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

• Published in: European journal of pediatric surgery Vol. 27; no. 1; p. 96
• Main Authors: Zani, Augusto, Eaton, Simon, Morini, Francesco, Puri, Prem, Rintala, Risto, Heurn, Ernest van, Lukac, Marija, Bagolan, Pietro, Kuebler, Joachim F, Friedmacher, Florian, Wijnen, Rene, Tovar, Juan A, Hoellwarth, Michael E, Pierro, Agostino
• Format: Journal Article
• Language: English
• Published: United States 01.02.2017
• Subjects: Digestive System Surgical Procedures - methods; Digestive System Surgical Procedures - statistics & numerical data; Digestive System Surgical Procedures - utilization; Europe; Hirschsprung Disease - diagnosis; Hirschsprung Disease - surgery; Humans; Pediatrics; Postoperative Care - methods; Postoperative Care - statistics & numerical data; Practice Patterns, Physicians' - statistics & numerical data; Societies, Medical; Specialties, Surgical
• ISSN: 1439-359X
• DOI: 10.1055/s-0036-1593991

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