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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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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DOI: https://doi.org/10.1007/s00383-018-04432-7