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Congenital Abnormalities (Meningomyelocele and Spinal Dysraphism)

  • Ryuji Sakakibara
Chapter

Abstract

Cystic and occult spina bifida (SB) presents with a wide spectrum of urodynamic abnormalities including upper and lower neuron types of bladder and urethral dysfunction. Careful uro-neurological assessment and spinal MRI are important for diagnosing young adult patients with occult SB, because they may present with urinary symptoms as the sole initial symptom and have no other obvious neurological abnormalities. Despite consensus regarding the need for early urological management, controversy remains regarding the types of intervention. CIC, anticholinergics, botulinum A toxin, and reconstructive surgery have good outcomes for the primary goals, e.g., protection of renal function, improved urinary continence, and independence. Future studies are warranted to maximize bladder quality of life in the patients with SB.

Keywords

Spina bifida Cystic spina bifida Occult spina bifida Urinary continence Child Urodynamics Urinary dysfunction Clean intermittent catheterization Anticholinergics Botulinum A toxin 

References

  1. 1.
    Zerah M, Kulkarni AV. Spinal cord malformations. Handb Clin Neurol. 2013;112:975–91.CrossRefPubMedGoogle Scholar
  2. 2.
    Welch K, Winston KR. Spina bifida. In: Myrianthopoulos NC, editor. Handbook of clinical neurology, Vol. 6 (50): malformations. Amsterdam: Elsevier Science Publishers BV; 1987. p. 477–508.Google Scholar
  3. 3.
    Kroovand RL, Bell W, Hart LJ, Benfield KY. The effect of back closure on detrusor function in neonates with myelomeningocele. J Urol. 1990;144:423–5.CrossRefPubMedGoogle Scholar
  4. 4.
    James CCM, Lassman LP. Spinal dysraphism; spina bifida occulta. London: Butterworths; 1972.Google Scholar
  5. 5.
    Bourque PR, D’Alton JG, Russell NA, Gerridzen RG, Benoit BG. Congenital lumbosacral lipoma causing primary enuresis in an adult. CMAJ. 1986;135:1007–8.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Ritchey ML, Sinha A, DiPietro MA, Huang C, Flood H, Bloom DA. Significance of spina bifida occulta in children with diurnal enuresis. J Urol. 1994;152:815–8.CrossRefPubMedGoogle Scholar
  7. 7.
    de Jong TP, Chrzan R, Klijn AJ, Dik P. Treatment of the neurogenic bladder in spina bifida. Pediatr Nephrol. 2008;23:889–96.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Fidas A, MacDonald HL, Elton RA, McInnes A, Chisholm GD. Neurological defects of the voiding reflex arcs in chronic urinary retention and their relation to spina bifida occulta. Br J Urol. 1989;63:16–20.CrossRefPubMedGoogle Scholar
  9. 9.
    Snow-Lisy DC, Yerkes EB, Cheng EY. Update on urological management of spina bifida from prenatal diagnosis to adulthood. J Urol. 2015;194:288–96.CrossRefPubMedGoogle Scholar
  10. 10.
    Kondo A, Kamihira O, Ozawa H. Neural tube defects: prevalence, etiology and prevention. Int J Urol. 2009;16:49–57.CrossRefPubMedGoogle Scholar
  11. 11.
    Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology. 2000;42:471–91.CrossRefPubMedGoogle Scholar
  12. 12.
    Fowler CJ, Griffiths D, de Groat WC. The neural control of micturition. Nat Rev Neurosci. 2008;9:453–66.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Sakakibara R. Lower urinary tract dysfunction in patients with brain lesions. Handb Clin Neurol. 2015;130:269–87.CrossRefPubMedGoogle Scholar
  14. 14.
    Szymanski KM, Misseri R, Whittam B, Raposo SM, King SJ, Kaefer M, Rink RC, Cain MP. QUAlity of Life Assessment in Spina bifida for Adults (QUALASA): development and international validation of a novel healthrelated quality of life instrument. Qual Life Res. 2015;24:2355–64.CrossRefPubMedGoogle Scholar
  15. 15.
