Abstract : Objective
To assess and analyze the contemporary causes of in-hospital deaths of spina bifida patients.
Methods
It was a cross-sectional observational study of the longitudinal national cohort of all patients hospitalized in French public and private hospitals. We analyzed the data from the French hospital discharge database (Programme de Médicalisation des Systemes d'Information, PMSI) from 2009 to 2014. The number of in-hospital deaths was extracted using the combination of the ICD-10 codes "Q05" or "Q760" and a discharge code = 9.
Results
There were 138 in-hospital deaths of spina bifida patients over the 6-year study period. The median age at death was 41 years (IQR: 25-52). The median age at death was significantly lower in patients with vs without hydrocephalus (26.6 vs 45.5 years; P <.0001). The leading cause of in-hospital death was urologic disorders (n = 24; 17.3%). Other main causes of death were pulmonary disorders (n = 23; 16.7%), neurologic disorders (n = 19; 13.8%), and bowel disorders (n = 15; 10.9%). Upper urinary tract damage accounted for most of the urologic causes of death: 8 patients died from urinary tract infections (33.3%), 7 patients died from renal failure (29.2%), 4 died from bladder cancer (16.7%), and 5 from other urologic causes. The only variable significantly associated with a death from urologic causes was the absence of hydrocephalus (OR = 0.26; P = .009).
Conclusion
Urologic disorders remain the leading cause of in-hospital death in spina bifida patients in France. The present study highlights that efforts to improve the urologic management of the spina bifida population are still greatly needed.
Spina bifida is a birth defect originally defined as an incomplete closure of the neural tube in the caudal region resulting in protrusion of part or all of the content of the spinal canal through this dorsal defect.1 Spina bifida is the most common congenital cause of neurogenic bladder with an incidence of 1/10,000 births in developed countries.1,2 Neurogenic lower urinary tract dysfunction (NLUTD) is present in more than 90% of spina bifida patients and carries a high risk of upper urinary tract damage; namely pyelonephritis, urolithiasis and chronic kidney disease.3 Urologic disorders in general, and upper urinary tract damage due to NLUTD in particular, have for long been reported as the leading cause of death in spina bifida patients.4, 5, 6 However, most of the available data focused on the pediatric population and date back to over 2 decades.4,5 Owing to improvement in healthcare and understanding of this condition, a larger proportion of spina bifida patients survive into adulthood nowadays.7 The second reason why urologic disorders might no longer be the predominant cause of death in spina bifida patients is the significant improvement of neurourologic care over the past 2 decades. The primary aim of the present study was to assess and analyze the contemporary causes of in-hospital deaths of spina bifida patients.
