51. Recommandations sur la gestion du risque et la prise en charge urologique du patient adulte atteint de dysraphisme spinal (spina bifida)
- Author
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Manunta, Andréa, Peyronnet, Benoît, Olivari-Philiponnet, Camille, Chartier-Kastler, Emmanuel J., Saussine, Christian C., Phé, Véronique, Robain, Gilberte, Denys, Pierre, Even, Alexia, Samson, Emmanuelle, Grise, Phillippe, Karsenty, G., Hascoet, Juliette, Castel-Lacanal, Evelyne, Charvier, Kathleen F., Guinet-Lacoste, Amandine, Chesnel, Camille, Amarenco, Gérard, Haffner, F., Haddad, Mirna, Le Normand, Loïc, Perrouin-Verbe, Marie Aimée, Perrouin-Verbe, Brigitte, de Seze, Marianne, Ruffion, Alain, Gamé, Xavier, CHU Pontchaillou [Rennes], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Sorbonne Université (SU), Hôpital Raymond Poincaré [Garches], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Strasbourg, CHU Tenon [AP-HP], Université de Versailles Saint-Quentin-en-Yvelines - UFR Sciences de la santé Simone Veil (UVSQ Santé), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), CHU Rouen, Normandie Université (NU), Chirurgie urologique et transplantation rénale [Hôpital de la Conception - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Service Médecine physique et de réadaptation [CHU Toulouse], Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Santé publique et médecine publique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Médecine Physique et Réadaptation [Hôpital Henry Gabrielle - Lyon], Hôpital Henry Gabrielle [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Groupe de RecherchE CliniquE en Neuro-urologie [CHU Tenon] (GRC 1 - GREEN ), CHU Marseille, Centre hospitalier universitaire de Nantes (CHU Nantes), Clinique Saint Augustin, and Hospices Civils de Lyon (HCL)
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Quality of life ,Complications ,[SDV]Life Sciences [q-bio] ,Urology ,Urinary incontinence ,Neurogenic ,Spinal dysraphism ,Spina bifida ,Urinary bladder - Abstract
International audience; 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.
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- 2023
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