Pediatric Surgery pp 1213-1235 | Cite as

Spina Bifida and Encephalocele

  • Jonathan R. EllenbogenEmail author
  • Michael D. Jenkinson
  • Conor L. Mallucci
Reference work entry


This chapter aims to explain the range of congenital anomies that are encompassed by the term neural tube defects (NTD). Both cranial and spinal abnormalities occur which arise during embryological development due to defects in morphogenesis of the neural tube. They range from anencephaly to spina bifida occulta. The commonest congenital nervous system defect encountered remains spina bifida despite the overall incidence of neural tube defects declining. The chapter explains the classification, pathogenesis, assessment, management, treatment, and prognosis related to these conditions.


Hydrocephalus Cerebrospinal fluid (CSF) Ultrasound Computed tomography (CT) Magnetic resonance imaging (MRI) Shunt Neural tube defects (NTD) Spina bifida Anencephaly Myelomeningocele Embryology Encephalocele Meningocele Serum alphafetoprotein Counseling In-utero surgery Hydrocephalus Arnold–Chiari type II malformation Craniorachischisis Dysraphism 


  1. Adzick NS. Fetal surgery for spina bifida: past, present, future. Semin Pediatr Surg. 2013;22(1):10–7.PubMedPubMedCentralCrossRefGoogle Scholar
  2. Adzick NS, Thom EA, Spong CY, Brock JW, Burrows PK, Johnson MP, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993–1004.PubMedPubMedCentralCrossRefGoogle Scholar
  3. Aitken DA, Morrison NM, Ferguson-Smith MA. Predictive value of amniotic acetylcholinesterase analysis in the diagnosis of fetal abnormality in 3700 pregnancies. Prenat Diagn. 1984;4(5):329–40.PubMedCrossRefGoogle Scholar
  4. Van Allen MI. Multisite neural tube closure in humans. Birth Defects Orig Artic Ser. 1996;30(1):203–25.PubMedGoogle Scholar
  5. Angerpointner TA, Pockrandt L, Schroer K. Course of pregnancy, family history and genetics in children with spina bifida. Z Kinderchir. 1990;45(2):72–7.PubMedGoogle Scholar
  6. Asher M, Olson J. Factors affecting the ambulatory status of patients with spina bifida cystica. J Bone Joint Surg Am. 1983;65(3):350–6.PubMedCrossRefGoogle Scholar
  7. Atta CA, Fiest KM, Frolkis AD, et al. Global birth prevalence of spina bifida by folic acid fortification status: a systematic review and meta-analysis. Am J Public Health. 2016;106(1):e24–34.PubMedPubMedCentralCrossRefGoogle Scholar
  8. Barf HA, Verhoef M, Post MW, Jennekens-Schinkel A, Gooskens RH, Mullaart RA, et al. Educational career and predictors of type of education in young adults with spina bifida. Int J Rehabil Res. 2004;27(1):45–52.PubMedCrossRefGoogle Scholar
  9. Bayer C. Zur technik der operation der spina bifida and encephalocoele. Prag Med Wochenschr. 1892;17:317.Google Scholar
  10. Bentley JF, Smith JR. Developmental posterior enteric remnants and spinal malformations: the split notochord syndrome. Arch Dis Child. 1960;35:76–86.PubMedPubMedCentralCrossRefGoogle Scholar
  11. Bhide P, Sagoo GS, Moorthie S, et al. Systematic review of birth prevalence of neural tube defects in India. Birth Defects Res A Clin Mol Teratol. 2013;97(7):437–43.PubMedCrossRefGoogle Scholar
  12. Boone D, Parsons D, Lachmann SM, Sherwood T. Spina bifida occulta: lesion or anomaly? Clin Radiol. 1985;36(2):159–61.PubMedCrossRefGoogle Scholar
  13. Boulet SL, Yang Q, Mai C, Kirby RS, Collins JS, Robbins JM, et al. Trends in the postfortification prevalence of spina bifida and anencephaly in the United States. Birth Defects Res A Clin Mol Teratol. 2008;82(7):527–32.PubMedCrossRefGoogle Scholar
  14. Bower C, D’Antoine H, Stanley FJ. Neural tube defects in Australia: trends in encephaloceles and other neural tube defects before and after promotion of folic acid supplementation and voluntary food fortification. Birth Defects Res A Clin Mol Teratol. 2009;85(4):269–73.PubMedCrossRefGoogle Scholar
