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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)

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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.

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Abbreviations

HD:

Hirschsprung disease

PT:

Pull-through

ANZ:

Australian and New Zealand

ANZAPS:

Australian and New Zealand Association of Paediatric Surgeons

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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.

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Correspondence to Maurizio Pacilli.

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

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Nataraja, R.M., Ferguson, P., King, S. et al. Management of Hirschsprung disease in Australia and New Zealand: a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS). Pediatr Surg Int 35, 419–423 (2019). https://doi.org/10.1007/s00383-018-04432-7

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