Challenging Problems of Shunt Management in Spina Bifida

  • Harold L. Rekate


Hydrocephalus in the context of spina bifida may represent the most complicated and confusing form of congenital hydrocephalus. Essentially all patients with spina bifida aperta have an associated Chiari II malformation. In some, especially those with very low lesion levels, this malformation may be very mild. Experimental evidence suggests that the Chiari II malformation results from in utero loss of cerebrospinal fluid (CSF) into the amniotic fluid and subsequent failure of distension of the fetal head leading to a very small posterior fossa and severe herniation of the hindbrain [1]. One of the major indications for prenatal repair of the myelomeningocele is to minimize this descent of the cerebellum and brainstem and therefore maximize the chance that the patient may be able to thrive without a shunt [2, 3, 4].


Spina Bifida Shunt Failure Dural Venous Sinus Spinal Subarachnoid Space Small Ventricle 
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  1. 1.
    McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12PubMedCrossRefGoogle Scholar
  2. 2.
    Bruner JP, Tulipan N, Reed G et al (2004) Intrauterine repair of spina bifida: preoperative predictors of shunt-dependent hydrocephalus. Am J Obstet Gynecol 190:1305–1312PubMedCrossRefGoogle Scholar
  3. 3.
    Johnson MP, Gerdes M, Rintoul N et al (2006) Maternal-fetal surgery for myelomeningocele: neurodevelopmental outcomes at 2 years of age. Am J Obstet Gynecol 194:1145–1150PubMedCrossRefGoogle Scholar
  4. 4.
    Sutton LN, Adzick NS (2004) Fetal surgery for myelomeningocele. Clin Neurosurg 51:155–162PubMedGoogle Scholar
  5. 5.
    Rekate HL (1988) Management of hydrocephalus and the erroneus concept of shunt independence in spina bifida. BNI Quarterly 4:17–20Google Scholar
  6. 6.
    Rekate HL (1991) Neurosurgical management of the child with spina bifida. CRC Press, Boca RatonGoogle Scholar
  7. 7.
    Rekate HL, Nulsen FE, Mack H et al (1982) Establishing the diagnosis of shunt independence. Monogr Neural Sci 8:223–226Google Scholar
  8. 8.
    Engel M, Carmel PW, Chutorian AM (1979) Increased intraventricular pressure without ventriculomegaly in children with shunts: “normal volume” hydrocephalus. Neurosurgery 5:549–552PubMedCrossRefGoogle Scholar
  9. 9.
    Rekate HL (1993) Classification of slit-ventricle syndromes using intracranial pressure monitoring. Pediatr Neurosurg 19:15–20PubMedCrossRefGoogle Scholar
  10. 10.
    Hall P, Lindseth R, Campbell R et al (1979) Scoliosis and hydrocephalus in myelocele patients. The effects of ventricular shunting. J Neurosurg 50:174–178PubMedGoogle Scholar
  11. 11.
    Dias MS, McLone DG (1993) Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am 4:715–726PubMedGoogle Scholar
  12. 12.
    Le H, Yamini B, Frim DM (2002) Lumboperitoneal shunting as a treatment for slit ventricle syndrome. Pediatr Neurosurg 36:178–182PubMedCrossRefGoogle Scholar
  13. 13.
    Rekate HL, Wallace D (2003) Lumboperitoneal shunts in children. Pediatr Neurosurg 38:41–46PubMedCrossRefGoogle Scholar
  14. 14.
    Johnston IH, Sheridan MM (1993) CSF shunting from the cisterna magna: a report of 16 cases. Br J Neurosurg 7:39–43PubMedCrossRefGoogle Scholar
  15. 15.
    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
  16. 16.
    Rekate HL, Nadkarni T, Wallace D (2006) Severe intracranial hypertension in slit ventricle syndrome managed using a cisterna magna-ventricle-peritoneum shunt. J Neurosurg 104:240–244PubMedGoogle Scholar
  17. 17.
    Baskin JJ, Manwaring KH, Rekate HL (1998) Ventricular shunt removal: the ultimate treatment of the slit ventricle syndrome. J Neurosurg 88:478–484PubMedCrossRefGoogle Scholar
  18. 18.
    Rekate HL, Brodkey JA, Chizeck HJ et al (1988) Ventricular volume regulation: a mathematical model and computer simulation. Pediatr Neurosci 14:77–84PubMedCrossRefGoogle Scholar
  19. 19.
    Ransohoff J, Shulman K, Fishman RA (1960) Hydrocephalus: a review of etiology and treatment. J Pediatr 56:399–411PubMedCrossRefGoogle Scholar
  20. 20.
    Rekate HL (1989) Circuit diagram of the circulation of cerebrospinal fluid. In: Marlin AE (ed) Concepts in Pediatric Neurosurgery. Karger, Zurich, pp 46–56Google Scholar
  21. 21.
    Rekate HL (1989) Resistance elements within the cerebrospinal fluid circulation. In: Gjerris F, Borgesen SE, Solberg-Sorensen P (eds) Alfred Benzon symposium. Munksgaard, Copenhagen, pp 45–52Google Scholar

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© Springer-Verlag Italia 2008

Authors and Affiliations

  • Harold L. Rekate
    • 1
    • 2
  1. 1.Pediatric NeurosciencesBarrow Neurologic InstitutePhoenixUSA
  2. 2.Department of Surgery Section of NeurosurgeryUniversity of Arizona School of MedicineTucsonUSA

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