    Veenboer PW, Ruud Bosch JL, de Kort LM. Assessment of bladder and kidney functioning in adult spina bifida patients by Dutch urologists: a survey. Neurourol Urodyn. 2014a;33:289–95.CrossRefPubMedGoogle Scholar
  16. 16.
    Veenboer PW, Ruud Bosch JL, Rosier PFWM, Dik P, van Asbeck FWA, de Jong TPVM, de Kort LMO. 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. 2014b;192:477–82.CrossRefPubMedGoogle Scholar
  17. 17.
    Sakakibara R, Hattori T, Uchiyama T, Kamura K, Yamanishi T. Uro-neurological assessment in patient with spinal bifida occulta and cystica. Neurourol Urodynam. 2003;22:328–34.CrossRefGoogle Scholar
  18. 18.
    Drzewiecki BA, Bauer SB. Urodynamic testing in children: indications, technique, interpretation and significance. J Urol. 2011;186:1190–7.CrossRefPubMedGoogle Scholar
  19. 19.
    Glazier DB, Murphy DP, Fleisher MH, Cummings KB, Barone JG. Evaluation of the utility of video-urodynamics in children with urinary tract infection and voiding dysfunction. Br J Urol. 1997;80:806–8.CrossRefPubMedGoogle Scholar
  20. 20.
    Jansson UB, Hanson M, Sillén U, Hellström AL. Voiding pattern and acquisition of bladder control from birth to age 6 years—a longitudinal study. J Urol. 2005;174:289–93.CrossRefPubMedGoogle Scholar
  21. 21.
    Almodhen F, Capolicchio JP, Jednak R, El Sherbiny M. Postpubertal urodynamic and upper urinary tract changes in children with conservatively treated myelomeningocele. J Urol. 2007;178:1479–82.CrossRefPubMedGoogle Scholar
  22. 22.
    Torre M, Planche D, Louis-Borrione C, Sabiani F, Lena G, Guys JM. Value of electrophysiological assessment after surgical treatment of spinal dysraphism. J Urol. 2002;168:1759–62.CrossRefPubMedGoogle Scholar
  23. 23.
    Wyndaele JJ, de Sy WA. Correlation between the findings of a clinical neurological examination and the urodynamic dysfunction in children with myelodysplasia. J Urol. 1985;133:638–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Blaivas JG. The neurophysiology of micturition; a clinical study of 550 patients. J Urol. 1982;127:958–63.CrossRefPubMedGoogle Scholar
  25. 25.
    Lapides J, Friend CR, Ajemian EP, Reus WS. Denervation supersensibility as a test for neurogenic bladder. Surg Gyn Obst. 1962;114:241–4.Google Scholar
  26. 26.
    Giddens JL, Radomski SB, Hirshberg ED, Hassouna M, Fehlings M. Urodynamic findings in adults with the tethered cord syndrome. J Urol. 1999;161:1249–54.CrossRefPubMedGoogle Scholar
  27. 27.
    Sutherland RS, Mevorach RA, Baskin LS, Kogan BA. Spinal dysraphism in children; an overview and an approach to prevent complications. Urology. 1995;46:294–304.CrossRefPubMedGoogle Scholar
  28. 28.
    van Gool JD, Dik P, de Jong TP. Bladder-sphincter dysfunction in myelomeningocele. Eur J Pediatr. 2001;160:414–20.CrossRefPubMedGoogle Scholar
  29. 29.
    Sakakibara R, Hattori T, Tojo M, Yamanishi T, Yasuda K, Hirayama K. The location of the paths subserving micturition; studies in patients with cervical myelopathy. J Autonom Nerv Syst. 1995;55:165–8.CrossRefGoogle Scholar
  30. 30.
