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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., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: To investigate computed tomography (CT) texture analysis of the bladder wall as a predictor of urodynamics findings in adult patient with spina bifida., Methods: A single-center prospective trial was conducted from March 2015 to March 2017 including all consecutive adult spina bifida patients seen for urodynamic testing. A contrast-enhanced abdominal CT was systematically performed in all patients during the same visit. Texture features of the bladder wall related to the gray-level histogram and gray-level co-occurrence were evaluated on CT images. Multivariate analysis was performed to identify independent predictors of poor bladder compliance and detrusor overactivity among clinical and texture parameters., Results: Fourty patients were included. The Lasso penalized logistic regression analysis identified 2 texture parameters as potential predictors of poor bladder compliance: Skewness (coefficient weight, -1.81) and S.1.1.SumVarnc (coefficient weight, -3.52). Multivariate logistic regression analysis confirmed skewness (odds ratio [confidence interval 95%] = 0.40 [0.14, 0.97], P = .04) as an independent predictor of poor bladder compliance. The Lasso penalized logistic regression analysis identified one texture parameters as potential predictor of detrusor overactivity: Kurtosis (coefficient weight, -3.52), which was confirmed in multivariate logistic regression analysis (odds ratio [confidence interval 95%] = 1.12 [1.01, 1.55], P = .02)., Conclusion: Our findings demonstrate that CT texture analysis of the bladder wall might be an interesting tool to identify spina bifida patients with high risk urodynamic features., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aims: To assess the predictive values of six urinary markers (nerve growth factor [NGF], brain-derived neurotrophic factor [BDNF], matrix metalloproteinase 2 [MMP-2], tissue inhibitor metalloproteinase 2 [TIMP-2], transformation growth factor β-1 [TGF-B1], and prostaglandin 2 [PGE2]) for adverse urodynamic features and for upper urinary tract damage in adult patients with spina bifida., Materials and Methods: A single-center prospective trial was conducted from March 2015 to March 2017 including all consecutive adult patients with spina bifida seen for urodynamic testing. The urine was collected and stored at -80°C. A urodynamic and an upper urinary tract were systematically performed. At the end of the inclusion period, urines were defrosted and urinary nerve growth factor, BDNF, TIMP-2, and TGF-B1 were assessed using validated ELISA kits. The urinary markers levels were adjusted on the urinary creatinine level. Urinary MMP-2 levels were assessed by zymography., Results: Fourty patients were included. Only TIMP-2 and MMP-2 were significantly associated with poor bladder compliance (P = .043 and P = .039, respectively). TIMP-2 was also the only urinary marker significantly associated with upper urinary tract damage on imaging (OR = 19.81; P = .02). Of all urodynamic parameters, bladder compliance and maximum detrusor pressure were the only ones associated with upper urinary tract damage on imaging (P = .01 and P = .02), The diagnostic performances of urinary TIMP-2 for upper urinary tract damage were slightly superior to PdetMax and bladder compliance with an area under the curve of 0.72., Conclusion: Urinary TIMP-2 and MMP-2 were significantly associated with poor bladder compliance and urinary TIMP-2 was significantly associated with upper urinary tract damage. These findings support a pathophysiological role of extracellular matrix remodeling in poor bladder compliance of adult patients with spina bifida., (© 2019 Wiley Periodicals, Inc.)