  15. Boyd PA, Devigan C, Khoshnood B, Loane M, Garne E, Dolk H. Survey of prenatal screening policies in Europe for structural malformations and chromosome anomalies, and their impact on detection and termination rates for neural tube defects and Down’s syndrome. BJOG. 2008;115(6):689–96.PubMedPubMedCentralCrossRefGoogle Scholar
  16. Brown MS, Sheridan-Pereira M. Outlook for the child with a cephalocele. Pediatrics. 1992;90(6):914–9.PubMedGoogle Scholar
  17. Bruinings AL, van den Berg-Emons HJ, Buffart LM, van der Heijden-Maessen HC, Roebroeck ME, Stam HJ. Energy cost and physical strain of daily activities in adolescents and young adults with myelomeningocele. Dev Med Child Neurol. 2007;49(9):672–7.PubMedCrossRefGoogle Scholar
  18. Bui CJ, Tubbs RS, Shannon CN, Acakpo-Satchivi L, Wellons JC, Blount JP, et al. Institutional experience with cranial vault encephaloceles. J Neurosurg. 2007;107(1 Suppl):22–5.PubMedGoogle Scholar
  19. Caldarelli M, Di Rocco C, La Marca F. Shunt complications in the first postoperative year in children with myelomeningocoele. Childs Nerv Syst. 1996;12(12):748–54.PubMedCrossRefGoogle Scholar
  20. Cameron M, Moran P. Prenatal screening and diagnosis of neural tube defects. Prenat Diagn. 2009;29(4):402–11.PubMedCrossRefGoogle Scholar
  21. Carter CO. Clues to the aetiology of neural tube malformations. Dev Med Child Neurol. 1974;16(6 Suppl 32):3–15.PubMedGoogle Scholar
  22. Centers for Disease Control (CDC). MMWR Morb Mortal Wkly Rep. 1983;32(33):438–9. Valproate: a new cause of birth defects–report from Italy and follow-up from France.Google Scholar
  23. Chadduck WM, Reding DL. Experience with simultaneous ventriculo-peritoneal shunt placement and myelomeningocele repair. J Pediatr Surg. 1988;23(10):913–6.PubMedCrossRefGoogle Scholar
  24. Charney EB. Parental attitudes toward management of newborns with myelomeningocele. Dev Med Child Neurol. 1990;32(1):14–9.PubMedCrossRefGoogle Scholar
  25. Charney EB, Weller SC, Sutton LN, Bruce DA, Schut LB. Management of the newborn with myelomeningocele: time for a decision-making process. Pediatrics. 1985;75(1):58–64.PubMedGoogle Scholar
  26. Choux M, Di Rocco C, Hockley AD, Walker ML, editors. Pediatric neurosurgery. 1st ed. London: Churchill Livingstone; 1999. p. 875.Google Scholar
  27. Copp AJ, Stanier P, De GN. Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol. 2013;4422(13):1–12.Google Scholar
  28. Copp AJ, Adzick NS, Chitty LS, et al. Spina bifida. Nat Rev Dis Primers. 2015;1:15007.Google Scholar
  29. Date I, Yagyu Y, Asari S, Ohmoto T. Long-term outcome in surgically treated encephalocele. Surg Neurol. 1993;40(2):125–30.PubMedCrossRefGoogle Scholar
  30. Davis BE, Daley CM, Shurtleff DB, Duguay S, Seidel K, Loeser JD, et al. Long-term survival of individuals with myelomeningocele. Pediatr Neurosurg. 2005;41(4):186–91.PubMedCrossRefGoogle Scholar
  31. Dicianno BE, Karmarkar A, Houtrow A, et al. Factors associated with mobility outcomes in a National Spina Bifida Patient Registry. Am J Phys Med Rehabil. 2015;94(12):1015–25.PubMedPubMedCentralCrossRefGoogle Scholar
  32. Doran PA, Guthkelch AN. Studies in spina bifida cystica I: general surgery and reassessment of the problem. J Neurol Neurosurg Psychiatry. 1961;24:331–45.PubMedPubMedCentralCrossRefGoogle Scholar
  33. EUROCAT Working Group. Prevalence of neural tube defects in 20 regions of Europe and the impact of prenatal diagnosis, 1980–1986. J Epidemiol Community Health. 1991;45(1):52–8.CrossRefGoogle Scholar
  34. Fitzgerald RJ, Healy B. The spina bifida problem: a longer term review with special reference to the quality of survival. Ir Med J. 1974;67(21):565–7.PubMedGoogle Scholar
