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Management of Hirschsprung disease in Australia and New Zealand: a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS)

  • Ramesh Mark Nataraja
  • Peter Ferguson
  • Sebastian King
  • Amiria Lynch
  • Maurizio PacilliEmail author
Original Article

Abstract

Purpose

To define the practice of management for Hirschsprung disease (HD) in Australia and New Zealand.

Methods

Online survey of Australian and New Zealand Association of Paediatric Surgeons (ANZAPS) members.

Results

56/80 (70%) members from 17 centres responded.

Diagnosis

100% perform suction rectal biopsies; 40% perform a contrast enema.

Histopathological staining

H&E (94%), ACHE (70%) and calretinin (75%).

Surgery

Primary pull-through (PT) is performed by 88% (100% by < 6/12 months). The Soave–Boley PT is the preferred approach (85%), with laparoscopic assistance (77%) and muscle cuff division (93%). Routine post-operative dilatations are performed by 63% of respondents. If symptoms persist following PT, majority adopt a conservative approach (enemas/laxatives 90%; Botox 74%). If a long-segment is identified at PT, 60% fashion a stoma and delay definitive surgery. If total colonic aganglionosis is identified at PT, 76% fashion a stoma and delay definitive surgery. A dedicated bowel management program is available in 45% of centres with transition to adult services in 29%.

Conclusions

A laparoscopic-assisted Soave–Boley PT is the most common technique for recto-sigmoid HD. Differences are noted in both the management of long-segment/total aganglionosis HD and post-operative management/follow-up.

Keywords

Hirschsprung disease Paediatric surgery survey Diagnosis Pull-through procedure Post-operative management 

Abbreviations

HD

Hirschsprung disease

PT

Pull-through

ANZ

Australian and New Zealand

ANZAPS

Australian and New Zealand Association of Paediatric Surgeons

Notes

Acknowledgements

The authors thank Mr Russell Taylor, Associate Professor Jonathan Karpelowsky and Ms Terleetha Kruger for the support provided. We thank the ANZAPS members for taking the time to complete the survey. Associate Professor Sebastian King’s position as an Academic Paediatric Surgeon is possible due to a generous grant from The Royal Children’s Hospital Foundation.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

None of the authors has a conflict of interest to declare.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Supplementary material

383_2018_4432_MOESM1_ESM.docx (28 kb)
Supplementary material 1 (DOCX 27 KB)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Paediatric SurgeryMonash Children’s Hospital, MelbourneMelbourneAustralia
  2. 2.Department of Paediatrics, School of Clinical Sciences at Monash Health, Medicine, Nursing and Health SciencesMonash UniversityMelbourneAustralia
  3. 3.Department of Paediatric SurgeryThe Royal Children’s HospitalMelbourneAustralia
  4. 4.Department of PaediatricsUniversity of MelbourneMelbourneAustralia
  5. 5.F. Douglas Stephens Surgical ResearchMurdoch Children’s Research InstituteMelbourneAustralia
  6. 6.Department of Gastroenterology and Clinical NutritionThe Royal Children’s HospitalMelbourneAustralia

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