PDF herunterladen
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
1   Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
2   Department of Paediatric Surgery, Sapienza University of Rome, Rome, Italy
,
Simon Eaton
3   Department of Pediatric Surgery, University College London Institute of Child Health, London, United Kingdom
,
Francesco Morini
4   Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Prem Puri
5   Department of Paediatric Surgery, National Children's Research Centre, Dublin, Ireland
,
Risto Rintala
6   Department of Paediatric Surgery, Hospital for Children and Adolescents, Helsinki, Finland
,
Ernest van Heurn
7   Department of Pediatric Surgery, AMC/VUMC, Amsterdam, The Netherlands
,
Marija Lukac
8   Department of Pediatric Surgery, Faculty of Medicine, Belgrade, Serbia
9   Department of Neonatal Surgery, University Children's Hospital, Belgrade, Serbia
,
Pietro Bagolan
4   Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy
,
Joachim F. Kuebler
10   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Florian Friedmacher
11   Department of Paediatric Surgery, King's College Hospital, London, United Kingdom
,
Rene Wijnen
12   Department of Pediatric Surgery Sophia Children's Hospital, ErasmusMC, Rotterdam, The Netherlands
,
Juan A. Tovar
13   Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain
,
Michael E. Hoellwarth
11   Department of Paediatric Surgery, King's College Hospital, London, United Kingdom
,
Agostino Pierro
14   Department of Paediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
,
on behalf of the EUPSA Network Office› Institutsangaben
Weitere Informationen

Publikationsverlauf

15. Mai 2016

23. September 2016

Publikationsdatum:
29. November 2016 (online)

Auch verfügbar auf
   Lizenzen und Reprints

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
 

  • References

  • 1 Grosfeld JL. Hirschsprung's disease: A historical perspective—1691–2005. In: Holschneider A, Puri P, , eds. Hirschsprung's Disease and Allied Disorders. Berlin, Germany: Springer-Verlag; 2008: 1-12
    CrossrefSuche in Google Scholar
  • 2 Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung's disease–a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg 1979; 14 (5) 588-597
    CrossrefPubMedSuche in Google Scholar
  • 3 Keckler SJ, Yang JC, Fraser JD , et al. Contemporary practice patterns in the surgical management of Hirschsprung's disease. J Pediatr Surg 2009; 44 (6) 1257-1260 , discussion 1260
    CrossrefPubMedSuche in Google Scholar
  • 4 Huddart SN. Hirschsprung's disease: present UK practice. Ann R Coll Surg Engl 1998; 80 (1) 46-48
    PubMedSuche in Google Scholar
  • 5 Bradnock TJ, Walker GM. Evolution in the management of Hirschsprung's disease in the UK and Ireland: a national survey of practice revisited. Ann R Coll Surg Engl 2011; 93 (1) 34-38
    CrossrefPubMedSuche in Google Scholar
  • 6 Ikeda K, Goto S. Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients. Ann Surg 1984; 199 (4) 400-405
    Suche in Google Scholar
  • 7 Suita S, Taguchi T, Kamimura T, Yanai K. Total colonic aganglionosis with or without small bowel involvement: a changing profile. J Pediatr Surg 1997; 32 (11) 1537-1541
    CrossrefPubMedSuche in Google Scholar
  • 8 Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg 2005; 40 (1) 197-201 , discussion 201–202
    CrossrefPubMedSuche in Google Scholar
  • 9 Zani A, Zani-Ruttenstock E, Eaton S, Pierro A. The value of surveys in pediatric surgery. Eur J Pediatr Surg 2015; 25 (6) 500-503
    Thieme ConnectPubMedSuche in Google Scholar
  • 10 Noblett HR. A rectal suction biopsy tube for use in the diagnosis of Hirschsprung's disease. J Pediatr Surg 1969; 4 (4) 406-409
    CrossrefPubMedSuche in Google Scholar
  • 11 Aldridge RT, Campbell PE. Ganglion cell distribution in the normal rectum and anal canal. A basis for the diagnosis of Hirschsprung's disease by anorectal biopsy. J Pediatr Surg 1968; 3 (4) 475-490
    Suche in Google Scholar
  • 12 Barshack I, Fridman E, Goldberg I, Chowers Y, Kopolovic J. The loss of calretinin expression indicates aganglionosis in Hirschsprung's disease. J Clin Pathol 2004; 57 (7) 712-716
    CrossrefPubMedSuche in Google Scholar
  • 13 Kapur RP, Reed RC, Finn LS, Patterson K, Johanson J, Rutledge JC. Calretinin immunohistochemistry versus acetylcholinesterase histochemistry in the evaluation of suction rectal biopsies for Hirschsprung Disease. Pediatr Dev Pathol 2009; 12 (1) 6-15
    CrossrefPubMedSuche in Google Scholar
  • 14 Morris MI, Soglio DB, Ouimet A, Aspirot A, Patey N. A study of calretinin in Hirschsprung pathology, particularly in total colonic aganglionosis. J Pediatr Surg 2013; 48 (5) 1037-1043
    CrossrefPubMedSuche in Google Scholar
  • 15 Martucciello G, Pini Prato A, Puri P , et al. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies. J Pediatr Surg 2005; 40 (10) 1527-1531
    CrossrefPubMedSuche in Google Scholar
  • 16 Chen JZ, Jamieson DH, Skarsgard ED. Does pre-biopsy contrast enema delay the diagnosis of long segment Hirschsprung's disease?. Eur J Pediatr Surg 2010; 20 (6) 375-378
    Thieme ConnectPubMedSuche in Google Scholar
  • 17 Muller CO, Mignot C, Belarbi N, Berrebi D, Bonnard A. Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung's disease?. Pediatr Surg Int 2012; 28 (6) 597-601
    CrossrefPubMedSuche in Google Scholar
  • 18 Singh SJ, Croaker GD, Manglick P , et al. Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience. Pediatr Surg Int 2003; 19 (4) 247-250
    CrossrefPubMedSuche in Google Scholar
  • 19 Swenson O, Bill Jr AH. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon; an experimental study. Surgery 1948; 24 (2) 212-220
    PubMedSuche in Google Scholar
  • 20 Duhamel B. Retrorectal and transanal pull-through procedure for the treatment of Hirschsprung's disease. Dis Colon Rectum 1964; 7: 455-458
    CrossrefPubMedSuche in Google Scholar
  • 21 Soave F. A new original technique for treatment of Hirschsprung's disease. Surgery 1964; 56: 1007-1014
    PubMedSuche in Google Scholar
  • 22 Rintala RJ, Lindahl HG. Proctocolectomy and J-pouch ileo-anal anastomosis in children. J Pediatr Surg 2002; 37 (1) 66-70
    CrossrefPubMedSuche in Google Scholar
  • 23 Lal DR, Nichol PF, Harms BA, Go LL, Lund DP. Ileo-anal S-Pouch reconstruction in patients with total colonic aganglionosis after failed pull-through procedure. J Pediatr Surg 2004; 39 (7) e7-e9
    CrossrefPubMedSuche in Google Scholar