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Early diagnosis and treatment of Hirschsprung's disease in New Mexico
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Early diagnosis and surgical treatment of Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Care
Background:
- Hirschsprung's disease, a congenital condition, historically had high mortality rates.
- Advances in diagnostics and surgery reduced mortality, but enterocolitis remains a significant cause of death.
- Enterocolitis is a severe complication associated with Hirschsprung's disease.
Purpose of the Study:
- To evaluate the impact of neonatal diagnosis and prompt surgical intervention on Hirschsprung's disease outcomes.
- To assess the effectiveness of early treatment in preventing fatal enterocolitis.
Main Methods:
- Retrospective analysis of 18 pediatric patients diagnosed with Hirschsprung's disease.
- Focus on the timing of diagnosis (neonatal) and surgical management.
- Assessment of enterocolitis presence at diagnosis and patient survival rates.
Main Results:
- The majority of the 18 children were diagnosed as neonates.
- Four patients (22%) presented with enterocolitis at diagnosis.
- All patients survived following prompt surgical therapy, indicating successful management.
Conclusions:
- Neonatal diagnosis of Hirschsprung's disease is crucial for improved outcomes.
- Prompt surgical treatment following neonatal diagnosis can effectively prevent fatal enterocolitis.
- Early intervention virtually eliminates mortality associated with Hirschsprung's disease.
Abstract:
Four decades ago, the mortality of congenital aganglionosis of the distal part of the colon (Hirschsprung's disease) approached 100 per cent. The development of diagnostic methods and curative operations lowered the mortality to 5 to 10 per cent. Enterocolitis remained the major cause of death. In this series of 18 children with Hirschsprung's disease, the majority were diagnosed as neonates. Although enterocolitis was present at diagnosis in four (22 per cent), all children survived after prompt surgical therapy. Neonatal diagnosis and treatment may prevent the development of fatal enterocolitis, virtually eliminating the mortality of Hirschsprung's disease.