TY - JOUR
T1 - Management of Hirschsprung disease in Australia and New Zealand
T2 - a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS)
AU - Nataraja, Ramesh Mark
AU - Ferguson, Peter
AU - King, Sebastian
AU - Lynch, Amiria
AU - Pacilli, Maurizio
PY - 2019/4/1
Y1 - 2019/4/1
N2 - 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.
AB - 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.
KW - Diagnosis
KW - Hirschsprung disease
KW - Paediatric surgery survey
KW - Post-operative management
KW - Pull-through procedure
UR - http://www.scopus.com/inward/record.url?scp=85059571102&partnerID=8YFLogxK
U2 - 10.1007/s00383-018-04432-7
DO - 10.1007/s00383-018-04432-7
M3 - Article
AN - SCOPUS:85059571102
VL - 35
SP - 419
EP - 423
JO - Pediatric Surgery International
JF - Pediatric Surgery International
SN - 0179-0358
IS - 4
ER -