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Myelomeningocele: the management of the associated hydrocephalus

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Abstract

Background

The pathogenesis of the hydrocephalus associated with myelomeningocele (MMC) has been the subject of an extensive number of studies. The contemporary reduction of the incidence of the Chiari II malformation and of the associated active hydrocephalus after closure of the spinal defect in utero is in line with previous studies suggesting a prominent role of the posterior cranial fossa abnormalities, where even the increased venous pressure might be at least mostly a consequence of the constriction of the posterior cranial fossa structures. Pure absorptive abnormalities however coexist, the main ones documented to be abnormal cisternal spaces and peculiar cerebrospinal fluid chemical features.

Materials and methods

We reviewed the pertinent literature concerning the pathogenesis and management of the hydrocephalus associated to MMC. We also reviewed our personal experience in managing the hydrocephalus in such patients through an endoscopic third ventriculostomy.

Results and conclusions

The literature review demonstrated an overall reduction in more recent series of children with MMC needing to be treated for the associated hydrocephalus postnatally, questioning the role of the prenatal care of the disease in this context. Less severe conditions and a more conservative neurosurgical attitude have certainly contributed to the reduction of the reported active postnatal hydrocephalus rate. Long-term cognitive evaluation of the children with MMC that we managed with an endoscopic third ventriculocisternostomy (ETV) as primary as well as secondary procedure did not demonstrate significant differences in the outcome compared with non-complicated extrathecally shunted children, favouring ETV as a valuable option in this subset of patients.

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References

  1. Arslan M, Eseoglu M, Gudu BO, Demir I, Kozan A, Gokalp A, Sosuncu E, Kiymaz N (2011) Comparison of simultaneous shunting to delayed shunting in infants with myelomeningocele in terms of shunt infection rate. Turk Neurosurg 21(3):397–402

    PubMed  Google Scholar 

  2. Beems T, Grotenhuis JA (2002) Is the success rate of endoscopic third ventriculostomy age dependent? An analysis of the results of third ventriculostomy in young children. Childs Nerv Syst 18:605–608

    Article  PubMed  Google Scholar 

  3. Bell WO, Arbit E, Fraser RA (1987) One-stage meningomyelocele closure and ventriculoperitoneal shunt placement. Surg Neurol 27(3):233–236

    Article  PubMed  CAS  Google Scholar 

  4. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA (2001) Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34(3):114–120

    Article  PubMed  CAS  Google Scholar 

  5. Bruner JP, Tulipan N, Paschall RL, Boehm FH, Walsh WF, Silva SR, Hernanz-Schulman M, Lowe LH, Reed GW (1999) Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282(19):1819–1825

    Article  PubMed  CAS  Google Scholar 

  6. Bruner JP, Tulipan N, Reed G, Davis GH, Bennett K, Luker KS, Dabrowiak ME (2004) Intrauterine repair of spina bifida: preoperative predictors of shunt-dependent hydrocephalus. Am J Obstet Gynecol 190(5):1305–1312

    Article  PubMed  Google Scholar 

  7. Caldarelli M, Di Rocco C, La Marca F (1996) Shunt complications in the first postoperative year in children with meningomyelocele. Childs Nerv Syst 12(12):748–754

    Article  PubMed  CAS  Google Scholar 

  8. Castellino RA, Zatz LM, DeNardo GL (1969) Radioisotope ventriculography in the Arnold-Chiari malformation. Radiology 93(4):817–821

    PubMed  CAS  Google Scholar 

  9. Chadduck WM, Reding DL (1988) Experience with simultaneous ventriculo-peritoneal shunt placement and myelomeningocele repair. J Pediatr Surg 23(10):913–916

    Article  PubMed  CAS  Google Scholar 

  10. Chakraborty A, Crimmins D, Hayward R, Thompson D (2008) Toward reducing shunt placement rates in patients with myelomeningocele. J Neurosurg Pediatr 1:361–365

    Article  PubMed  Google Scholar 

  11. Cinalli G (2004) Endoscopic third ventriculostomy. In: Cinalli G, Maixner WJ, Sainte-Rose C (eds) Pediatric hydrocephalus. Springer, Milan, pp 361–388

    Google Scholar 

  12. Clemmensen D, Rasmussen MM, Mosdal C (2010) A retrospective study of infections after primary VP shunt placement in the newborn with myelomeningocele without prophylactic antibiotics. Childs Nerv Syst 26:1517–1521

    Article  PubMed  Google Scholar 

  13. Di Rocco C, Cinalli G, Massimi L, Spennato P, Cianciulli E, Tamburrini G (2006) Endoscopic third ventriculostomy in the treatment of hydrocephalus in pediatric patients. Adv Tech Stand Neurosurg 31:119–219