  35. Folate supplements prevent recurrence of neural tube defects. Nutr Rev. 1992;50:22–24.
  36. Frazier CH. Surgery of the spine and spinal cord. New York: Appleton & Co.; 1918.Google Scholar
  37. Fritsch MJ, Kienke S, Ankermann T, Padoin M, Mehdorn HM. Endoscopic third ventriculostomy in infants. J Neurosurg. 2005;56(6):1271–8.CrossRefGoogle Scholar
  38. Gardner WJ. Rupture of the neural tube. Arch Neurol. 1961;4:1–7.PubMedCrossRefGoogle Scholar
  39. Gorlin RJ, Cohen MM, Hennekam RCM. Syndromes of the head and neck. New York: Oxford University Press; 2001.Google Scholar
  40. Greenberg M. Handbook of neurosurgery. 7th ed. New York: Thieme; 2010. p. 1352.Google Scholar
  41. Greig JD, Young DG, Azmy AF. Follow-up of spina bifida children with and without upper renal tract changes at birth. Eur J Pediatr Surg. 1991;1(1):5–9.PubMedCrossRefGoogle Scholar
  42. Group MRCVSR. Prevention of neural tube defects: results of the Medical Research Council vitamin study MRC vitamin study research group. Lancet. 1991;338(8760):131–7.CrossRefGoogle Scholar
  43. Hunt GM. Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol. 1990;32(2):108–18.PubMedCrossRefGoogle Scholar
  44. Hunt GM, Oakeshott P. Outcome in people with open spina bifida at age 35: prospective community based cohort study. BMJ. 2003;326(7403):1365–6.PubMedPubMedCentralCrossRefGoogle Scholar
  45. Isada NB, Qureshi F, Jacques SM, Holzgreve W, Tout MJ, Johnson MP, et al. Meroanencephaly: pathology and prenatal diagnosis. Fetal Diagn Ther. 1993;8(6):423–8.PubMedCrossRefGoogle Scholar
  46. Janerich DT. Influenza and neural-tube defects. Lancet. 1971;2(7723):551–2.PubMedCrossRefGoogle Scholar
  47. Jenkinson MD, Campbell S, Hayhurst C, Clark S, Kandasamy J, Lee MK, et al. Cognitive and functional outcome in spina bifida-Chiari II malformation. Childs Nerv Syst. 2011;27(6):967–74.PubMedCrossRefGoogle Scholar
  48. Jenkinson MD, Hayhurst C, Al-Jumaily M, Kandasamy J, Clark S, Mallucci CL. The role of endoscopic third ventriculostomy in adult patients with hydrocephalus. J Neurosurg. 2009;110(5):861–6.PubMedCrossRefGoogle Scholar
  49. Jenkinson MD, Hayhurst C, Clark S, Campbell S, Murphy P, Mallucci CL. Long-term functional and neuropsychological outcome in Chiari II malformation. Childs Nerv Syst. 2008;24(10):1270.Google Scholar
  50. Johnson MP, Bennett KA, Rand L, et al. Management of myelomeningocele study investigators. The management of myelomeningocele study: obstetrical outcomes and risk factors for obstetrical complications following prenatal surgery. Am J Obstet Gynecol. 2016;215(6):778.e1–9.CrossRefGoogle Scholar
  51. Joyeux L, Danzer E, Flake AW, et al. Fetal surgery for spina bifida aperta. Arch Dis Child Fetal Neonatal Ed. 2018;103(6):F589–F595.PubMedCrossRefGoogle Scholar
  52. Kasabian NG, Bauer SB, Dyro FM, Colodny AH, Mandell J, Retik AB. The prophylactic value of clean intermittent catheterization and anticholinergic medication in newborns and infants with myelodysplasia at risk of developing urinary tract deterioration. Am J Dis Child. 1992;146(7):840–3.PubMedGoogle Scholar
