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[Hirschsprung's disease in children]
Insights
This study on Hirschsprung's disease (HSCR) highlights diagnostic methods and treatment protocols, showing excellent long-term outcomes in 85% of operated patients. Early diagnosis and appropriate surgical intervention are key for managing this congenital condition.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Medical Diagnostics
Context:
- Hirschsprung's disease (HSCR) is a congenital disorder affecting the large intestine.
- The study analyzes 283 cases treated at Hôpital des Enfants-Malades, Paris.
- Focuses on neonatal and post-natal presentations.
Purpose:
- To describe current features, diagnostic modalities, and therapeutic strategies for Hirschsprung's disease.
- To establish a prognosis and treatment protocol based on rectal wall elasticity and colonic distension.
- To detail the pathophysiology, prevention, and treatment of enterocolitis, a major complication.
Summary:
- Diagnostic approaches emphasize manometric and histochemical techniques over radiology.
- Treatment protocols are guided by rectal elasticity and colonic distension.
- Enterocolitis pathophysiology, prevention, and treatment are discussed.
- 100 patients underwent surgery with 5-25 year follow-up.
Impact:
- 85% of surgically treated patients achieved excellent or good long-term results.
- Provides insights into effective management strategies for Hirschsprung's disease.
- Contributes to understanding and managing HSCR complications like enterocolitis.
Abstract:
Based on a series of 283 cases of Hirschsprung's disease seen and treated in the Surgical Clinic of the Hôpital des Enfants-Malades in Paris, the authors describe the current features of the disease which generally presents in the neonatal or post-natal period, the modalities of diagnosis which involve manometric and histochemical techniques more than radiology, the established of a prognosis and a protocol of therapeutic indications based on the evaluation of the coefficient of elasticity of the rectal wall and of the site of colonic distension. The pathophysiology of the major complication, enterocolitis, is described together with methods of prevention and treatment. 100 patients have been operated with a follow-up of between 5 and 25 years. In 85% of cases, the long term results are excellent or good.