法国儿童脊髓畸形的流行病学：基于医学管理数据库的全国横断面研究。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Neurochirurgie Pub Date : 2025-09-05 DOI:10.1016/j.neuchi.2025.101713

C. Hervet , C. Le Roux , C. Gaborit , J. Maheut-Lourmiere , C. Fremont , H. Lardy , L. Grammatico-Guillon , T. Odent

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引用次数: 0

摘要

目的：法国缺乏儿童罕见脊髓畸形的流行病学资料。利用国家医院出院数据库（PMSI），我们研究了护理轨迹，并估计了这些疾病的发病率和死亡率负担。研究设计：我们进行了一项2010年至2020年的全国性历史队列研究，包括C-MAVEM网络范围内诊断为罕见脊髓疾病的儿童。病例通过ICD-10代码识别，采用半自动、多中心验证的提取算法（阳性预测值>80%）。估计了以下情况的发病率和住院死亡率：脊柱裂（SB）、Arnold Chiari综合征（ACS）、脊髓空洞和脊髓空洞（SM）、脊髓空洞（DM）和脊髓水肿（HM）。结果：共有10,114名儿童被确定，相当于每10万人中估计有67人患病。每10万活产婴儿的发病率为：SB 2.6, ACS 2.2, SM 1.0, DM 0.2, HM 0.04。HM的死亡率分别为2.5%、1.7%、1.1%、0.95%。大多数患者在大学医院接受治疗，根据病因和相关畸形，手术治疗存在相当大的异质性。结论：这项研究首次在法国提供了罕见小儿脊髓畸形的全国流行病学估计。使用经过验证的医疗管理数据提取方法可以对这些罕见疾病进行大规模监测，提高对其医疗负担的了解，并支持专门护理和公共卫生战略的规划。

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Spinal cord malformations’s epidemiology in French children: National cross sectional study based on medico-administrative database

Objective

Epidemiological data on rare spinal cord malformations in children are lacking in France. Using the national hospital discharge database (PMSI), we studied the care trajectories and estimated the morbidity and mortality burden of these conditions.

Study design

We conducted a nationwide historical cohort study from 2010 to 2020, including children diagnosed with rare spinal cord diseases within the scope of the C-MAVEM network. Cases were identified through ICD-10 codes using a semi-automated, multicenter-validated extraction algorithm (positive predictive value >80%). Incidence and in-hospital mortality rates were estimated for the following conditions: Spina Bifida (SB), Arnold Chiari syndrome (ACS), Syringomyelia and Syringobulbia (SM), Diastematomyelia (DM), and Hydromyelia (HM).

Results

A total of 10,114 children were identified, corresponding to an estimated prevalence of 67 per 100,000. Incidence rates per 100,000 live births were: 2.6 for SB, 2.2 for ACS, 1.0 for SM, 0.2 for DM, and 0.04 for HM. The mortality was 2.5%, 1.7%, 1.1%, 0.95%, no available for HM, respectively. Most patients were treated in university hospitals with considerable heterogeneity in surgical management depending on etiology and associated malformations.

Conclusion

This study provides, for the first time in France, national epidemiological estimates for rare pediatric spinal cord malformations. The use of a validated medico-administrative data extraction approach enables largescale surveillance of these rare conditions, improves understanding of their healthcare burden, and supports planning for specialized care and public health strategies.

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来源期刊

Neurochirurgie 医学-临床神经学

CiteScore

2.70

自引率

6.20%

发文量

100

审稿时长

29 days

期刊介绍： Neurochirurgie publishes articles on treatment, teaching and research, neurosurgery training and the professional aspects of our discipline, and also the history and progress of neurosurgery. It focuses on pathologies of the head, spine and central and peripheral nervous systems and their vascularization. All aspects of the specialty are dealt with: trauma, tumor, degenerative disease, infection, vascular pathology, and radiosurgery, and pediatrics. Transversal studies are also welcome: neuroanatomy, neurophysiology, neurology, neuropediatrics, psychiatry, neuropsychology, physical medicine and neurologic rehabilitation, neuro-anesthesia, neurologic intensive care, neuroradiology, functional exploration, neuropathology, neuro-ophthalmology, otoneurology, maxillofacial surgery, neuro-endocrinology and spine surgery. Technical and methodological aspects are also taken onboard: diagnostic and therapeutic techniques, methods for assessing results, epidemiology, surgical, interventional and radiological techniques, simulations and pathophysiological hypotheses, and educational tools. The editorial board may refuse submissions that fail to meet the journal''s aims and scope; such studies will not be peer-reviewed, and the editor in chief will promptly inform the corresponding author, so as not to delay submission to a more suitable journal. With a view to attracting an international audience of both readers and writers, Neurochirurgie especially welcomes articles in English, and gives priority to original studies. Other kinds of article - reviews, case reports, technical notes and meta-analyses - are equally published. Every year, a special edition is dedicated to the topic selected by the French Society of Neurosurgery for its annual report.