Advertisement

Pathophysiology of Hydrocephalus

  • Giuseppe Cinalli
  • Pietro Spennato
  • Maria Consiglio Buonocore
  • Emilio Cianciulli
  • Matthieu Vinchon
  • Spyros Sgouros

Abstract

The three major clinical manifestations of spina bifida (hydrocephalus, paraplegia and urinary and bowel incontinence) are easily observable and have been described since ancient times, though they were not described in relationship to spina bifida until the seventeenth century [1].

Keywords

Posterior Fossa Spina Bifida Aqueductal Stenosis Foramen Magnum Shunt Malfunction 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    Smith GK (2001) The history of spina bifida, hydrocephalus, paraplegia and incontinence. Pediatr Surg Int 17:424–432PubMedCrossRefGoogle Scholar
  2. 2.
    Morgagni GB (1960) The seats and causes of diseases investigated by anatomy in 5 Books, Bk 1, Letter 12. Translated from the Latin by Benjamin Alexander, vol. 1. Hafner (1760) New York, pp 244–274Google Scholar
  3. 3.
    Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatr 28:247–259PubMedGoogle Scholar
  4. 4.
    Laurence KM, Coates S (1962) The natural history of hydrocephalus. Detailed analysis of 182 unoperated cases. Arch Dis Child 37:345–362PubMedGoogle Scholar
  5. 5.
    Sgouros S (2004) Hydrocephalus with myelomeningocele. In: Cinalli G, Maixner WJ, Sainte-Rose C (eds) Pediatric hydrocephalus. Springer-Verlag, Italia, pp 133–144Google Scholar
  6. 6.
    Mirzai H, Eriahin Y, Mutluer S, Kayahan A (1998) Outcome of patients with meningomyelocele: The Ege University experience. Childs Nerv Syst 14:120–123PubMedCrossRefGoogle Scholar
  7. 7.
    Steinbok P, Irvine B, Cochrane DD, Irwin BJ (1992) Long-term outcome and complications of children born with meningomyelocele. Childs Nerv Syst 8:92–96PubMedCrossRefGoogle Scholar
  8. 8.
    Rintoul NE, Sutton LN, Hubbard AM et al (2002) A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics 109:409–413PubMedCrossRefGoogle Scholar
  9. 9.
    Nishino A, Shirane R, So K et al (1998) Cervical myelocystocele with Chiari II malformation: Magnetic resonance imaging and surgical treatment. Surg Neurol 49:269–273PubMedCrossRefGoogle Scholar
  10. 10.
    Pang D, Dias MS (1993) Cervical myelomeningoceles. Neurosurgery 33:363–372PubMedCrossRefGoogle Scholar
  11. 11.
    Rossi A, Piatelli G, Gandolfo C, Pavanello M et al (2006) Spectrum of nonterminal myelocystoceles. Neurosurgery 58:509–15PubMedGoogle Scholar
  12. 12.
    Salomao JF, Cavalheiro S, Matushita H et al (2006) Cystic spinal dysraphism of the cervical and upper thoracic region. Childs Nerv Syst 22:234–42PubMedCrossRefGoogle Scholar
  13. 13.
    Dias MS, McLone DG (1993) Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am 4:715–726PubMedGoogle Scholar
  14. 14.
    Rekate HL (1991–1992) Shunt revision: complications and their prevention. Pediatr Neurosurg 17:155–162PubMedCrossRefGoogle Scholar
  15. 15.
    Aubry MC, Aubry JP, Dommergues M (2003) Sonographic prenatal diagnosis of central nervous system abnormalities. Childs Nerv Syst 19:391–402PubMedCrossRefGoogle Scholar
  16. 16.
    Wilhelm C, Keck C, Hess S et al (1998) Ventriculomegaly diagnosed by prenatal ultrasound and mental development of the children. Fetal Diagn Ther 13:162–166PubMedCrossRefGoogle Scholar
  17. 17.
    Garel C, Luton D, Oury JF, Gressens P (2003) Ventricular dilatations. Childs Nerv Syst 19:517–523PubMedCrossRefGoogle Scholar
  18. 18.
