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 Pacilli

Research output: Contribution to journalArticleResearchpeer-review

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.

Original languageEnglish
Pages (from-to)419-423
Number of pages5
JournalPediatric Surgery International
Volume35
Issue number4
DOIs
Publication statusPublished - 1 Apr 2019

Keywords

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

Cite this

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title = "Management of Hirschsprung disease in Australia and New Zealand: a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS)",
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 = "Diagnosis, Hirschsprung disease, Paediatric surgery survey, Post-operative management, Pull-through procedure",
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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). / Nataraja, Ramesh Mark; Ferguson, Peter; King, Sebastian; Lynch, Amiria; Pacilli, Maurizio.

In: Pediatric Surgery International, Vol. 35, No. 4, 01.04.2019, p. 419-423.

Research output: Contribution to journalArticleResearchpeer-review

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T1 - Management of Hirschsprung disease in Australia and New Zealand

T2 - a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS)

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AU - Ferguson, Peter

AU - King, Sebastian

AU - Lynch, Amiria

AU - Pacilli, Maurizio

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