title: European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease.
abstract: AimThis study aims to define patterns of Hirschsprung disease (HD) management.MethodsAn online questionnaire was sent to all European Paediatric Surgeons' Association (EUPSA) members.ResultsA total of 294 members (61 countries) answered (response rate: 61%).
  
  
  METHODS
  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%).
  
  
  METHODS
  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.ConclusionsMost 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.
authors.affiliation: Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Augusto
authors.initials: A
authors.lastname: Zani
authors.affiliation: Department of Pediatric Surgery, University College London Institute of Child Health, London, United Kingdom.
authors.forename: Simon
authors.initials: S
authors.lastname: Eaton
authors.affiliation: Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Morini
authors.affiliation: Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland.
authors.forename: Prem
authors.initials: P
authors.lastname: Puri
authors.affiliation: Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Risto
authors.initials: R
authors.lastname: Rintala
authors.affiliation: Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands.
authors.forename: Ernest van
authors.initials: EV
authors.lastname: Heurn
authors.affiliation: Department of Pediatric Surgery, Faculty of Medicine, Belgrade, Serbia.
authors.forename: Marija
authors.initials: M
authors.lastname: Lukac
authors.affiliation: Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Bagolan
authors.affiliation: Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
authors.forename: Joachim F
authors.initials: JF
authors.lastname: Kuebler
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, London, United Kingdom.
authors.forename: Florian
authors.initials: F
authors.lastname: Friedmacher
authors.affiliation: Department of Pediatric Surgery Sophia Children's Hospital, ErasmusMC, Rotterdam, The Netherlands.
authors.forename: Rene
authors.initials: R
authors.lastname: Wijnen
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Juan A
authors.initials: JA
authors.lastname: Tovar
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, London, United Kingdom.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Hoellwarth
authors.affiliation: Department of Paediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Agostino
authors.initials: A
authors.lastname: Pierro
mesh_terms: D013505:Digestive System Surgical Procedures / Q000379:methods* / Q000706:statistics & numerical data; D005060:Europe; D006627:Hirschsprung Disease / Q000175:diagnosis* / Q000601:surgery*; D006801:Humans; D010372:Pediatrics; D011182:Postoperative Care / Q000379:methods / Q000706:statistics & numerical data; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D012955:Societies, Medical; D013043:Specialties, Surgical