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  • Review Article
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Urinary considerations for adult patients with spinal dysraphism

Nature Reviews Urology volume 12, pages 331–339 (2015)Cite this article

Subjects

Key Points

  • An increasing number of patients with spinal dysraphism reach adulthood, owing to improved care during childhood

  • A completely new group of older patients with spinal dysraphism is, therefore, emerging and requires specialized urological care

  • Well-known issues in patients with spinal dysraphism include neurogenic bladder and pelvic floor dysfunction, which often result in urinary incontinence

  • Attention should also be paid to patients' sexual function, quality of life and the long-term effects of treatments received during childhood

  • Lifelong follow-up monitoring of these patients does seem to be necessary as treatment and management is often complex

Abstract

The incidence of newborns with spinal dysraphism is diminishing worldwide, although survival of individuals with this condition into adulthood continues to improve. The number of adults with spinal dysraphism will, therefore, increase in the coming years, which will pose new challenges in patient management. Urological manifestations of spinal dysraphism can include increased risks of urinary incontinence, urinary tract infection, urinary calculi, sexual dysfunction, end-stage renal disease and iatrogenic metabolic disturbances; however, the severity and incidence of these symptoms varies substantially between patients. Owing to the presence of multiple comorbidities, treatment and follow-up protocols often have to be adapted to best suit the needs of specific patients. Authors describe bladder and kidney function and long-term complications of treatments initiated in childhood, as well as the potential for improvements in quality of life through better follow-up schedules and future developments.

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Figure 1: Schematic representations of different types of spinal dysraphism.
Figure 2: Simplified algorithm of surgical treatment options for management of urinary incontinence in young patients with spinal dysraphism.

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References

  1. Tortori-Donati, P., Rossi, A. & Cama, A. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology 42, 471–491 (2000).

    CAS  PubMed  Google Scholar 

  2. International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) version for 2010. The World Health Organization [online], (2010).

  3. Parker, S. E. et al. Updated national birth prevalence estimates for selected birth defects in the United States, 2004–2006. Birth Defects Res. A. Clin. Mol. Teratol. 88, 1008–1016 (2010).

    CAS  PubMed  Google Scholar 

  4. Fleurke-Rozema, J. H. et al. Impact of introduction of mid-trimester scan on pregnancy outcome of open spina bifida in The Netherlands. Ultrasound Obstet. Gynecol. 43, 553–556 (2014).

    CAS  PubMed  Google Scholar 

  5. Hunt, G. M. 'The median survival time in open spina bifida'. Dev. Med. Child Neurol. 39, 568 (1997).

    CAS  PubMed  Google Scholar 

  6. Oakeshott, P., Hunt, G. M., Poulton, A. & Reid, F. Open spina bifida: birth findings predict long-term outcome. Arch. Dis. Child. 97, 474–476 (2012).

    PubMed  Google Scholar 

  7. Bowman, R. M., McLone, D. G., Grant, J. A., Tomita, T. & Ito, J. A. Spina bifida outcome: a 25-year prospective. Pediatr. Neurosurg. 34, 114–120 (2001).

    CAS  PubMed  Google Scholar 

  8. Davis, B. E. et al. Long-term survival of individuals with myelomeningocele. Pediatr. Neurosurg. 41, 186–191 (2005).

    PubMed  Google Scholar 

  9. Tennant, P. W., Pearce, M. S., Bythell, M. & Rankin, J. 20-Year survival of children born with congenital anomalies: a population-based study. Lancet 375, 649–656 (2010).

    PubMed  Google Scholar 

  10. Roach, J. W., Short, B. F. & Saltzman, H. M. Adult consequences of spina bifida: a cohort study. Clin. Orthop. Relat. Res. 469, 1246–1252 (2011).

    PubMed  Google Scholar 

  11. Veenboer, P. W., Bosch, J. L., van Asbeck, F. W. & de Kort, L. M. Upper and lower urinary tract outcomes in adult myelomeningocele patients: a systematic review. PLoS ONE 7, e48399 (2012).

    CAS  PubMed  PubMed Central  Google Scholar 

  12. Veenboer, P. W., Bosch, J. L., van Asbeck, F. W. & de Kort, L. M. Paucity of evidence for urinary tract outcomes in closed spinal dysraphism: a systematic review. BJU Int. 112, 1009–1017 (2013).