  53. Kondo A, Kamihira O, Ozawa H. Neural tube defects: prevalence, etiology and prevention. Int J Urol. 2009;16(1):49–57.PubMedCrossRefGoogle Scholar
  54. Kooper AJ, de Bruijn D, van Ravenwaaij-Arts CM, Faas BH, Creemers JW, Thomas CM, et al. Fetal anomaly scan potentially will replace routine AFAFP assays for the detection of neural tube defects. Prenat Diagn. 2007;27(1):29–33.PubMedCrossRefGoogle Scholar
  55. Larsen W. Human Embryology. 1st ed. New York: Churchill Livingstone; 1993. p. 479.Google Scholar
  56. Laurence KM, James N, Miller MH, Tennant GB, Campbell H. Double-blind randomised controlled trial of folate treatment before conception to prevent recurrence of neural-tube defects. Br Med J (Clin Res Ed). 1981;282(6275):1509–11.CrossRefGoogle Scholar
  57. Layde PM, Edmonds LD, Erickson JD. Maternal fever and neural tube defects. Teratology. 1980;21(1):105–8.PubMedCrossRefGoogle Scholar
  58. Leck I. The etiology of human malformations: insights from epidemiology. Teratology. 1972;5(3):303–14.PubMedCrossRefGoogle Scholar
  59. Leck I. The geographical distribution of neural tube defects and oral clefts. Br Med Bull. 1984;40(4):390–5.PubMedCrossRefGoogle Scholar
  60. Lemire RJ, Beckwith JB, Warkny J. Anencephaly. New York: Raven Press; 1978.Google Scholar
  61. Li Z, Ren A, Zhang L, et al. A population-based case-control study of risk factors for neural tube defects in four high-prevalence areas of Shanxi province, China. Paediatr Perinat Epidemiol. 2006;20(1):45–53.PubMedCrossRefGoogle Scholar
  62. Lindhout D, Omtzigt JG, Cornel MC. Spectrum of neural-tube defects in 34 infants prenatally exposed to antiepileptic drugs. Neurology. 1992;42(4 Suppl 5):111–8.PubMedGoogle Scholar
  63. Van Lith JMM, Johnson MP, Wilson RD. Current controversies in prenatal diagnosis 3: fetal surgery after MOMS: is fetal therapy better than neonatal? Prenat Diagn. 2013;33(1):13–6.PubMedCrossRefGoogle Scholar
  64. Lorber J. Results of treatment of myelomeningocele. An analysis of 524 unselected cases, with special reference to possible selection for treatment. Dev Med Child Neurol. 1971;13(3):279–303.PubMedCrossRefGoogle Scholar
  65. Lorber J, Salfield SA. Results of selective treatment of spina bifida cystica. Arch Dis Child. 1981;56(11):822–30.PubMedPubMedCentralCrossRefGoogle Scholar
  66. Luthy DA, Wardinsky T, Shurtleff DB, Hollenbach KA, Hickok DE, Nyberg DA, et al. Cesarean section before the onset of labor and subsequent motor function in infants with meningomyelocele diagnosed antenatally. N Engl J Med. 1991;324(10):662–6.PubMedCrossRefGoogle Scholar
  67. McCarthy GT. Treating children with spina bifida. BMJ. 1991;302(6768):65–6.PubMedPubMedCentralCrossRefGoogle Scholar
  68. McConnell JR, Holder JC, Menick JR, Alexander JE. The radiology of neural tube defects. Curr Probl Diagn Radiol. 1986;15(4):244–76.Google Scholar
  69. Milunsky A, Jick SS, Bruell CL, MacLaughlin DS, Tsung YK, Jick H, et al. Predictive values, relative risks, and overall benefits of high and low maternal serum alpha-fetoprotein screening in singleton pregnancies: new epidemiologic data. Am J Obstet Gynecol. 1989;161(2):291–7.PubMedCrossRefGoogle Scholar
