Acta paediatrica (Oslo, Norway : 1992) · 2025
Epidemiology and Surgical Management of Hirschsprung's Disease in France: A 12-Year Retrospective Analysis (2012-2023).
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Wang Xavier Xu, Doisy Wilguy, Goro Seydou, Harichane Sadek, Rousseau Mathilde, Bellaiche Marc, Jung Camille
Аннотация
AIM: Hirschsprung's disease (HSCR) is a congenital disorder of the enteric nervous system, leading to functional intestinal obstruction in neonates and young children. This study aimed to assess the epidemiology and surgical management of HSCR in France over a 12-year period using nationwide data.
METHODS: We conducted a retrospective cohort study using the French National Hospital Discharge Database (PMSI), including patients aged 0 to 3 years diagnosed and surgically treated for HSCR between 2012 and 2023.
RESULTS: Among 1512 patients, the average incidence was 17.26 per 100 000 live births. Short-segment disease accounted for 70% of cases, and 78.6% were male. A progressive shift toward minimally invasive techniques was observed, with laparoscopic surgery increasing significantly. Mortality was low (0.3%). Long-segment disease was associated with higher reoperation rates and lower reoperation-free survival.
CONCLUSION: This study confirms the stable incidence of HSCR in France and highlights the increasing use of minimally invasive surgery. Regional disparities in care and the higher surgical burden in long-segment cases underline the need for ongoing surveillance, equitable access and improved follow-up strategies.
METHODS: We conducted a retrospective cohort study using the French National Hospital Discharge Database (PMSI), including patients aged 0 to 3 years diagnosed and surgically treated for HSCR between 2012 and 2023.
RESULTS: Among 1512 patients, the average incidence was 17.26 per 100 000 live births. Short-segment disease accounted for 70% of cases, and 78.6% were male. A progressive shift toward minimally invasive techniques was observed, with laparoscopic surgery increasing significantly. Mortality was low (0.3%). Long-segment disease was associated with higher reoperation rates and lower reoperation-free survival.
CONCLUSION: This study confirms the stable incidence of HSCR in France and highlights the increasing use of minimally invasive surgery. Regional disparities in care and the higher surgical burden in long-segment cases underline the need for ongoing surveillance, equitable access and improved follow-up strategies.