Zani, A;
Eaton, SJ;
Morini, F;
Puri, P;
Rintala, R;
van Heurn, E;
Lukac, M;
... Pierro, A; + view all
(2017)
European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease.
European Journal of Pediatric Surgery
, 27
(1)
, Article Eur J Pediatr Surg 2017; 27(01): 096-101. 10.1055/s-0036-1593991.
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Abstract
AIMS: 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.
| Type: | Article |
|---|---|
| Title: | European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1055/s-0036-1593991 |
| Publisher version: | http://dx.doi.org/10.1055/s-0036-1593991 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Congenital megacolon - aganglionosis - Soave - Duhamel - transanal |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1529246 |
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