Urological disorders are still the leading cause of death in patients with spinal dysraphism

Introduction/Background Upper urinary tract damage due to neurogenic lower urinary tract dysfunction has long been reported as the first cause of death in spina bifida patients. However, recent data regarding the causes of death of patients with spinal dysraphism are scarce. The aim of the present study was to assess the current epidemiology of spina bifida in France report the contemporary causes of in-hospital death of spina bifida patients. Material and method We analyzed data from the French national code registry database (Programme de Medicalisation des Systemes d’Information) from 2009 to 2014 to estimate the number of living births of spina bifida patients and the number of in-hospital deaths and their cause categorized as follows: urological, pulmonary, neurological, digestive, muscular/skin, cardiovascular, skeletal, infectious, cancer, others. The prevalence was calculated by doing the ratio of spina bifida living births over the total number of living births per year based on the Institut National de la Statistique et des Etudes Economiques (INSEE) data. Results The number of spina bifida living births per year ranged from 113 (2009) to 131 (2013) and was stable over the study period. The total number of living births during this period was between 811,510 (2013) and 832,799 (2010). The prevalence of spina bifida in France was therefore estimated between 1.3 and 1.6/100,000. The number of in-hospital deaths of spina bifida patients per year fluctuated between 14 (2012) and 31 (2011) making a total of 138 in-hospital deaths over the study period. The leading cause of in-hospital death was urological disorders (17%). Other main causes of death were pulmonary disorders (17%), neurological disorders (14%) and bowel disorders (11%). Conclusion The current prevalence of spina bifida in France range from 1.3 to 1.6/100,000. Urological disorders remain the leading cause of death in spina bifida patients.