,

Joachim F. Kuebler

¹⁰Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany

,

Florian Friedmacher

¹¹Department of Paediatric Surgery, King's College Hospital, London, United Kingdom

,

Rene Wijnen

¹²Department of Pediatric Surgery Sophia Children's Hospital, ErasmusMC, Rotterdam, The Netherlands

,

Juan A. Tovar

¹³Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain

,

Michael E. Hoellwarth

¹¹Department of Paediatric Surgery, King's College Hospital, London, United Kingdom

,

Agostino Pierro

¹⁴Department of Paediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada

,

on behalf of the EUPSA Network Office

› Author Affiliations

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

congenital megacolon - aganglionosis - Soave - Duhamel - transanal

Full Text

References

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