  70. Milunsky A, Ulcickas M, Rothman KJ, Willett W, Jick SS, Jick H. Maternal heat exposure and neural tube defects. JAMA. 1992;268(7):882–5.PubMedCrossRefGoogle Scholar
  71. Moore K, Persaud T, Torchia M. The developing human. 9th ed. Saint Louis: Elsevier; 2011. p. 562.Google Scholar
  72. Morgagni JB. Je sedibus et causis morborum per indagatis. Naples: Typographia Simoniana; 1762. p. 1962.Google Scholar
  73. Morton J. Case of spina bifida cured by injection. Br Med J. 1872;1:364.PubMedCentralCrossRefPubMedGoogle Scholar
  74. O’Brien DF, Javadpour M, Collins DR, Spennato P, Mallucci CL. Endoscopic third ventriculostomy: an outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction. J Neurosurg. 2005;103(5 Suppl):393–400.Google Scholar
  75. Oakeshott P, Hunt GM. Long-term outcome in open spina bifida. Br J Gen Pract. 2003;53(493):632–6.PubMedPubMedCentralGoogle Scholar
  76. Oakeshott P, Hunt GM, Poulton A, Reid F. Open spina bifida: birth findings predict long-term outcome. Arch Dis Child. 2012;97(5):474–6.PubMedCrossRefGoogle Scholar
  77. Obladen M. Cats, frogs, and snakes: early concepts of neural tube defects. J Child Neurol. 2011;26(11):1452–61.PubMedCrossRefGoogle Scholar
  78. Olney RS, Mulinare J. Trends in neural tube defect prevalence, folic acid fortification, and vitamin supplement use. Semin Perinatol. 2002;26(4):277–85.PubMedCrossRefGoogle Scholar
  79. Pang D. Surgical complications of open spinal dysraphism. Neurosurg Clin N Am. 1995;6(2):243–57.PubMedCrossRefGoogle Scholar
  80. Parent AD, McMillan T. Contemporaneous shunting with repair of myelomeningocele. Pediatr Neurosurg. 1995;22(3):132–5. discussion 136PubMedCrossRefGoogle Scholar
  81. Parker SE, Yazdy MM, Tinker SC, Mitchell AA, Werler MM. The impact of folic acid intake on the association among diabetes mellitus, obesity, and spina bifida. Am J Obstet Gynecol. 2013;209(3):239.e1–8.CrossRefGoogle Scholar
  82. Patten BM. Overgrowth of the neural tube in young human embryos. Anat Rec. 1952;113(4):381–93.PubMedCrossRefGoogle Scholar
  83. Pollack IF, Pang D, Albright AL, Krieger D. Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg. 1992;77(6):881–8.PubMedCrossRefGoogle Scholar
  84. Report of a committee of the society nominated to investigate spina bifida. Trans Chir Soc. London; 1882 p. 339.Google Scholar
  85. Rickwood AMK. Infective problems encountered in neonatal closure of the neural tube defects. Dev Med Child Neurol. 1976;18:164–5.Google Scholar
  86. Robards MF, Thomas GG, Rosenbloom L. Survival of infants with unoperated myeloceles. Br Med J. 1975;4(5987):12–3.PubMedPubMedCentralCrossRefGoogle Scholar
  87. Romero R, Mathisen JM, Ghidini A, Sirtori M, Hobbins JC. Accuracy of ultrasound in the prenatal diagnosis of spinal anomalies. Am J Perinatol. 1989;6(3):320–3.PubMedCrossRefGoogle Scholar
  88. Rosa FW. Spina bifida in infants of women treated with carbamazepine during pregnancy. N Engl J Med. 1991;324(10):674–7.PubMedCrossRefGoogle Scholar
  89. Rudy DC, Woodside JR. The incontinent myelodysplastic patient. Urol Clin North Am. 1991;18(2):295–308.PubMedGoogle Scholar
