BACKGROUND: Hirschsprung's disease (HD) is widely quoted to have an incidence of approximately 1 in 5000 live births. However, it is also known that regional variation in incidence can occur. The aim of this paper was to study the incidence of Hirschsprung's disease in Tasmania and to document a regional experience of HD. METHODS: A retrospective review of all patients with newly diagnosed HD between January 1998 and December 2005 was carried out. Data were extracted from medical records and reported. Tasmanian birth statistics from 1998 to 2005 were obtained from the Australian Bureau of Statistics website and the incidence was calculated. RESULTS: Over the 8-year study period, 14 new cases of HD were identified in Tasmania. The estimated incidence of HD in Tasmania based on this study is 1 in 3429. This incidence is higher than the widely quoted incidence of 1 in 5000, but the difference did not reach statistical significance. Between 2003 and 2005, there was a surge in the number of cases with 11 of 14 cases being derived from these 3 years. No obvious reasons were found to explain this surge in the number of cases. Abdominal distension and vomiting were most common modes of presentation (100 and 71%, respectively). There was delayed passage of meconium in 43%. CONCLUSION: The incidence of Hirschsprung's disease seems higher in Tasmania. The surge in the number of cases is also of interest, although the reasons behind this remain unknown. Delayed passage of meconium was also less common than previously cited.
BACKGROUND:Hirschsprung's disease (HD) is widely quoted to have an incidence of approximately 1 in 5000 live births. However, it is also known that regional variation in incidence can occur. The aim of this paper was to study the incidence of Hirschsprung's disease in Tasmania and to document a regional experience of HD. METHODS: A retrospective review of all patients with newly diagnosed HD between January 1998 and December 2005 was carried out. Data were extracted from medical records and reported. Tasmanian birth statistics from 1998 to 2005 were obtained from the Australian Bureau of Statistics website and the incidence was calculated. RESULTS: Over the 8-year study period, 14 new cases of HD were identified in Tasmania. The estimated incidence of HD in Tasmania based on this study is 1 in 3429. This incidence is higher than the widely quoted incidence of 1 in 5000, but the difference did not reach statistical significance. Between 2003 and 2005, there was a surge in the number of cases with 11 of 14 cases being derived from these 3 years. No obvious reasons were found to explain this surge in the number of cases. Abdominal distension and vomiting were most common modes of presentation (100 and 71%, respectively). There was delayed passage of meconium in 43%. CONCLUSION: The incidence of Hirschsprung's disease seems higher in Tasmania. The surge in the number of cases is also of interest, although the reasons behind this remain unknown. Delayed passage of meconium was also less common than previously cited.