Skip to main content

The Role of Fetal Neurosurgery in Spina Bifida

  • Chapter
The Spina Bifida

Abstract

Myelomeningocele (MMC) is a nonlethal form of neural tube defect (NTD) that results from failure of the neural tube to fuse during early embryogenesis. The lesion is characterized by protrusion of the meninges through a midline bony defect of the spine, a sac containing cerebrospinal fluid and dysplastic neural tissue not covered by skin. MMC represents an important congenital defect of the brain and spinal cord that affects approximately one in 2,000 live births and about 23% of pregnancies that end in elective abortion [1, 2]. MMC leads to lifelong and significant physical disabilities including paraplegia, hydrocephalus, bladder and fecal incontinence, sexual dysfunction, skeletal deformation and mental impairment [3]. The mortality rate, which can be as high as 47% on long-term followup, is principally attributable to the hindbrain herniation observed in Chiari malformation and to the renal failure observed in neurogenic bladder dysfunction [4].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Velie EM, Shaw GM (1996) Impact of prenatal diagnosis and elective termination on prevalence and risks estimates of neural tube defects in California. 1989–1991. Am J Epidemiol 144:473–479

    PubMed  CAS  Google Scholar 

  2. Copp AJ (1993) Neural tube defects. Trends Neurosci 16:381–383

    Article  PubMed  CAS  Google Scholar 

  3. Hunt GM, Poulton A (1995) Open spina bifida: a complete cohort reviewed 25 years after closure. Dev Med Child Neurol 37:19–29

    PubMed  CAS  Google Scholar 

  4. Center for Disease Control and Prevention (1992) Economic costs of birth defects and cerebral palsy — United States. MMWR 1995; 44:694–699

    Google Scholar 

  5. National Institute of Child Health and Human Development. Management of Myelomeningocele study. Available at http://www.spinabifidamoms.com

  6. Correia-Pinto J, Reis JL, Hutchins GM (2002) In utero meconuim exposure increases spinal cord necrosis in a rat model of myelomeningocele. J Pedriatr Surg 37:488–492

    Article  Google Scholar 

  7. Petzold A, Stiefel D, Copp AJ (2005) Amniotic fluid brain-specific proteins are biomarkers for spinal cord injury in experimental myelomeningocele. J Neurochem 95:594–598

    Article  PubMed  CAS  Google Scholar 

  8. Lemire RJ (1975) In: Normal and abnormal development of the human nervous system. Harper & Row, New York, pp 1–421

    Google Scholar 

  9. Hirose S, Meuli-Simmen C, Meuli M (2003) Fetal surgery for myelomeningocele: panacea or peril? World J Surg 27:87–94

    Article  PubMed  Google Scholar 

  10. Koremromp MJ, Van Good JD, Bruinesse HW et al (1986) Early fetal movements in myelomeningocele. Lancet 1:917–918

    Article  Google Scholar 

  11. Sival DA, Begger JH, Staal-Schreinemachers AL et al (1997) Perinatal motor behaviour and neurological outcome in spina bifida aperta. Erly Human Dev 50:27–37

    Article  CAS  Google Scholar 

  12. Luthy DA, Wardinsky T, Shurtleff DB et al (1991) Cesarean section before the onset of labor and subsequent motor function in infants with myelomeningocele diagnosed antenatally. N Engl J Med 324:662–68

    PubMed  CAS  Google Scholar 

  13. Jonhson MP, Sutton L, Rintoul N et al (2003) Fetal myelomeningocele repair: short term clinical outcomes. Am J Obstet Gynecol 189:482–487

    Article  Google Scholar 

  14. Kollias SS, Goldstein RB, Cogen PH et al (1992) Prenatally detected myelomeningocele: sonographic accuracy in estimation of the spinal level. Radiology 185:109–112

    PubMed  CAS  Google Scholar 

  15. Nicolaides KH, Campbell S, Gabbe, SG et al (1986) Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 2:72–74

    Article  PubMed  CAS  Google Scholar 

  16. Thiagarajah S, Henke J, Hogge A et al (1987) Early diagnosis of spina bifida: the value of cranial ultrasound markers. Obstet Gynecol 70:247–250

    Google Scholar 

  17. Nyberg DA, Mack LA, Hirsch J et al (1988) Abnormalities of fetal cranial contour in sonographic detection of spina bifida: evaluation of the “lemon” sign. Radiology 167:387–392

    PubMed  CAS  Google Scholar 

  18. Mangels KJ, Tulipan N, Tsao LY et al (2002). Fetal MRI in the evaluation of intrauterine myelomeningocele. Pediatr Neurosurg 32:124–131

    Article  Google Scholar 

  19. Aaronson OS, Hernnz-Schulman, Bruner JP et al (2003) Myelomeningocele: prenatal evaluation — comparison between transabdominal US and MR imaging. Radiology 227:839–843