    Article  PubMed  Google Scholar 

  14. Di Rocco C, Rende M (1987) Neural tube defects. Some remarks on the possible role of glycosaminoglycans in the genesis of the dysraphic state, the anomaly in the configuration of the posterior cranial fossa, and hydrocephalus. Childs Nerv Syst 3(6):334–341

    Article  PubMed  Google Scholar 

  15. Dirks PB, Drake JM, Lamberti-Pasculli M, Rutka JT, Humphreys RP, McDonald P (2003) Falling ventriculoperitoneal shunt rates in myelomeningocele. Childs Nerv Syst 19:607

    Google Scholar 

  16. Elgamal EA (2012) Natural history of hydrocephalus in children with spinal open neural tube defect. Surg Neurol Int 3:112

    Article  PubMed  Google Scholar 

  17. Epstein NE, Rosenthal AD, Zito J, Osipoff M (1985) Shunt placement and myelomeningocele repair: simultaneous vs sequential shunting. Review of 12 cases. Childs Nerv Syst 1:145–147

    Article  PubMed  CAS  Google Scholar 

  18. Fritsch MJ, Mehdorn HM (2003) Indication and controversies for endoscopic third ventriculostomy in children. Childs Nerv Syst 19(9):706–707

    Google Scholar 

  19. Fukuhara T, Vorster SJ, Luciano MG (2000) Risk factors for failure of endoscopic third ventriculostomy for obstructive hydrocephalus. Neurosurgery 46:1100–1111

    Article  PubMed  CAS  Google Scholar 

  20. Genitori L, Peretta P, Mussa F, Giordano F (2004) Endoscopic third ventriculostomy in children: are age and etiology of hydrocephalus predictive factors influencing the outcome in primary and secondary treated patients? A series of 328 patients and 353 procedures. II CURAC Congress, Munich, October 8th

  21. Hubballah MY, Hoffman HJ (1987) Early repair of myelomeningocele and simultaneous insertion of ventriculoperitoneal shunt: technique and results. Neurosurgery 20(1):21–23

    Article  PubMed  CAS  Google Scholar 

  22. Hunt GM, Oakeshott P, Kerry S (1999) Link between the CSF shunt and achievement in adults with spina bifida. J Neurol Neurosurg Psychiatry 67(5):591–595

    Article  PubMed  CAS  Google Scholar 

  23. Iskandar BJ, Tubbs S, Mapstone TB, Grabb PA, Bartolucci AA, Oakes WJ (1998) Death in shunted hydrocephalic children in the 1990s. Pediatr Neurosurg 28:173–176

    Article  PubMed  CAS  Google Scholar 

  24. Jenkinson MD, Hayhurst C, Al-Jumaily M, Kandasamy J, Clark S, Mallucci CL (2009) The role of endoscopic third ventriculostomy in adult patients with hydrocephalus. J Neurosurg 110(5):861–866

    Article  PubMed  Google Scholar 

  25. Jones RF, Kwok BC, Stening WA, Vonau M (1996) Third ventriculostomy for hydrocephalus associated with spinal dysraphism: indications and contraindications. Eur J Pediatr Surg 6(Suppl 1):5–6

    Article  PubMed  Google Scholar 

  26. Kadri H, Mawla AA (2004) Variations of endoscopic ventricular anatomy in children suffering from hydrocephalus associated with myelomeningocele. Minim Invasive Neurosurg 47(6):339–341

    Article  PubMed  CAS  Google Scholar 

  27. Kestle JR, Walker ML, Investigators S (2005) A multicenter prospective cohort study of the strata valve for the management of hydrocephalus in pediatric patients. J Neurosurg 102(2 Suppl):141–145

    PubMed  Google Scholar 

  28. Koch D, Wagner W (2004) Endoscopic third ventriculostomy in infants of less than 1 year of age: which factors influence the outcome? Childs Nerv Syst 20:405–411

    Article  PubMed  Google Scholar 

  29. Kwick SJ, Mandera M, Bazowski P, Luszawski J, Duda I, Wolwender A, Zymon-Zagòrska A, Grzybowska K (2003) Endoscopic third ventriculostomy for hydrocephalus: early and late efficacy in relation to etiology. Acta Neurochir 145:181–184

    Article  Google Scholar 

  30. Machado HR, de Oliveira RS (2004) Simultaneous repair of myelomeningocele and shunt insertion. Childs Nerv Syst 20:107–109