    Babcook CJ, Goldstein RB, Barth RA (1994) Prevalence of ventriculomegaly in association with myelomeningocele. Radiology 190:703–707PubMedGoogle Scholar
  19. 19.
    Van der Hof MC, Nicolaides KH, Campbell J, Campbell S (1991) Evaluation of the lemon and banana signs in one hundred thirty fetuses with open spina bifida. Am J Obstet Gynecol 162:322–327Google Scholar
  20. 20.
    Bloom SL, Bloom DD, Dellanebbia C et al (1997) The developmental outcome of children with antenatal mild isolated ventriculomegaly. Obstet Gynecol 90:93–97PubMedCrossRefGoogle Scholar
  21. 21.
    Gupta JK, Bryce FC, Lilford RJ (1994) Management of apparently isolated fetal ventriculomegaly. Obstet Gynecol Surv 49:716–721PubMedCrossRefGoogle Scholar
  22. 22.
    Hammond MK, Milhorat TH, Baron IS (1976) Normal pressure hydrocephalus in patients with myelomeningocele. Dev Med Child Neurol Suppl 37:55–68Google Scholar
  23. 23.
    Iborra J, Pages E, Cuxart A et al (2000) Increased intracranial pressure in myelomeningocele (MMC) patients never shunted: results of a prospective preliminary study. Spinal Cord 38:495–497PubMedCrossRefGoogle Scholar
  24. 24.
    McLone DG, Nakahara S, Knepper PA (1991) Chiari II malformation: pathogenesis and dynamics. Concepts Pediatr Neurosurg 11:1–17Google Scholar
  25. 25.
    McLone DG, Dias MS (2003) The Chiari II malformation: cause and impact. Childs Nerv Syst 19:540–550PubMedCrossRefGoogle Scholar
  26. 26.
    Chiari H (1891) Uber Veränderungen des Kleinhirns infolge von Hydrocephaliedes Grosshirns. Dtsch Med Wschr 17:1172–1175Google Scholar
  27. 27.
    Stevenson KL (2004) Chiari Type II malformation: past, present, and future. Neurosurg Focus 16(2):Article 5CrossRefGoogle Scholar
  28. 28.
    Naidich TP, Pudlowski RM, Naidich JB et al (1980) Computed tomographic signs of the Chiari II malformation. Part I: skull and dural partitions. Radiology 134:65–71PubMedGoogle Scholar
  29. 29.
    Rekate HL (1984) To shunt or not to shunt: hydrocephalus and dysraphism. Clin Neurosurg 32:593–607Google Scholar
  30. 30.
    Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50:29–55PubMedCrossRefGoogle Scholar
  31. 31.
    Padget DH (1972) Development of so-called dysraphism; with embryologic evidence of clinical Arnold-Chiari and Dandy-Walker malformations. Johns Hopkins Med J 130:127–165PubMedGoogle Scholar
  32. 32.
    Penfield W, Coburn DF (1938) Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatry 40:328–336Google Scholar
  33. 33.
    McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12PubMedCrossRefGoogle Scholar
  34. 34.
    Andreussi L, Clarisse J, Jomin M et al (1977) Diagnostic value of water-soluble contrast iodoventriculography in the study of Arnold-Chiari syndrome. Mod Probl Paediatr 18:137–141Google Scholar
  35. 35.
    Tulipan N (2003) Intrauterine myelomeningocele repair. Clin Perinatol 30:521–530PubMedCrossRefGoogle Scholar
  36. 36.
    Tulipan N, Hernanz-Schulman M, Bruner JP (1998) Reduced hindbrain herniation after intrauterine myelomeningocele repair: a report of four cases. Pediatr Neurosurg 29:274–278PubMedCrossRefGoogle Scholar
  37. 37.
    Tulipan N, Hernanz-Schulman M, Lowe LH et al (1999) Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation. Pediatr Neurosurg 31:137–142PubMedCrossRefGoogle Scholar
  38. 38.
    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 282:1826–1831PubMedCrossRefGoogle Scholar
  39. 39.
    Holinger PC, Holinger LD, Reichert TJ et al (1978) Respiratory obstruction and apnea in infants with bilateral abductor vocal cord paralysis, meningomyelocele, hydrocephalus, and Arnold-Chiari malformation. J Pediatr 92:368–373PubMedCrossRefGoogle Scholar
