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Multidisciplinary management of people with spina bifida across the lifespan

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Abstract

The average worldwide prevalence of neural tube defects (NTDs) is 1.0 per 1000 births. Its development is multifactorial due to genetic and non-genetic factors. Spina bifida (SB) is one of main representatives of NTD. The spinal cord lesion level is the main determinant of the level of paralysis, numbness, and difficulties with bladder/bowel functions. Myelomeningocele prenatal repair reduces hydrocephalus and hindbrain herniation and improves motor function. The severity of hydrocephalus is associated with poorer neurodevelopmental outcomes whether operated on prenatally or after birth. People with SB tend to have a lower IQ and cognitive difficulties. Early diagnosis, proactivity, and lifelong multidisciplinary follow-up are key protective issues. Invasive urological interventions should be considered in selected patients after failure of conservative treatment. Transition to adult care should be well planned as it is challenging. Health literacy is directly associated with success at transition. Sexuality and fertility should be addressed before/during puberty. Overall, the rates of fecal and urinary continence and skin breakdown increase with age, whereas the ability to ambulate declines with age. Bowel and urinary incontinence are independent predictors of lower health-related quality of life (HRQoL) in adults with SB. Bowel incontinence has negative impact on HRQoL regardless of frequency or amount. Long-term caregiver support should be offered at diagnosis. Survival at a mean of 50 years is poor, at 32%, due to central nervous system deaths, cancer, urological disease, and sepsis. Challenges to implementation of recommended practices exist, especially in low and middle-income countries.

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  1. Pediatric Nephrology Unit, Department of Pediatrics, University of São Paulo Medical School, Children’s Hospital, Hospital das Clinicas, Sau Paulo, Brazil

    Vera H. Koch, MarcosTomasin Lopes & Erika Furusawa

  2. Division of Urology, Federal University of Bahia - UFBA, Salvador, BA, Brazil

    Katharinne Vaz

  3. Division of Urology – Federal University of Bahia, Bahiana School of Medicine, Salvador, BA, Brazil

    Ubirajara Barroso

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Koch, V.H., Lopes, M., Furusawa, E. et al. Multidisciplinary management of people with spina bifida across the lifespan. Pediatr Nephrol 39, 681–697 (2024). https://doi.org/10.1007/s00467-023-06067-w

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