    PubMed  Google Scholar 

  13. Woodhouse, C. R. Myelomeningocele in young adults. BJU Int. 95, 223–230 (2005).

    PubMed  Google Scholar 

  14. Hertzler, D. A. 2nd, DePowell, J. J., Stevenson, C. B. & Mangano, F. T. Tethered cord syndrome: a review of the literature from embryology to adult presentation. Neurosurg. Focus. 29, E1 (2010).

    PubMed  Google Scholar 

  15. Siroky, M. B. The aging bladder. Rev. Urol. 6, S3–S7 (2004).

    PubMed  PubMed Central  Google Scholar 

  16. Veenboer, P. W. et al. Enterocystoplasty vs detrusorectomy: outcome in the adult with spina bifida. J. Urol. 189, 1066–1070 (2013).

    PubMed  Google Scholar 

  17. Vainrib, M., Reyblat, P. & Ginsberg, D. A. Differences in urodynamic study variables in adult patients with neurogenic bladder and myelomeningocele before and after augmentation enterocystoplasty. Neurourol. Urodyn. 32, 250–253 (2013).

    PubMed  Google Scholar 

  18. Cartwright, P. C. & Snow, B. W. Bladder autoaugmentation: partial detrusor excision to augment the bladder without use of bowel. J. Urol. 142, 1050–1053 (1989).

    CAS  PubMed  Google Scholar 

  19. Kennelly, M. J., Gormley, E. A. & McGuire, E. J. Early clinical experience with adult bladder auto-augmentation. J. Urol. 152, 303–306 (1994).

    CAS  PubMed  Google Scholar 

  20. Stohrer, M. et al. Bladder auto-augmentation-an alternative for enterocystoplasty: preliminary results. Neurourol. Urodyn. 14, 11–23 (1995).

    CAS  PubMed  Google Scholar 

  21. Stohrer, M. et al. Bladder autoaugmentation in adult patients with neurogenic voiding dysfunction. Spinal Cord 35, 456–462 (1997).

    CAS  PubMed  Google Scholar 

  22. Westney, O. L. & McGuire, E. J. Surgical procedures for the treatment of urge incontinence. Tech. Urol. 7, 126–132 (2001).

    CAS  PubMed  Google Scholar 

  23. Marte, A. et al. A long-term follow-up of autoaugmentation in myelodysplastic children. BJU Int. 89, 928–931 (2002).

    CAS  PubMed  Google Scholar 

  24. MacNeily, A. E., Afshar, K., Coleman, G. U. & Johnson, H. W. Autoaugmentation by detrusor myotomy: its lack of effectiveness in the management of congenital neuropathic bladder. J. Urol. 170, 1643–1646 (2003).

    PubMed  Google Scholar 

  25. Aslam, M. Z. & Agarwal, M. Detrusor myectomy: long-term functional outcomes. Int. J. Urol. 19, 1099–1102 (2012).

    PubMed  Google Scholar 

  26. Leslie, B. et al. Long-term followup and time to event outcome analysis of continent catheterizable channels. J. Urol. 185, 2298–2302 (2011).

    PubMed  Google Scholar 

  27. Blok, B. et al. Guidelines on Neuro-Urology, Uroweb [online], (2015).

    Google Scholar 

  28. Veenboer, P. W. et al. Cross-sectional study of determinants of upper and lower urinary tract outcomes in adults with spinal dysraphism-new recommendations for urodynamic followup guidelines? J. Urol. 192, 477–482 (2014).

    PubMed  Google Scholar 

  29. Veenboer, P. W. & Bosch, J. L. Long-term adherence to antimuscarinic therapy in everyday practice: a systematic review. J. Urol. 191, 1003–1008 (2014).

    CAS  PubMed  Google Scholar 

  30. Ab, E., Dik, P., Klijn, A. J., van Gool, J. D. & de Jong, T. P. Detrusor overactivity in spina bifida: how long does it need to be treated? Neurourol. Urodyn. 23, 685–688 (2004).

    PubMed  Google Scholar 

  31. Lay, A. H. & Das, A. K. The role of neuromodulation in patients with neurogenic overactive bladder. Curr. Urol. Rep. 13, 343–347 (2012).