Aims: Spina Bifida (SB) is a rare congenital condition that frequently impairs the neurological control of both fecal continence and defecation. Several therapeutic strategies have been proposed but impact assessment is lacking. Our objectives were to quantify the symptomatic improvement and to determine the optimal strategy in this rare condition where randomized controlled trials are difficult to conduct., Methods: Data were extracted from a prospective database. The present analysis focused on patients having undergone at least two gastroenterological assessments. A standardized therapeutic approach was used from the first visit. Improvement was quantified by the variation of quantified symptomatic scores., Results: The data of of 57 adults with SB (gender F/M: 30/27 [52.6/47.4%]; mean age: 33.8 [18.5] years) were extracted. After a mean follow-up of 46 months, 23/57 patients (40.4%) had at least improvement of one point of the Cleveland Clinic Incontinence score (CCIS); 13/57 (22.8%) reported a significant improvement of continence (delta score >50%). Five of the twelve patients (41.6%) with CCIS < 5 at baseline became incontinent over time. The neurological level was not associated with a worse continence outcome. Work on stool consistency and transanal irrigation were the most useful strategies in those with significant improvement of continence., Conclusions: Using conventional strategies, a benefit on fecal continence occurs in only one out of five patients suffering from Spina Bifida and continent patients at baseline can develop fecal incontinence over time. A strategy targeting improved control of defecation (transanal irrigation) and a standardization of follow-up protocol might be beneficial., (© 2018 Wiley Periodicals, Inc.)

Aims: To assess the relative risks of pelvic organ prolapse (POP) and urinary complications in adult spina bifida patients with neurogenic acontractile detrusor voiding with Valsalva versus those using clean-intermittent catheterization (CIC)., Methods: We conducted a retrospective analysis including all spina bifida patients with neurogenic acontractile detrusor with a minimum follow-up of 12 months. Patients were then divided in two groups according to their bladder management: voiding with Valsalva versus CIC. The primary endpoint was any de novo or worsened rectal and/or pelvic organ prolapse (POP) diagnosed during follow-up. The secondary outcome was urinary complications defined as febrile urinary tract infections (UTI) and/or urolithiasis and/or renal failure., Results: Fifty-five patients (50.9% were males) met the inclusion/exclusion criteria: 28 voiding with Valsalva and 27 performing CIC. At baseline, the rates of vaginal prolapse (44.4% vs 50%; P = 0.99), and rectal prolapse/intussusception (25.9% vs 21.4%; P = 0.76) were similar in both groups. After a median follow-up of 80.6 and 65.6 months, respectively (P = 0.29), the rate of de novo or worsened rectal prolapse/intussusception was higher in the Valsalva voiding group than in the CIC group (32.1% vs 3.7%; P = 0.01). De novo or worsened vaginal prolapses were also more common in the Valsalva voiding group, but it did not reach statistical significance (33.3% vs 11.1%; P = 0.29)., Conclusions: Valsalva voiding might be harmful in adult spina bifida patients with neurogenic acontractile detrusor as it may increase the risk of rectal prolapse/intussusception. Overall, the prevalence of POP and rectal prolapse was high in both groups., (© 2018 Wiley Periodicals, Inc.)

Aims: To compare the neurogenic lower urinary tract dysfunction (NLUTD) in patients with closed spinal dysraphism (CSD) versus patients with open spinal dysraphism (OSD) as well as their management patterns., Methods: A prospective cross-sectional study was conducted between September 2007 and December 2015 including all spina bifida patients seen at the multidisciplinary French national referral center for spina bifida. NLUTD and its management were compared between the OSD and CSD groups., Results: Three hundred and eighteen patients were included for analysis: 100 with a CSD (31.5%) and 218 with an OSD (68.6%). The prevalence of urinary incontinence did not differ significantly between the two groups (43% vs 52.8%; P = 0.11), the mean Qualiveen score was also similar (2.7 vs 2.5, P = 0.22). The voiding mechanism was clean intermittent catheterization, spontaneous voiding, suprapubic tube, and ileal conduit in 55% versus 44%; 29.8% versus 47%; 2.8% versus 3% and 11.9% versus 6% of OSD and CSD patients, respectively (P = 0.02). There were comparable prevalences of detrusor overactivity (36.5% vs 38.8%; P = 0.68) and impaired bladder compliance (34.9% vs 31.7%; P = 0.56) in both groups. Augmentation cystoplasty was more common in patients with OSD (32.1% vs 11%; P < 0.0001)., Conclusions: In this prospective cohort, NLUTD were more common in OSD with a higher rate of patients requiring a surgical treatment and a lower rate of patients with preserved spontaneous voiding. However, when present, NLUTD was as severe and troublesome in patients with closed versus open spinal dysraphism., (© 2018 Wiley Periodicals, Inc.)