    Wyndaele JJ. Correlation between clinical neurological data and urodynamic function in spinal cord injured patients. Spinal Cord. 1997;35:213–6.CrossRefPubMedGoogle Scholar
  31. 31.
    Nathan PW, Smith MC. The centrifugal pathway for micturition within the spinal cord. J Neurol Neurosurg Psychiatry. 1958;21:177–89.CrossRefPubMedPubMedCentralGoogle Scholar
  32. 32.
    Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J. 2005;5:564–76.CrossRefPubMedGoogle Scholar
  33. 33.
    Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: a 30-year experience. Pediatrics. 2003;112:641–7.CrossRefPubMedGoogle Scholar
  34. 34.
    Assaad A, Mansy A, Kotb M, Hafez M. Spinal dysraphism: experience with 250 cases operated upon. Childs Nerv Syst. 1989;5:324–9.CrossRefPubMedGoogle Scholar
  35. 35.
    Gibson PJ, Britton J, Hall DM, Hill CR. Lumbosacral skin markers and identification of occult spinal dysraphism in neonates. Acta Paediatr. 1995;84:208–9.CrossRefPubMedGoogle Scholar
  36. 36.
    Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol. 2004;140:1109–15.CrossRefPubMedGoogle Scholar
  37. 37.
    Kanev PM, Lemire RJ, Loeser JD, Berger MS. Management and long-term follow-up review of children with lipomyelomeningocele, 1952–1987. J Neurosurg. 1990;73:48–52.CrossRefPubMedGoogle Scholar
  38. 38.
    McAtee-Smith J, Hebert AA, Rapini RP, Goldberg NS. Skin lesions of the spinal axis and spinal dysraphism. Fifteen cases and a review of the literature. Arch Pediatr Adolesc Med. 1994;148:740–8.CrossRefPubMedGoogle Scholar
  39. 39.
    Pierre-Kahn A, Lacombe J, Pichon J, Giudicelli Y, Renier D, Sainte-Rose C, et al. Intraspinal lipomas with spina bifida. Prognosis and treatment in 73 cases. J Neurosurg. 1986;65:756–61.CrossRefPubMedGoogle Scholar
  40. 40.
    Mak V, Radomski SB. Value of magnetic resonance imaging of the lumbosacral spinal cord in patients with voiding dysfunction. J Urol. 1996;156:1421–3.CrossRefPubMedGoogle Scholar
  41. 41.
    Salle JLP, Capolicchio G, Houle AM, Vernet O, Jednak R, O’Gorman AM, Montes JL, Farmer JP. Magnetic resonance imaging in children with voiding dysfunction; is it indicated? J Urol. 1998;160:1080–3.CrossRefGoogle Scholar
  42. 42.
    Sadiq S, Faiq SM, Idrees MK. Lumbosacral dysraphism as cause of neurogenic bladder: magnetic resonance imaging based study from SIUT Pakistan. J Pak Med Assoc. 2015;65:501–5.PubMedGoogle Scholar
  43. 43.
    Madden-Fuentes RJ, McNamara ER, Lloyd LC, Wiener JS, Routh JC, Seed PC, Ross SS. Variation in definitions of urinary tract infections in spina bifida patients: a systematic review. Pediatrics. 2013;132:132–9.CrossRefPubMedGoogle Scholar
  44. 44.
    Verpoorten C, Buyse GM. The neurogenic bladder: medical treatment. Pediatr Nephrol. 2008;23:717–25.CrossRefPubMedGoogle Scholar
  45. 45.
    Snodgrass WT, Adams R. Initial urologic management of myelomeningocele. Urol Clin N Am. 2004;31:427–34.CrossRefGoogle Scholar
  46. 46.
    Muller T, Arbeiter K, Aufricht C. Renal function in meningomyelocele: risk factors, chronic renal failure, renal replacement therapy and transplantation. Curr Opin Urol. 2002;12:479–84.CrossRefPubMedGoogle Scholar
  47. 47.