    PubMed  Google Scholar 

  32. Andersson, K. E. Beta3-receptor agonists for overactive bladder-new frontier or more of the same? Curr. Urol. Rep. 14, 435–441 (2013).

    PubMed  Google Scholar 

  33. Schlomer, B. J., Saperston, K. & Baskin, L. National trends in augmentation cystoplasty in the 2000s and factors associated with patient outcomes. J. Urol. 190, 1352–1357 (2013).

    PubMed  Google Scholar 

  34. Soljanik, I. Efficacy and safety of botulinum toxin A intradetrusor injections in adults with neurogenic detrusor overactivity/neurogenic overactive bladder: a systematic review. Drugs 73, 1055–1066 (2013).

    CAS  PubMed  Google Scholar 

  35. Kajbafzadeh, A. M. et al. Intravesical electromotive botulinum toxin type A administration—part II: Clinical application. Urology 77, 439–445 (2011).

    PubMed  Google Scholar 

  36. Campbell, J. B., Moore, K. N., Voaklander, D. C. & Mix, L. W. Complications associated with clean intermittent catheterization in children with spina bifida. J. Urol. 171, 2420–2422 (2004).

    PubMed  Google Scholar 

  37. Afsar, S. I., Yemisci, O. U., Cosar, S. N. & Cetin, N. Compliance with clean intermittent catheterization in spinal cord injury patients: a long-term follow-up study. Spinal Cord 51, 645–649 (2013).

    CAS  PubMed  Google Scholar 

  38. Lemelle, J. L. et al. A multicenter evaluation of urinary incontinence management and outcome in spina bifida. J. Urol. 175, 208–212 (2006).

    CAS  PubMed  Google Scholar 

  39. Dik, P., Klijn, A. J., van Gool, J. D., de Jong-de Vos van Steenwijk, C. C. & de Jong, T. P. Early start to therapy preserves kidney function in spina bifida patients. Eur. Urol. 49, 908–913 (2006).

    PubMed  Google Scholar 

  40. Ouyang, L. et al. Characteristics and survival of end stage renal disease (ESRD) patients with spina bifida in the United States Renal Data System (USRDS). J. Urol. 193, 558–564 (2015).

    PubMed  Google Scholar 

  41. Veenboer, P. W. et al. Diagnostic accuracy of Tc-99m DMSA scintigraphy and renal ultrasonography for detecting renal scarring and relative function in patients with spinal dysraphism. Neurourol. Urodyn. http://dx.doi.org/10.1002/nau.22608.

  42. Woodhouse, C. R., Neild, G. H., Yu, R. N. & Bauer, S. Adult care of children from pediatric urology. J. Urol. 187, 1164–1171 (2012).

    PubMed  Google Scholar 

  43. Smith, K., Mizokawa, S., Neville-Jan, A. & Macias, K. Urinary continence across the life course. Pediatr. Clin. North Am. 57, 997–1011 (2010).

    PubMed  Google Scholar 

  44. Wang, H. H., Wiener, J. S., Ferrandino, M. N., Lipkin, M. E. & Routh, J. C. Complications in surgical management of upper tract calculi in spina bifida patients: analysis of nationwide data. J. Urol. 193, 1270–1274 (2015).

    PubMed  Google Scholar 

  45. Veenboer, P. W., Ruud Bosch, J. L., van Asbeck, F. W. & de Kort, L. M. Urolithiasis in adult spina bifida patients: study in 260 patients and discussion of the literature. Int. Urol. Nephrol. 45, 695–702 (2013).

    PubMed  Google Scholar 

  46. Armour, B. S. et al. Hospitalization for urinary tract infections and the quality of preventive health care received by people with spina bifida. Disabil. Health. J. 2, 145–152 (2009).

    PubMed  Google Scholar 

  47. Mann, J. R. et al. Inpatient and emergency room visits for adolescents and young Adults with spina bifida. PM R http://dx.doi.org/10.1016/j.pmrj.2014.11.011

  48. Avallone, M., Prince, M., Guralnick, M. & O'Connor, R. C. Long-term enterocystoplasty follow-up: metabolic and neoplastic concerns. Curr. Bladder Dysfunct. Rep. 9, 302–309 (2014).

