TY - JOUR
T1 - European Paediatric Surgeons' Association Survey on the Management of Hirschsprung Disease
AU - EUPSA Network Office
AU - Zani, Augusto
AU - Eaton, Simon
AU - Morini, Francesco
AU - Puri, Prem
AU - Rintala, Risto
AU - Heurn, Ernest van
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
PY - 2017/2
Y1 - 2017/2
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 - Digestive System Surgical Procedures
KW - Europe
KW - Hirschsprung Disease
KW - Humans
KW - Journal Article
KW - Pediatrics
KW - Postoperative Care
KW - Practice Patterns, Physicians'
KW - Societies, Medical
KW - Specialties, Surgical
U2 - https://doi.org/10.1055/s-0036-1593991
DO - https://doi.org/10.1055/s-0036-1593991
M3 - Article
C2 - 27898990
SN - 0939-7248
VL - 27
SP - 96
EP - 101
JO - European journal of pediatric surgery
JF - European journal of pediatric surgery
IS - 1
ER -