    Article  PubMed  Google Scholar 

  31. Massimi L, Paternoster G, Fasano T, Di Rocco C (2009) On the changing epidemiology of hydrocephalus. Childs Nerv Syst 25(7):795–800

    Article  PubMed  Google Scholar 

  32. McLone D, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosc 15:1–12

    Article  CAS  Google Scholar 

  33. McLone DG, Czyzewski D, Raimondi AJ, Sommers RC (1982) Central nervous system infections as a limiting factor in the intelligence of children with myelomeningocele. Pediatrics 70:338–342

    Google Scholar 

  34. McLone DG, Dias MS (2003) The Chiari II malformation: cause and impact. Childs Nerv Syst 19(7–8):540–550

    Article  PubMed  Google Scholar 

  35. Miller PD, Pollack IF, Pang D, Albright AL (1996) Comparison of simultaneous versus delayed ventriculoperitoneal shunt insertion in children undergoing myelomeningocele repair. J Child Neurol 11(5):370–372

    Article  PubMed  CAS  Google Scholar 

  36. Mirzai H, Erşahin Y, Mutluer S, Kayahan A (1998) Outcome of patients with meningomyelocele: the Ege University experience. Childs Nerv Syst 14(3):120–123

    Article  PubMed  CAS  Google Scholar 

  37. Mori H, Oi S, Nonaka Y, Tamogami R, Muroi A (2008) Ventricular anatomy of hydrocephalus associated with myeloschisis and endoscopic third ventriculostomy. Childs Nerv Syst 24(6):717–722

    Article  PubMed  Google Scholar 

  38. Murshid WR (2000) Endoscopic third ventriculostomy: towards more indications for the treatment of non-communicating hydrocephalus. Minim Invas Neurosurg 43:75–82

    Article  CAS  Google Scholar 

  39. Nejat F, Tajik P, El Khashab M, Kazmi SS, Khotaei GT, Salahesh S (2008) A randomized trial of ceftriaxone versus trimethoprim-sulfamethoxazole to prevent ventriculoperitoneal shunt infection. J Microbiol Immunol Infect 41(2):112–117

    PubMed  CAS  Google Scholar 

  40. O’Hayon BB, Drake JM, Ossip MG, Tuli S, Clarke M (1998) Frontal and occipital horn ratio: a linear estimate of ventricular size for multiple imaging modalities in pediatric hydrocephalus. Pediatr Neurosurg 29(5):245–249

    Article  PubMed  Google Scholar 

  41. Oi S, Di Rocco C (2006) Proposal of “evolution theory in cerebrospinal fluid dynamics” and minor pathway hydrocephalus in developing immature brain. Childs Nerv Syst 22(7):662–669

    Article  PubMed  Google Scholar 

  42. Oktem IS, Menkü A, Ozdemir A (2008) When should ventriculoperitoneal shunt placement be performed in cases with myelomeningocele and hydrocephalus? Turk Neurosurg 18(4):387–391

    PubMed  Google Scholar 

  43. Pang D (1995) Surgical complications of open spinal dysraphism. Neurosurg Clin N Am 6(2):243–257

    PubMed  CAS  Google Scholar 

  44. Parent AD, McMillan T (1995) Contemporaneous shunting with repair of myelomeningocele. Pediatr Neurosurg 22(3):132–136

    Article  PubMed  CAS  Google Scholar 

  45. Pavez A, Salazar C, Rivera R, Contreras J, Orellana A, Guzman C, Iribarren O, Hernandez H, Elzo J, Moraga D (2006) Description of endoscopic ventricular anatomy in myelomeningocele. Minim Invasive Neurosurg 49(3):161–167

    Article  PubMed  CAS  Google Scholar 

  46. Peretta P, Ragazzi P, Galarza M, Genitori L, Giordano F, Mussa F, Cinalli G (2006) Complications and pitfalls of neuroendoscopic surgery in children. J Neurosurg 105(3 Suppl):187–93

    PubMed  Google Scholar 

  47. Portillo S, Zuccaro G, Fernandez-Molina A, Houssay A, Sosa F, Konsol O, Jaimovich R, Olivella E, Ledesma J, Guevara M, Ajler G, Picco P (2004) Endoscopic third ventriculostomy in the treatment of pediatric hydrocephalus. A multicentric study. Child’s Nerv Syst 20(8–9):666–667

    Google Scholar 

  48. Radmanesh F, Nejat F, El Kashab M, Ghodsi SM, Ardebili HE (2009) Shunt complications in children with myelomeningocele: effect of timing of shunt placement. J Neurosurg Pediatr 3:516–520

    Article  PubMed  Google Scholar 

  49. Rintoul NE, Sutton LN, Hubbard AM, Cohen B, Melchionni J, Pasquariello PS, Adzick NS (2002) A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics 109(3):409–413