    Google Scholar 

  49. Mehan, R., Misseri, R., Vanderbrink, B., Rink, R. C. & Beck, S. D. Metastatic transitional cell carcinoma of the bladder arising in a patient with bladder autoaugmentation. J. Pediatr. Urol. 7, 137–139 (2011).

    PubMed  Google Scholar 

  50. Veenboer, P. W. & de Kort, L. M. Bladder carcinoma in a 31-year-old female spina bifida patient with an auto-augmented bladder. Int. Urol. Nephrol. 44, 1027–1030 (2012).

    PubMed  Google Scholar 

  51. Austin, J. C., Elliott, S. & Cooper, C. S. Patients with spina bifida and bladder cancer: atypical presentation, advanced stage and poor survival. J. Urol. 178, 798–801 (2007).

    PubMed  Google Scholar 

  52. Husmann, D. A. & Rathbun, S. R. Long-term follow up of enteric bladder augmentations: the risk for malignancy. J. Pediatr. Urol. 4, 381–385 (2008).

    CAS  PubMed  Google Scholar 

  53. Higuchi, T. T., Fox, J. A. & Husmann, D. A. Annual endoscopy and urine cytology for the surveillance of bladder tumors after enterocystoplasty for congenital bladder anomalies. J. Urol. 186, 1791–1795 (2011).

    PubMed  Google Scholar 

  54. Hamid, R. et al. Routine surveillance cystoscopy for patients with augmentation and substitution cystoplasty for benign urological conditions: is it necessary? BJU Int. 104, 392–395 (2009).

    PubMed  Google Scholar 

  55. Gilbert, S. M. & Hensle, T. W. Metabolic consequences and long-term complications of enterocystoplasty in children: a review. J. Urol. 173, 1080–1086 (2005).

    PubMed  Google Scholar 

  56. Mingin, G., Maroni, P., Gerharz, E. W., Woodhouse, C. R. & Baskin, L. S. Linear growth after enterocystoplasty in children and adolescents: a review. World J. Urol. 22, 196–199 (2004).

    PubMed  Google Scholar 

  57. Hensle, T. W., Mingin, G. & Fam, M. Metabolic bone loss after urinary diversion in children. Curr. Opin. Urol. 24, 619–623 (2014).

    PubMed  Google Scholar 

  58. Vyas, P. & Crispen, P. L. Metabolic bone disease following urinary diversion in adults. Curr. Opin. Urol. 24, 614–618 (2014).

    PubMed  Google Scholar 

  59. Blackburn, S. C. et al. Ileal bladder augmentation and vitamin B12: levels decrease with time after surgery. J. Pediatr. Urol. 8, 47–50 (2012).

    CAS  PubMed  Google Scholar 

  60. Rosenbaum, D. H. et al. Ileal enterocystoplasty and B12 deficiency in pediatric patients. J. Urol. 179, 1544–1547 (2008).

    PubMed  Google Scholar 

  61. Cardenas, D. D., Topolski, T. D., White, C. J., McLaughlin, J. F. & Walker, W. O. Sexual functioning in adolescents and young adults with spina bifida. Arch. Phys. Med. Rehabil. 89, 31–35 (2008).

    PubMed  Google Scholar 

  62. Verhoef, M. et al. Sex education, relationships, and sexuality in young adults with spina bifida. Arch. Phys. Med. Rehabil. 86, 979–987 (2005).

    PubMed  Google Scholar 

  63. Bong, G. W. & Rovner, E. S. Sexual health in adult men with spina bifida. ScientificWorldJournal 7, 1466–1469 (2007).

    PubMed  PubMed Central  Google Scholar 

  64. Diamond, D. A., Rickwood, A. M. & Thomas, D. G. Penile erections in myelomeningocele patients. Br. J. Urol. 58, 434–435 (1986).

    CAS  PubMed  Google Scholar 

  65. Sandler, A. D., Worley, G., Leroy, E. C., Stanley, S. D. & Kalman, S. Sexual function and erection capability among young men with spina bifida. Dev. Med. Child Neurol. 38, 823–829 (1996).

    CAS  PubMed  Google Scholar 

  66. Palmer, J. S., Kaplan, W. E. & Firlit, C. F. Erectile dysfunction in patients with spina bifida is a treatable condition. J. Urol. 164, 958–961 (2000).