  40. 40.
    Berger MS, Sundsten J, Lemire RJ et al (1990) Pathophysiology of isolated lateral ventriculomegaly in shunt myelodysplastic children. Pediatr Neurosurg 16:301–304PubMedCrossRefGoogle Scholar
  41. 41.
    Spennato P, Cinalli G, Carannante G et al (2004) Multiloculated hydrocephalus. In: Cinalli G, Maixner WJ, Saint-Rose C (eds) Pediatric hydrocephalus. Springer-Verlag, Milan, pp 219–244Google Scholar
  42. 42.
    Dandy WE, Blackfan KD (1914) Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child 8: 406–482Google Scholar
  43. 43.
    Gilbert JN, Jones KL, Rorke LB et al (1986) Central nervous system anomalies associated with menin — gomyelocele, hydrocephalus, and the Arnold-Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects. Neurosurgery 18: 559–564PubMedCrossRefGoogle Scholar
  44. 44.
    Shurtleff DB, Kronmal R, Foltz EL (1975) Follow-up comparison of hydrocephalus with and without myelomeningocele. J Neurosurg 42: 61–68PubMedGoogle Scholar
  45. 45.
    Williams B (1975) Cerebrospinal fluid pressure-gradients in spina bifida cystica, with special reference to Arnold-Chiari malformation and aqueductal stenosis. Dev Med Child Neurol Suppl 35:138–150PubMedGoogle Scholar
  46. 46.
    Andweg J (1989) Intracranial venous pressures, hydrocephalus and effects on cerebrospinal fluid shunts. Childs Nerv Syst 5:318–323CrossRefGoogle Scholar
  47. 47.
    Sainte-Rose C, LaCombe J, Pierre-Khan A et al (1984) Intracranial venous sinus hypertension: cause or consequence of hydrocephalus in infants? J Neurosurg 60:727–736PubMedGoogle Scholar
  48. 48.
    Nadkarni TD, Rekate HL (2005) Treatment of refractory intracranial hypertension in a spina bifida patient by a concurrent ventricular and cisterna magna-to-peritoneal shunt. Childs Nerv Syst 21:579–582PubMedCrossRefGoogle Scholar
  49. 49.
    Greitz D (2004) Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev 27:145–165PubMedGoogle Scholar
  50. 50.
    Teo C, Jones R (1996) Management of hydrocephalus by endoscopic third ventriculostomy in patients with myelomeningocele. Pediatr Neurosurg 25:57–63PubMedCrossRefGoogle Scholar
  51. 51.
    Encha-Razavi F (2003) Identification of brain malformations: neuropathological approach. Childs Nerv Syst 19:448–454PubMedCrossRefGoogle Scholar
  52. 52.
    Rollins N, Joglar J, Perlman J (1999) Coexistent holoprosencephaly and Chiari II malformation. AJNR Am J Neuroradiol 20:1678–1681PubMedGoogle Scholar
  53. 53.
    Osaka J, Tanimura T, Hirayama A et al (1978) Myelo — meningocele before birth. J Neurosurg 49:711–724PubMedCrossRefGoogle Scholar
  54. 54.
    Chen CY, Zimmerman RA (2000) Congenital brain anomalies. In: Zimmerman RA, Gibby WA, Carmody RG (Eds) Neuroimaging: clinical and physical principles. Springer-Verlag, New York, pp 491–530Google Scholar
  55. 55.
    Friede RL (1989) Developmental neuropathology. Springer, Berlin HeidelbergGoogle Scholar
  56. 56.
    Pavez A, Salazar C, Rivera R et al (2006) Description of endoscopic ventricular anatomy in myelomeningocele. Minim Invas Neurosurg 49:161–167CrossRefGoogle Scholar
  57. 57.
    Russell Dorothy S (1935) The mechanism of internal hydrocephalus in spina bifida. Brain 58:203–215CrossRefGoogle Scholar
  58. 58.
    Garonzik IM, Samdani AF, Carson BS, Avellino A (2001) Pneumocephalus in a newborn with an open myelomeningocele. Pediatr Neurosurg 35:334PubMedCrossRefGoogle Scholar
  59. 59.