    Saito M, Kato K, Goto M, Kondo A. Lower urinary tract function in myelomeningocele. Nippon Hinyokigakkai Zasshi. 1990;81:75–81.Google Scholar
  48. 48.
    Baskin LS, Kogan BA, Benard F. Treatment of infants with neurogenic bladder dysfunction using aticholinergic drugs and intermittent catheterisation. Br J Urol. 1990;66:532–4.CrossRefPubMedGoogle Scholar
  49. 49.
    Edelstein RA, Bauer SB, Kelly MD, Darbey MM, Peters CA, Atala A, et al. The long-term urological response of neonates with myelodysplasia treated proactively with intermittent catheterization and anticholinergic therapy. J Urol. 1995;154(4):1500–4.CrossRefPubMedGoogle Scholar
  50. 50.
    Kaefer M, Pabby A, Kelly M, Darbey M, Bauer SB. Improved bladder function after prophylactic treatment of the high risk neurogenic bladder in newborns with myelomeningocele. J Urol. 1999;162:1068–71.CrossRefPubMedGoogle Scholar
  51. 51.
    Wu HY, Baskin LS, Kogan BA. Neurogenic bladder dysfunction due to myelomeningocele: neonatal versus childhood treatment. J Urol. 1997;157:2295–7.CrossRefPubMedGoogle Scholar
  52. 52.
    Churchill BM, Abramson RP, Wahl EF. Dysfunction of the lower urinary and distal gastrointestinal tracts in pediatric patients with known spinal cord problems. Pediatr Clin N Am. 2001;48:1587–630.CrossRefGoogle Scholar
  53. 53.
    Ferrara CM, D’aleo E, Tarquini S, Salvatore S, Salvaggio E. Side-effects of oral or intravesical oxybutynin chloride in children with spina bifida. BJU Int. 2001;87:674–8.CrossRefPubMedGoogle Scholar
  54. 54.
    Kessler TM, Lackner J, Kiss G, Rehder P, Madersbacher H. Early proactive management improves upper urinary tract function and reduces the need for surgery in patients with myelomeningocele. Neurourol Urodyn. 2006;25:758–62.CrossRefPubMedGoogle Scholar
  55. 55.
    Kessler TM, Lackner J, Kiss G, Rehder P, Madersbacher H. Predictive value of initial urodynamic pattern on urinary continence in patients with myelomeningocele. Neurourol Urodyn. 2006;25:361–7.CrossRefPubMedGoogle Scholar
  56. 56.
    Buyse G, Verpoorten C, Vereecken R, Casaer P. Intravesical application of a stable oxybutynin solution improves therapeutic compliance and acceptance in children with neurogenic bladder dysfunction. J Urol. 1998;160:1084–7.CrossRefPubMedGoogle Scholar
  57. 57.
    Tarcan T, Bauer S, Olmedo E, Khoshbin S, Kelly M, Darbey M. Long-term followup of newborns with myelodysplasia and normal urodynamic findings: is followup necessary? J Urol. 2001;165:564–7.CrossRefPubMedGoogle Scholar
  58. 58.
    Hascoet J, Manunta A, Brochard C, Arnaud A, Damphousse M, Menard H, Kerdraon J, Journel H, Bonan I, Odent S, Fremond B, Siproudhis L, Gamé X, Peyronnet B, French Referral Network of Spina Bifida. Outcomes of intra-detrusor injections of botulinum toxin in patients with spina bifida: a systematic review. J Neurosurg Pediatr. 2016;18:150–63.CrossRefGoogle Scholar
  59. 59.
    Kuo YC, Kuo HC. Botulinum toxin injection for lower urinary tract dysfunction. Int J Urol. 2013;20(1):40–55.Google Scholar
  60. 60.
    González R, Schimke CM. Strategies in urological reconstruction in myelomeningocele. Curr Opin Urol. 2002;12:485–90.CrossRefPubMedGoogle Scholar
  61. 61.