  90. Sadler TW. Embryology of neural tube development. Am J Med Genet C Semin Med Genet. 2005;135C(1):2–8.PubMedCrossRefGoogle Scholar
  91. Sakala EP, Andree I. Optimal route of delivery for meningomyelocele. Obstet Gynecol Surv. 1990;45(4):209–12.PubMedCrossRefGoogle Scholar
  92. Sandford MK, Kissling GE, Joubert PE. Neural tube defect etiology: new evidence concerning maternal hyperthermia, health and diet. Dev Med Child Neurol. 1992;34(8):661–75.PubMedCrossRefGoogle Scholar
  93. Seitzberg A, Lind M, Biering-Sorensen F. Ambulation in adults with myelomeningocele. Is it possible to predict the level of ambulation in early life? Childs Nerv Syst. 2008;24(2):231–7.PubMedCrossRefGoogle Scholar
  94. Sharrard WJ, Zachary RB, Lorber J. Survival and paralysis in open myelomeningocele with special reference to the time of repair of the spinal lesion. Dev Med Child Neurol. 1967;Suppl 13:35–50.Google Scholar
  95. Smithells RW, Chinn ER. Spina bifida in Liverpool. Dev Med Child Neurol. 1965;7:258–68.PubMedCrossRefGoogle Scholar
  96. Snow-Lisy DC, Yerkes EB, Cheng EY. Update on urological management of spina bifida from prenatal diagnosis to adulthood. J Urol. 2015;194(2):288–96.PubMedCrossRefGoogle Scholar
  97. Stein SC, Schut L. Hydrocephalus in myelomeningocele. Childs Brain. 1979;5(4):413–9.PubMedGoogle Scholar
  98. Stevenson KL. Chiari type II malformation: past, present, and future. Neurosurg Focus. 2004;16(2):E5.PubMedCrossRefGoogle Scholar
  99. Stevenson RE, Allen WP, Pai GS, Best R, Seaver LH, Dean J, et al. Decline in prevalence of neural tube defects in a high-risk region of the United States. Pediatrics. 2000;106(4):677–83.PubMedCrossRefGoogle Scholar
  100. Stoneking BJ, Brock JW, Pope JC, Adams MC. Early evolution of bladder emptying after myelomeningocele closure. Urology. 2001;58(5):767–71.PubMedCrossRefGoogle Scholar
  101. Surana RH, Quinn FM, Guiney EJ, Fitzgerald RJ. Are the selection criteria for the conservative management in spina bifida still applicable? Eur J Pediatr Surg. 1991;1(Suppl 1):35–7.PubMedCrossRefGoogle Scholar
  102. Sutton LN. Fetal surgery for neural tube defects. Best Pract Res Clin Obstet Gynaecol. 2008;22(1):175–88.PubMedCrossRefGoogle Scholar
  103. Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningoceles. J Neurosurg. 1972;36(2):201–11.PubMedCrossRefGoogle Scholar
  104. Takeuchi H. Prenatal ultrasound diagnosis of central nervous system anomalies. No To Hattatsu. 1991;23(2):183–8.PubMedGoogle Scholar
  105. Thompson DN. Postnatal management and outcome for neural tube defects including spina bifida and encephalocoeles. Prenat Diagn. 2009;29(4):412–9.PubMedCrossRefGoogle Scholar
  106. Tuli S, Drake J, Lamberti-Pasculli M. Long-term outcome of hydrocephalus management in myelomeningoceles. Childs Nerv Syst. 2003;19(5–6):286–91.PubMedCrossRefGoogle Scholar
  107. Tulp N. Observationes medicae. Amsterdam: Elsevier; 1672.Google Scholar
  108. Vachha B, Adams RC, Rollins NK. Limbic tract anomalies in pediatric myelomeningocele and Chiari II malformation: anatomic correlations with memory and learning: initial investigation. Radiology. 2006;240(1):194–202.PubMedCrossRefGoogle Scholar
