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.

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