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

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.