    Westney OL. The neurogenic bladder and incontinent urinary diversion. Urol Clin North Am. 2010;37:581–92.CrossRefPubMedGoogle Scholar
  62. 62.
    Wiener JS, Antonelli J, Shea AM, Curtis LH, Schulman KA, Krupski TL, et al. Bladder augmentation versus urinary diversion in patients with spina bifida in the United States. J Urol. 2011;186:161–5.CrossRefPubMedGoogle Scholar
  63. 63.
    Ouyang L, Grosse SD, Armour BS, Waitzman NJ. Health care expenditures of children and adults with spina bifida in a privately insured U.S. population. Birth Defects Res A Clin Mol Teratol. 2007;79:552–8.CrossRefPubMedGoogle Scholar
  64. 64.
    Holzbeierlein J, Pope JC IV, Adams MC, Bruner J, Tulipan N, Brock JW 3rd. The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol. 2000;164:1336–9.CrossRefPubMedGoogle Scholar
  65. 65.
    Clayton DB, Tanaka ST, Trusler L, Thomas JC, Pope JC IV, Adams MC, et al. Long-term urological impact of fetal myelomeningocele closure. J Urol. 2011;186(4 Suppl):1581–5.CrossRefPubMedGoogle Scholar
  66. 66.
    Carr MC. Fetal myelomeningocele repair: urologic aspects. Curr Opin Urol. 2007;17:257–62.CrossRefPubMedGoogle Scholar
  67. 67.
    Tseng JH, Kuo MF, Tu YK, Tseng MY. Outcome of untethering for symptomatic spina bifida occulta with lumbosacral spinal cord tethering in 31 patients: analysis of preoperative prognostic factors. Spine J. 2008;8:630–8.CrossRefPubMedGoogle Scholar
  68. 68.
    Balkan E, Kiliç N, Avşar I, Boyaci S, Aksoy K, Doğruyol H. Urodynamic findings in the tethered spinal cord: the effect of tethered cord division on lower urinary tract functions. Eur J Pediatr Surg. 2001;11:116–9.CrossRefPubMedGoogle Scholar
  69. 69.
    Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 2001;34:114–20.CrossRefPubMedGoogle Scholar
  70. 70.
    Dias MS. Neurosurgical management of myelomeningocele (spina bifida). Pediatr Rev. 2005;26:50–60.CrossRefPubMedGoogle Scholar
  71. 71.
    Herman JM, McLone DG, Storrs BB, Dauser RC. Analysis of 153 patients with myelomeningocele or spinal lipoma reoperated upon for a tethered cord. Presentation, management and outcome. Pediatr Neurosurg. 1993;19:243–9.CrossRefPubMedGoogle Scholar
  72. 72.
    Kang JK, Lee KS, Jeun SS, Lee IW, Kim MC. Role of surgery for maintaining urological function and prevention of retethering in the treatment of lipomeningomyelocele: experience recorded in 75 lipomeningomyelocele patients. Childs Nerv Syst. 2003;19:23–9.PubMedGoogle Scholar
  73. 73.
    Morimoto K, Takemoto O, Wakayama A. Spinal lipomas in children—surgical management and long-term follow-up. Pediatr Neurosurg. 2005;41:84–7.CrossRefPubMedGoogle Scholar
  74. 74.
    Shurtleff DB, Duguay S, Duguay G, Moskowitz D, Weinberger E, Roberts T, et al. Epidemiology of tethered cord with meningomyelocele. Eur J Pediatr Surg. 1997;7(Suppl 1):7–11.CrossRefPubMedGoogle Scholar
  75. 75.
    Tamaki N, Shirataki K, Kojima N, Shouse Y, Matsumoto S. Tethered cord syndrome of delayed onset following repair of myelomeningocele. J Neurosurg. 1988;69:393–8.CrossRefPubMedGoogle Scholar
  76. 76.