    Article  PubMed  Google Scholar 

  50. Russell DS (1966) Observation on the pathology of hydrocephalus. Her Majesty’s Stationary Office, London, pp 21–37

    Google Scholar 

  51. Sgouros S (2004) Hydrocephalus with myelomeningocele. In: Cinalli G, Meixner W, Sainte-Rose C (eds) Pediatric hydrocephalus. Springer-Verlag, Milan, pp 133–144

    Google Scholar 

  52. Stein SC, Schut L (1979) Hydrocephalus in myelomeningocele. Childs Brain 5(4):413–419

    PubMed  CAS  Google Scholar 

  53. Steinbok P, Irvine B, Cochrane DD, Irwin BJ (1992) Long-term outcome and complications of children born with meningomyelocele. Childs Nerv Syst 8(2):92–96

    Article  PubMed  CAS  Google Scholar 

  54. Stevenson KL (2004) Chiari type II malformation: past, present, and future. Neurosurg Focus 16(2):E5

    Article  PubMed  Google Scholar 

  55. Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW (1999) Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282(19):1826–1831

    Article  PubMed  CAS  Google Scholar 

  56. Tamburrini G, Caldarelli M, Massimi L, Ramirez-Reyes G, Di Rocco C (2004) Primary and secondary third ventriculostomy in children with hydrocephalus and myelomeningocele. Child’s Nerv Syst 20(8–9):666

    Google Scholar 

  57. Teo C, Jones R (1996) Management of hydrocephalus by endoscopic third ventriculostomy in patients with myelomeningocele. Pediatr Neurosurg 25:57–63

    Article  PubMed  CAS  Google Scholar 

  58. Tortori-Donati P, Rossi A, Cama A (2000) Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology 42(7):471–491

    Article  PubMed  CAS  Google Scholar 

  59. Tubbs RS, Oakes WJ (2004) Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20(6):375–381

    Article  PubMed  Google Scholar 

  60. Tuli S, Drake J, Lamberti-Pasculli M (2003) Long-term outcome of hydrocephalus management in myelomeningoceles. Childs Nerv Syst 19(5–6):286–291

    Article  PubMed  Google Scholar 

  61. Tuli S, Drake J, Lawless J, Wigg M, Lamberti-Pasculli M (2000) Risk factors for repeated cerebrospinal shunt failures in pediatric patients with hydrocephalus. J Neurosurg 92(1):31–38

    Article  PubMed  CAS  Google Scholar 

  62. Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31(3):137–142

    Article  PubMed  CAS  Google Scholar 

  63. Tulipan N, Sutton LN, Bruner JP, Cohen BM, Johnson M, Adzick NS (2003) The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg 38(1):27–33

    Article  PubMed  Google Scholar 

  64. Wakhlu A, Ansari NA (2004) The prediction of postoperative hydrocephalus in patients with spina bifida. Childs Nerv Syst 20(2):104–106

    Article  PubMed  Google Scholar 

  65. Walker DG, Coyne TJ, Kahler RJm Tomlinson FH (2003) Failure of endoscopic third ventriculostomy in myelomeningocele patients: preoperative clinical and radiological features. Child’s Nerv Syst 19(9):707–708

    Google Scholar 

  66. Warf B, Ondoma S, Kulkarni A, Donnelly R, Ampeire M, Akona J, Kabachelor CR, Mulondo R, Nsubuga BK (2009) Neurocognitive outcome and ventricular volume in children with myelomeningocele treated for hydrocephalus in Uganda. J Neurosurg Pediatr 4(6):564–570

    Article  PubMed  Google Scholar 

  67. Warf BC (2011) Hydrocephalus associated with neural tube defects: characteristics, management, and outcome in sub-Saharan Africa. Childs Nerv Syst 27:1589–1594

    Article  PubMed  Google Scholar 

  68. Warf BC, Campbell JW (2008) Combined endoscopic third ventriculostomy and choroid plexus cauterization as primary treatment of hydrocephalus for infants with myelomeningocele: long-term results of a prospective intent-to-treat study in 115 East African infants. J Neurosurg Pediatr 2(5):310–316

    Article  PubMed  Google Scholar 

  69. Williams H (2008) A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida. Cerebrospinal Fluid Res 5:7

    Article  PubMed  Google Scholar 

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Tamburrini, G., Frassanito, P., Iakovaki, K. et al. Myelomeningocele: the management of the associated hydrocephalus. Childs Nerv Syst 29, 1569–1579 (2013). https://doi.org/10.1007/s00381-013-2179-4

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