    CAS  PubMed  Google Scholar 

  67. Joyner, B. D., McLorie, G. A. & Khoury, A. E. Sexuality and reproductive issues in children with myelomeningocele. Eur. J. Pediatr. Surg. 8, 29–34 (1998).

    CAS  PubMed  Google Scholar 

  68. Hultling, C., Levi, R., Amark, S. P. & Sjoblom, P. Semen retrieval and analysis in men with myelomeningocele. Dev. Med. Child Neurol. 42, 681–684 (2000).

    CAS  PubMed  Google Scholar 

  69. Overgoor, M. L., de Jong, T. P., Cohen-Kettenis, P. T., Edens, M. A. & Kon, M. Increased sexual health after restored genital sensation in male patients with spina bifida or a spinal cord injury: the TOMAX procedure. J. Urol. 189, 626–632 (2013).

    CAS  PubMed  Google Scholar 

  70. Jacobs, M. A., Avellino, A. M., Shurtleff, D. & Lendvay, T. S. Reinnervating the penis in spina bifida patients in the United States: ilioinguinal-to-dorsal-penile neurorrhaphy in two cases. J. Sex. Med. 10, 2593–2597 (2013).

    PubMed  Google Scholar 

  71. Cass, A. S., Bloom, B. A. & Luxenberg, M. Sexual function in adults with myelomeningocele. J. Urol. 136, 425–426 (1986).

    CAS  PubMed  Google Scholar 

  72. Richmond, D., Zaharievski, I. & Bond, A. Management of pregnancy in mothers with spina bifida. Eur. J. Obstet. Gynecol. Reprod. Biol. 25, 341–345 (1987).

    CAS  PubMed  Google Scholar 

  73. Visconti, D. et al. Sexuality, pre-conception counseling and urological management of pregnancy for young women with spina bifida. Eur. J. Obstet. Gynecol. Reprod. Biol. 163, 129–133 (2012).

    PubMed  Google Scholar 

  74. Sawin, K. J. & Bellin, M. H. Quality of life in individuals with spina bifida: a research update. Dev. Disabil. Res. Rev. 16, 47–59 (2010).

    PubMed  Google Scholar 

  75. Barf, H. A. et al. Life satisfaction of young adults with spina bifida. Dev. Med. Child Neurol. 49, 458–463 (2007).

    CAS  PubMed  Google Scholar 

  76. Singhal, B. & Mathew, K. M. Factors affecting mortality and morbidity in adult spina bifida. Eur. J. Pediatr. Surg. 9, S31–S32 (1999).

    Google Scholar 

  77. The deceased; suicide (residents), various characteristics. Statistics Netherlands [online], (2014).

  78. Begeer, I. H. & Staal-Schreinemachers, A. L. The benefits of team treatment and control of adult patients with spinal dysraphism. Eur. J. Pediatr. Surg. 6, S15–S16 (1996).

    Google Scholar 

  79. Kinsman, S. L. & Doehring, M. C. The cost of preventable conditions in adults with spina bifida. Eur. J. Pediatr. Surg. 6, S17–S20 (1996).

    Google Scholar 

  80. Thibadeau, J. K. et al. Testing the feasibility of a National Spina Bifida Patient Registry. Birth Defects Res. A. Clin. Mol. Teratol. 97, 36–41 (2013).

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Urology, University Medical Centre Utrecht, Room C.04.236, Heidelberglaan 100, P.O. Box 85500, 3584 CX, Utrecht, Netherlands

    Paul W. Veenboer & Laetitia M. O. de Kort

  2. Department of Paediatric Urology Paediatric Renal Centre, Department of Paediatric Urology, University Children's Hospital, UMCU, P.O. Box 85090, 3508 AB, Utrecht, Netherlands

    Rafal J. Chrzan & Tom P. V. M. de Jong

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  1. Paul W. Veenboer
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P.W.V. researched data for this article, P.W.V. and T.P.V.M.d.J. made substantial contributions to discussions of content, and all authors contributed to the writing and the reviewing and/or editing of this article before submission.

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Correspondence to Tom P. V. M. de Jong.

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Veenboer, P., de Kort, L., Chrzan, R. et al. Urinary considerations for adult patients with spinal dysraphism. Nat Rev Urol 12, 331–339 (2015). https://doi.org/10.1038/nrurol.2015.99

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