    Keating MA, Rink RC, Bauer SB, Krarup C, Dyro FM, Winston KR, Shillito J, Fischer EG, Retik AB. Neurological implications of the changing approach in management of occult spinal lesions. J Urol. 1988;140:1299–301.CrossRefPubMedGoogle Scholar
  77. 77.
    Khoury AE, Hendrik EB, McLorie GA, Kulkarni A, Churchill BM. Occult spinal dysraphism; clinical and urodynamic outcome after diversion of the filum terminale. J Urol. 1990;144:426–9.CrossRefPubMedGoogle Scholar
  78. 78.
    Satar N, Bauer SB, Shefner J, Kelly MD, Darbey MM. The effects of delayed diagnosis and treatment in patients with an occult spinal dysraphism. J Urol. 1995;154:754–8.CrossRefPubMedGoogle Scholar
  79. 79.
    Akay KM, Gonul E, Ocal E, Timurkaynak E. The initial treatment of meningocele and myelomeningocele lesions in adulthood: experiences with seven patients. Neurosurg Rev. 2003;26:162–7.CrossRefPubMedGoogle Scholar
  80. 80.
    Kumar R, Singhal Gupta NM, Kapoor R, Mahapatra AK. Evaluation of clinico-urodynamic outcome of bladder dysfunction after surgery in children with spinal dysraphism—a prospective study. Acta Neurochir. 2008;150:129–37.CrossRefPubMedGoogle Scholar
  81. 81.
    Wu HY, Kogan BA, Baskin LS, Edwards MS. Long-term benefits of early neurosurgery for lipomyelomeningocele. J Urol. 1998;160:511–4.CrossRefPubMedGoogle Scholar
  82. 82.
    Macejko AM, Cheng EY, Yerkes EB, Meyer T, Bowman RM, Kaplan WE. Clinical urological outcomes following primary tethered cord release in children younger than 3 years. J Urol. 2007;178:1738–43.CrossRefPubMedGoogle Scholar
  83. 83.
    Metcalfe PD, Luerssen TG, King SJ, Kaefer M, Meldrum KK, Cain MP, et al. Treatment of the occult tethered spinal cord for neuropathic bladder: results of sectioning the filum terminale. J Urol. 2006;176:1826–30.CrossRefPubMedGoogle Scholar
  84. 84.
    Tarcan T, Önol FF, Ilker Y, Alpay H, Simsek F, Özek M. The timing of primary neurosurgical repair significantly affects neurogenic bladder prognosis in children with myelomeningocele. J Urol. 2006;176:1161–5.CrossRefPubMedGoogle Scholar
  85. 85.
    Tarcan T, Onol FF, Ilker Y, Simsek F, Ozek M. Does surgical release of secondary spinal cord tethering improve the prognosis of neurogenic bladder in children with myelomeningocele? J Urol. 2006;176:1601–6. (Erratum in J Urol 176 (6 Pt 1):2749, 2006)CrossRefPubMedGoogle Scholar
  86. 86.
    Abrahamsson K, Olsson I, Sillén U. Urodynamic findings in children with myelomeningocele after untethering of the spinal cord. J Urol. 2007;177:331–4.CrossRefPubMedGoogle Scholar
  87. 87.
    Dushi G, Frey P, Ramseyer P, Vernet O, Meyrat BJ. Urodynamic score in children with lipomyelomeningocele: a prospective study. J Urol. 2011;186:655–9.CrossRefPubMedGoogle Scholar
  88. 88.
    Maher CO, Bauer SB, Goumnerova L, Proctor MR, Madsen JR, Scott RM. Urological outcome following multiple repeat spinal cord untethering operations. Clinical article. J Neurosurg Pediatr. 2009;4:275–9.CrossRefPubMedGoogle Scholar

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© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurologyInternal Medicine, Sakura Medical Center, Toho UniversitySakuraJapan

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