    Tulipan N, Sutton LN, Bruner JP et al (2003) The effect of intrauterine myelomeningocele repair on the incidence of shunt-dependent hydrocephalus. Pediatr Neurosurg 38:27–33PubMedCrossRefGoogle Scholar
  60. 60.
    Bruner JP, Tulipan N, Reed G et al (2004) Intrauterine repair of spina bifida: preoperative predictors of shunt-dependent hydrocephalus. Am J Obstetr Gynecol 190:1305–1312CrossRefGoogle Scholar
  61. 61.
    Crimmins D, Hayward RD, Thompson DNP (2005) Reducing shunt placement rate in myelomeningocele patients (abstr). Childs Nerv Syst 21:828–829Google Scholar
  62. 62.
    Bruner JP, Tulipan N, Paschall RL et al (1999) Fetal surgery for myelomeningocele and the incidence of shunt dependent hydrocephalus. JAMA 282:1819–1825PubMedCrossRefGoogle Scholar
  63. 63.
    Simpson JL (1999) Fetal surgery for myelomeningocele: promise, progress and problems. JAMA 17:1873–1874CrossRefGoogle Scholar
  64. 64.
    Di Rocco C, Cinalli G, Massimi L et al (2006) Endoscopic third ventriculostomy in the treatment of hydrocephalus in pediatric patients. Adv Tech Stand Neurosurg 31:119–219PubMedCrossRefGoogle Scholar
  65. 65.
    Fritsch MJ, Mehdorn HM (2003) Indication and controversies for endoscopic third ventriculostomy in children. Childs Nerv Syst 19:706–707Google Scholar
  66. 66.
    Teo C, Jones R (1996) Management of hydrocephalus by endoscopic third ventriculostomy in patients with myelomeningocele. Pediatr Neurosurg 25:57–63PubMedCrossRefGoogle Scholar
  67. 67.
    Portillo S, Zuccaro G, Fernandez-Molina A et al (2004) Endoscopic third ventriculostomy in the treatment of pediatric hydrocephalus. A multicentric study. Childs Nerv Syst 20: 666–667Google Scholar
  68. 68.
    Tamburrini G, Caldarelli M, Massimi L et al (2004) Primary and secondary third ventriculostomy in children with hydrocephalus and myelomeningocele. Childs Nerv Syst 20:666Google Scholar
  69. 69.
    Tada T, Kanaji M, Kobayashi S (1994) Induction of communicating hydrocephalus in mice by intrathecal injection of human recombinant transforming growth factor-beta 1. J Neuroimmunol 50:153–158PubMedCrossRefGoogle Scholar
  70. 70.
    Wyss-Coray T, Feng L, Masliah E et al (1995) Increased central nervous system production of extracellular matrix components and development of hydrocephalus in transgenic mice overexpressing transforming growth factor-beta 1. Am J Pathol 147:53–67PubMedGoogle Scholar
  71. 71.
    Heep A, Bartmann P, Stoffel-Wagner B et al (2006) Cerebrospinal fluid obstruction and malabsorption in human neonatal hydrocephaly. Childs Nerv Syst 22:1249–1255PubMedCrossRefGoogle Scholar
  72. 72.
    O’Brien DF, Javadpour M, Collins DR et al (2005) Endoscopic third ventriculostomy: an outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction. J Neurosurg (5 Suppl Pediatrics) 103:393–400Google Scholar

Copyright information

© Springer-Verlag Italia 2008

Authors and Affiliations

  • Giuseppe Cinalli
    • 1
  • Pietro Spennato
    • 1
  • Maria Consiglio Buonocore
    • 2
  • Emilio Cianciulli
    • 2
  • Matthieu Vinchon
    • 3
  • Spyros Sgouros
    • 4
    • 5
  1. 1.Department of Paediatric NeurosurgerySantobono-Pausilipon Children’s HospitalNaplesItaly
  2. 2.Department of NeuroradiologySantobono-Pausilipon Children’s HospitalNaplesItaly
  3. 3.Department of Pediatric NeurosurgeryUniversity HospitalLilleFrance
  4. 4.Institute of Child Health and Department of NeurosurgeryBirmingham Children’s HospitalBirminghamUK
  5. 5.Department of NeurosurgeryUniversity of AthensAthensGreece

Personalised recommendations