  109. Valproate: a new cause of birth defects—report from Italy and follow-up from France. MMWR Morb Mortal Wkly Rep. 1983;32(33):438–9.Google Scholar
  110. Valproate, spina bifida, and birth defect registries. Lancet. 1988;332(8625):1404–5.Google Scholar
  111. Valtonen K, Karlsson A-K, Alaranta H, Viikari-Juntura E. Work participation among persons with traumatic spinal cord injury and meningomyelocele. J Rehabil Med. 2006;38(3):192–200.PubMedCrossRefGoogle Scholar
  112. Vandertop WP, Asai A, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, et al. Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg. 1992;77(4):541–4.PubMedCrossRefGoogle Scholar
  113. Vinck A, Maassen B, Mullaart R, Rotteveel J. Arnold-Chiari-II malformation and cognitive functioning in spina bifida. J Neurol Neurosurg Psychiatry. 2006;77(9):1083–6.PubMedPubMedCentralCrossRefGoogle Scholar
  114. Virchow R. Ein fall von hypertrichosis circumscripta mediana kornbiniert mit spina bifida. Ztschaz Ethnol. 1875;7:279.Google Scholar
  115. Wald NJ, Bower C. Folic acid, pernicious anaemia, and prevention of neural tube defects. Lancet. 1994;343(8893):307.PubMedCrossRefGoogle Scholar
  116. Wald NJ, Cuckle H, Brock JH, Peto R, Polani PE, Woodford FP. Maternal serum-alpha-fetoprotein measurement in antenatal screening for anencephaly and spina bifida in early pregnancy. Report of U.K. collaborative study on alpha-fetoprotein in relation to neural-tube defects. Lancet. 1977;1(8026):1323–32.PubMedGoogle Scholar
  117. De Wals P, Tairou F, Van Allen MI, Uh SH, Lowry RB, Sibbald B, et al. Reduction in neural-tube defects after folic acid fortification in Canada. N Engl J Med. 2007;357(2):135–42.PubMedCrossRefGoogle Scholar
  118. White-Van Mourik MC, Connor JM, Ferguson-Smith MA. Patient care before and after termination of pregnancy for neural tube defects. Prenat Diagn. 1990;10(8):497–505.PubMedCrossRefGoogle Scholar
  119. Willett WC. Folic acid and neural tube defect: can’t we come to closure? Am J Public Health. 1992;82(5):666–8.PubMedPubMedCentralCrossRefGoogle Scholar
  120. Wiswell TE, Tuttle DJ, Northam RS, Simonds GR. Major congenital neurologic malformations: a 17-year survey. Am J Dis Child. 1990;144(1):61–7.PubMedCrossRefGoogle Scholar
  121. Woodhouse CR. Myelomeningocele: neglected aspects. Pediatr Nephrol. 2008;23(8):1223–31.PubMedPubMedCentralCrossRefGoogle Scholar
  122. Yen IH, Khoury MJ, Erickson JD, James LM, Waters GD, Berry RJ. The changing epidemiology of neural tube defects. United States, 1968–1989. Am J Dis Child. 1992;146(7):857–61.PubMedCrossRefGoogle Scholar

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© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  • Jonathan R. Ellenbogen
    • 1
    • 2
    Email author
  • Michael D. Jenkinson
    • 2
  • Conor L. Mallucci
    • 1
  1. 1.Department of NeurosurgeryAlder Hey Children’s NHS Foundation TrustLiverpoolUK
  2. 2.Department of NeurosurgeryThe Walton Centre NHS Foundation TrustLiverpoolUK

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