[Guidelines on the urological management of the adult patient with spinal dysraphism (spina bifida)]. / Recommandations sur la gestion du risque et la prise en charge urologique du patient adulte atteint de dysraphisme spinal (spina bifida).

INTRODUCTION: Improved life expectancy and prenatal screening have changed the demographics of spina bifida (spinal dysraphism) which has presently become a disease of adulthood. Urinary disorders affect almost all patients with spinal dysraphism and are still the leading cause of mortality in these patients. The aim of this work was to establish recommendations for urological management that take into account the specificities of the spina bifida population. MATERIALS AND METHODS: National Diagnosis and Management Guidelines (PNDS) were drafted within the framework of the French Rare Diseases Plan at the initiative of the Centre de Référence Maladies Rares Spina Bifida - Dysraphismes of Rennes University Hospital. It is a collaborative work involving experts from different specialties, mainly urologists and rehabilitation physicians. We conducted a systematic search of the literature in French and English in the various fields covered by these recommendations in the MEDLINE database. In accordance with the methodology recommended by the authorities (Guide_methodologique_pnds.pdf, 2006), proposed recommendations were drafted on the basis of this literature review and then submitted to a review group until a consensus was reached. RESULTS: Bladder dysfunctions induced by spinal dysraphism are multiple and varied and evolve over time. Management must be individually adapted and take into account all the patient's problems, and is therefore necessarily multi-disciplinary. Self-catheterisation is the appropriate micturition method for more than half of the patients and must sometimes be combined with treatments aimed at suppressing any neurogenic detrusor overactivity (NDO) or compliance alteration (anticholinergics, intra-detrusor botulinum toxin). Resort to surgery is sometimes necessary either after failure of non-invasive treatments (e.g. bladder augmentation in case of NDO resistant to pharmacological treatment), or as a first line treatment in the absence of other non-invasive alternatives (e.g. aponeurotic suburethral tape or artificial urinary sphincter for sphincter insufficiency; urinary diversion by ileal conduit if self-catheterisation is impossible). CONCLUSION: Spinal dysraphism is a complex pathology with multiple neurological, orthopedic, gastrointestinal and urological involvement. The management of bladder and bowel dysfunctions must continue throughout the life of these patients and must be integrated into a multidisciplinary context.

[Urethral meatus deformities and urethra hypospadias in women: prevalence, problems and definitions. Study of 12,739 patients]. / Méat urétral enfoui et urètre hypospade chez la femme : prévalence, problématiques et définitions. Étude sur 12 739 patientes.

UNLABELLED: The difficulty to access to the urethral meatus is found in women in relation to morphological abnormalities of urogenital or ectopic locations meatus, whether acquired (urethral meatus buried [UMB]) or congenital reality (urethra hypospadias [UH]). The pathophysiology is not unequivocal with lack of clear and specific studies. PURPOSE: Verify the existence of specific functional problems, assess the prevalence and identify the anatomical features of UMB and UH. MATERIALS: UMB and UH were sought in a computerized database listing the patients attending a neurourology department between 2000 and 2014 for a pelvic-perineal disease. Each case was analysed for specific reached leaks, urinary tract infections and difficulties catheterization. RESULTS: Of the 12,739, 131 patients (1%) met the inclusion criteria, including 18 UH and 113 UMB. Ninety-one patients consulted for urinary disorders of neurological origin. The circumstances of UMB and UH discovery were: perineal systematic review in 63 cases (48%); difficulties of urethral catheterization in 65 cases (49%); urinary incontinence for 3 patients (3%). Urinary tract infections have not led to the discovery of UMB or UH. Difficulties locating the urethral meatus were congenital (UH) in 18 cases (13%). In 113 cases (87%), it was an acquired abnormality (UMB) overweight for 16 patients (12%), troublesome spasticity of the adductor muscles in 18 cases (14%), vulvovaginal atrophy in 5 cases (4%) and a patient (1%) with a pelvic organ prolapse. No details were given for the remaining 72 patients (54%). CONCLUSION: The urethral meatus difficult access due to MUE or UH can be met in a specialized service but the exact concept of MUE be defined. It can be considered as urethral meatus difficult to access and the cause is acquired. Functional impairment may be particularly important in patients requiring catheterization for bladder emptying. LEVEL OF EVIDENCE: 4.