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Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience
S J Singh1, G D H Croaker, P Manglick
1Consultant Paediatric Surgeon, Department of Paediatric Surgery, University Hospital, Queen's Medical Centre, Nottingham NG7 2UH, UK. Shailinder@aol.com
Insights
Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease (HD) is a congenital condition affecting the large intestine, causing functional obstruction.
- Accurate data on HD demographics, incidence, and treatment in Australia were previously limited.
- The Australian Paediatric Surveillance Unit (APSU) initiated a system to gather comprehensive HD data.
Purpose of the Study:
- To collect and analyze data on the demographics, incidence, and clinical features of Hirschsprung's disease in Australian children.
- To investigate associated anomalies, diagnostic methods, and surgical treatments for HD.
- To evaluate the incidence and management of enterocolitis in HD patients.
Main Methods:
- A nationwide prospective study using the APSU system from January 1997 to December 2000.
- Inclusion criteria: Children under 15 years with biopsy-confirmed Hirschsprung's disease.
- Data collected on demographics, family history, associated anomalies, clinical presentation, investigations, and surgical procedures.
Main Results:
- 127 cases of HD reported; 126 with near-complete data.
- 90% of cases presented neonatally, with 57% experiencing delayed meconium passage.
- The Soave procedure (65%) was the most common surgery; primary pull-through (51%) is increasingly utilized. Enterocolitis remains a significant concern (12% pre-operative, 21% post-operative).
Conclusions:
- Hirschsprung's disease is predominantly diagnosed in neonates in Australia.
- The Soave procedure is the preferred surgical approach, with a rising trend in primary pull-through operations.
- Enterocolitis continues to be a significant complication requiring careful management in HD patients.
Abstract:
Hirschsprung's disease (HD) was introduced into the Australian Paediatric Surveillance Unit (APSU) system in 1996 with the objective to collect data on demographics, incidence, family history, associated anomalies, clinical features, investigation, and surgical treatment in Australia. Children under 15 years of age with HD (confirmed by biopsy) were entered into a database maintained at The Children's Hospital at Westmead in Sydney. Nationwide, 127 children with HD were reported from January 1997 to December 2000, and near-complete information was available on 126. Neonatal presentation was seen in 114 cases (90%), whereas 12 (10%) presented post-neonatally. There was a history of delayed passage of meconium (more than 24 h) in 65 of the 114 patients (57%) who presented in the neonatal period. In those presenting post-neonatally there was no history of delayed passage of meconium. Primary pull-through was performed in 42 of the 82 patients (51%) operated upon. The Soave procedure was performed in 53 of the total 82 (65%) operations and 29 of 42(69%) primary pull-through operations. Pre-operative enterocolitis was seen in 15 of 126 (12%) and postoperative enterocolitis in 17 of 82 (21%) children. The majority of children with HD are diagnosed in the neonatal period (90%). Primary pull-through is becoming more popular (51%). The Soave procedure is the most commonly performed operation (65%) in Australia. Pre- and post-operative enterocolitis is still a significant problem.
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