    Article  PubMed  Google Scholar 

  20. Iskandar BJ, Tubbs S, Mapstone TB et al (1998) Death in shunted hydrocephalic children in 1900s. Pediatric Neurosurg 28:173–176

    Article  CAS  Google Scholar 

  21. Northrup H, Volcik KA (2000) Spina bifida and other neural tube defects. Curr Probl Pediatr 30:313–340

    Article  PubMed  CAS  Google Scholar 

  22. Bowman RM, McLone DG, Grant JA et al (2001) Spina bifida outcome. A 25-year prospective. Pediatr Neurosurg 34:114–120

    Article  PubMed  CAS  Google Scholar 

  23. Cochrane DD (1996) Prenatal spinal evaluation and functional outcome of patients born with myelo — menin gocele: information for improved prenatal counselling and outcome prediction. Fetal Diagn Ther 11:159–168

    Article  PubMed  CAS  Google Scholar 

  24. Skobejo-Wlodarska (2002) Treatment of neuropathic urinary and faecal incontinence. Eur J Pedriat Surg 12:318–321

    Article  Google Scholar 

  25. Cate IMP, Kennedy C, Stevenson J (2002) Disability and quality of life in spina bifida and hydrocephalus. Dev Med Child Neurol 44:317–322

    Article  PubMed  Google Scholar 

  26. Michejda M (1984) Intrauterine treatment of spina bifida: primate model. Z Kinderchir 39:259–261

    PubMed  CAS  Google Scholar 

  27. Meuli M, Meuli-Simmen C, Yingling C et al (1995) In utero surgery rescues neurological function at birth in sheep. Nat Med 1:342–347

    Article  PubMed  CAS  Google Scholar 

  28. Bruner JP, Richards WO, Tulipan N et al (1999) Endoscopic coverage of fetal myelomeningocele in utero. Am J Obstet Gynecol 180:153–158

    Article  PubMed  CAS  Google Scholar 

  29. Tulipan N, Bruner JP (1998) Myelomeningocele repair in utero: a report of three cases. Pediatr Neurosurg 28:177–180

    Article  PubMed  CAS  Google Scholar 

  30. Adzick NS, Sutton L, Crombleholme T et al (1998) Successful fetal surgery for spina bifida. Lancet 352:1675–1676

    Article  PubMed  CAS  Google Scholar 

  31. Sutton L, Adzik NS, Johnson MP (2003) Fetal surgery of myelomeningocele. Childs Nerv Syst 19:587–591

    Article  PubMed  Google Scholar 

  32. Tulipan N, Sutton L, Bruner JP et al (2003) The effect of intrauterine myelomeningocele repair on the incidence of shunt dependent hydrocephalus. Pediatr Neurosurg 38:27–33

    Article  PubMed  Google Scholar 

  33. Tulipan N (2003) Intrauterine myelomeningocele repair. Clin Perinatol 30:521–530

    Article  PubMed  Google Scholar 

  34. Bruner JP, Tulipan N, Paschall RL et al (1999) Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819–1825

    Article  PubMed  CAS  Google Scholar 

  35. Tubbs RS, Chamber MR, Smyth MD et al (2003) Late gestational intrauterine repair does not improve lower extremity function. Pediatr Neurosurg 38:128–132

    Article  PubMed  Google Scholar 

  36. Sutton L, Adzick N, Bilaniuk L et al (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282:1826–1831

    Article  PubMed  CAS  Google Scholar 

  37. Holzbeierlein J, Pope JI, Adams MC et al (2000) The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation. J Urol 164:1336–1339

    Article  PubMed  CAS  Google Scholar 

  38. Bruner JP, Tulipan N (2005) Intrauterine repair of spina bifida. Clin Obstet Gynecol 48:942–955

    Article  PubMed  Google Scholar 

  39. Werler MM, Louik C, Shapiro S et al (1996) Prepregnant weight in relation to risk of neural tube defects. JAMA 275:1089–1092

    Article  PubMed  CAS  Google Scholar 

  40. Almodin CG, Moron AF, Cavalheiro S (2006) The Almodin-Moron trocar for uterine entry during fetal surgery. Fetal Diagn Ther 21:414–417

    Article  PubMed  CAS  Google Scholar 

  41. Bruner JP, Boehm FH, Tulipan N (1999) The Tulipan-Bruner trocar for uterine entry during fetal surgery. Am J Obstet Gynecol 181:1188–1191

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer-Verlag Italia

About this chapter

Cite this chapter

Cavalheiro, S., Hisaba, W.J., Moron, A.F., Almodin, C.G. (2008). The Role of Fetal Neurosurgery in Spina Bifida. In: The Spina Bifida. Springer, Milano. https://doi.org/10.1007/978-88-470-0651-5_7

Download citation

  • DOI: https://doi.org/10.1007/978-88-470-0651-5_7

  • Publisher Name: Springer, Milano

  • Print ISBN: 978-88-470-0650-8

  • Online ISBN: 978-88-470-0651-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics