Hydrocephalus in Chiari Malformations and Other Craniovertebral Junction Abnormalities

  • Brian J. DlouhyEmail author
  • Arnold H. Menezes
Living reference work entry


The craniovertebral junction (CVJ), or the craniocervical junction as it is otherwise known, is composed of the occipital bone that surrounds the foramen magnum, the atlas vertebrae, the axis vertebrae, and their associated ligaments and musculature. CVJ abnormalities are a varied group of conditions with different etiologies and have the potential to affect CSF flow by fourth ventricular outlet obstruction, venous flow abnormalities, or other mechanisms. Here we review the pathogenesis, presentation, and imaging characteristics of Chiari I and Chiari II malformations. We describe the pathogenesis of hydrocephalus associated with these conditions as well as the management strategy and treatment options. In addition, we describe other CVJ abnormalities associated with hydrocephalus and the optimal treatment strategy. These include basilar invagination, basilar impression, and achondroplasia.


Hydrocephalus Craniovertebral junction Craniocervical junction Syringomyelia Syringobulbia Chiari malformation 


  1. Aboulezz AO, Sartor K, Geyer CA et al (1985) Position of cerebellar tonsils in the normal population and in patients with Chiari malformation: a quantitative approach with MR imaging. J Comput Assist Tomogr 9:1033–1036CrossRefPubMedGoogle Scholar
  2. Adzick NS, Thom EA, Spong CY et al (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004CrossRefPubMedPubMedCentralGoogle Scholar
  3. Amacher AL, Page LK (1971) Hydrocephalus due to membranous obstruction of the fourth ventricle. J Neurosurg 35:672–676CrossRefPubMedGoogle Scholar
  4. Arnold J (1894) Myelocyste, Transposition von Gewebskeimen und Sympodie. Beitr Path Anat 16:1–28Google Scholar
  5. Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218CrossRefPubMedGoogle Scholar
  6. Barkovich AJ, Wippold FJ, Sherman JL et al (1986) Significance of cerebellar tonsillar position on MR. AJNR Am J Neuroradiol 7:795–799PubMedGoogle Scholar
  7. Barr ML (1948) Observations on the foramen of Magendie in a series of human brains. Brain 71:281–289CrossRefPubMedGoogle Scholar
  8. Bosemani T, Orman G, Hergan B et al (2015) Achondroplasia in children: correlation of ventriculomegaly, size of foramen magnum and jugular foramina, and emissary vein enlargement. Childs Nerv Syst 31:129–133CrossRefPubMedGoogle Scholar
  9. Chern JJ, Gordon AJ, Mortazavi MM et al (2011) Pediatric Chiari malformation type 0: a 12-year institutional experience. J Neurosurg Pediatr 8:1–5CrossRefPubMedGoogle Scholar
  10. Chiari H (1891) Ueber Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17:1172–1175CrossRefGoogle Scholar
  11. Chiari H (1896) Ueber Veranderungen des Kleinhirns, des Pons und der Medulla oblongata Infolge von congenitaler Hydrocephalie des Grosshirns. Denkschriften Kais Akad Wiss Math-Naturw 63:71–116Google Scholar
  12. Cinalli G, Sainte-Rose C, Chumas P et al (1999) Failure of third ventriculostomy in the treatment of aqueductal stenosis in children. J Neurosurg 90:448–454CrossRefPubMedGoogle Scholar
  13. Cleland J (1883) Contribution to the study of spina bifida, encephalocele, and anencephalus. J Anat Physiol 17:257–292PubMedPubMedCentralGoogle Scholar
  14. Decq P, Le Guerinel C, Sol JC et al (2001) Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy. J Neurosurg 95:783–790CrossRefPubMedGoogle Scholar
  15. Dlouhy BJ, Capuano AW, Madhavan K et al (2012) Preoperative third ventricular bowing as a predictor of endoscopic third ventriculostomy success. J Neurosurg Pediatr 9:182–190CrossRefPubMedGoogle Scholar
  16. Dlouhy BJ, Dahdaleh NS, Menezes AH (2015) Evolution of transoral approaches, endoscopic endonasal approaches, and reduction strategies for treatment of craniovertebral junction pathology: a treatment algorithm update. Neurosurg Focus 38:E8CrossRefPubMedGoogle Scholar
  17. Drake JM (1993) Ventriculostomy for treatment of hydrocephalus. Neurosurg Clin N Am 4:657–666PubMedCrossRefGoogle Scholar
  18. Dyste GN, Menezes AH (1988) Presentation and management of pediatric Chiari malformations without myelodysplasia. Neurosurgery 23:589–597CrossRefPubMedGoogle Scholar
  19. Dyste GN, Menezes AH, VanGilder JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168CrossRefPubMedGoogle Scholar
  20. el Gammal T, Mark EK, Brooks BS (1988) MR imaging of Chiari II malformation. AJR Am J Roentgenol 150:163–170CrossRefPubMedGoogle Scholar
  21. Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiologic reappraisal. Radiology 183:347–353CrossRefPubMedGoogle Scholar
  22. Fenoy AJ, Menezes AH, Fenoy KA (2008) Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia. J Neurosurg Spine 9:1–9CrossRefPubMedGoogle Scholar
  23. Greenlee JD, Donovan KA, Hasan DM et al (2002) Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics 110:1212–1219CrossRefPubMedGoogle Scholar
  24. Hayhurst C, Osman-Farah J, Das K et al (2008) Initial management of hydrocephalus associated with Chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg 108:1211–1214CrossRefPubMedGoogle Scholar
  25. Hida K, Iwasaki Y, Imamura H et al (1994) Birth injury as a causative factor of syringomyelia with Chiari type I deformity. J Neurol Neurosurg Psychiatry 57:373–374CrossRefPubMedPubMedCentralGoogle Scholar
  26. Iskandar BJ, Hedlund GL, Grabb PA et al (1998) The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212–216CrossRefPubMedGoogle Scholar
  27. Iwasaki Y, Hida K, Onishi K et al (2000) Chiari malformation and syringomyelia in monozygotic twins: birth injury as a possible cause of syringomyelia – case report. Neurol Med Chir (Tokyo) 40:176–178CrossRefGoogle Scholar
  28. Jones RF, Kwok BC, Stening WA et al (1996) Third ventriculostomy for hydrocephalus associated with spinal dysraphism: indications and contraindications. Eur J Pediatr Surg 6(Suppl 1):5–6CrossRefPubMedGoogle Scholar
  29. Kasapas K, Varthalitis D, Georgakoulias N et al (2015) Hydrocephalus due to membranous obstruction of Magendie’s foramen. J Korean Neurosurg Soc 57:68–71CrossRefPubMedPubMedCentralGoogle Scholar
  30. King JA, Vachhrajani S, Drake JM et al (2009) Neurosurgical implications of achondroplasia. J Neurosurg Pediatr 4:297–306CrossRefPubMedGoogle Scholar
  31. Massimi L, Pravata E, Tamburrini G et al (2011) Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery 68:950–956CrossRefPubMedGoogle Scholar
  32. McLendon RE, Crain BJ, Oakes WJ et al (1985) Cerebral polygyria in the Chiari type II (Arnold-Chiari) malformation. Clin Neuropathol 4:200–205PubMedGoogle Scholar
  33. McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12CrossRefPubMedGoogle Scholar
  34. Menezes AH (1991) Complications of surgery at the craniovertebral junction – avoidance and management. Pediatr Neurosurg 17:254–266CrossRefPubMedGoogle Scholar
  35. Menezes AH, Greenlee JD, Donovan KA (2005) Honored guest presentation: lifetime experiences and where we are going: Chiari I with syringohydromyelia – controversies and development of decision trees. Clin Neurosurg 52:297–305PubMedGoogle Scholar
  36. Metellus P, Dufour H, Levrier O et al (2002) Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations. Neurosurgery 51:500–503; discussion 503–504CrossRefPubMedGoogle Scholar
  37. Mikulis DJ, Diaz O, Egglin TK et al (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728CrossRefPubMedGoogle Scholar
  38. Milhorat TH, Chou MW, Trinidad EM et al (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017CrossRefPubMedGoogle Scholar
  39. Mohanty A, Anandh B, Kolluri VR et al (1999) Neuroendoscopic third ventriculostomy in the management of fourth ventricular outlet obstruction. Minim Invasive Neurosurg 42:18–21CrossRefPubMedGoogle Scholar
  40. Naidich TP, Pudlowski RM, Naidich JB et al (1980) Computed tomographic signs of the Chiari II malformation. Part I: skull and dural partitions. Radiology 134:65–71CrossRefPubMedGoogle Scholar
  41. Newman PK, Terenty TR, Foster JB (1981) Some observations on the pathogenesis of syringomyelia. J Neurol Neurosurg Psychiatry 44:964–969CrossRefPubMedPubMedCentralGoogle Scholar
  42. Nicolaides KH, Campbell S, Gabbe SG et al (1986) Ultrasound screening for spina bifida: cranial and cerebellar signs. Lancet 2:72–74CrossRefPubMedGoogle Scholar
  43. Nishikawa M, Sakamoto H, Hakuba A et al (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47CrossRefPubMedGoogle Scholar
  44. Oakes WJ, Tubbs RS (2004) Management of the Chiari malformation and spinal dysraphism. Clin Neurosurg 51:48–52PubMedGoogle Scholar
  45. Oi S, Hidaka M, Honda Y et al (1999) Neuroendoscopic surgery for specific forms of hydrocephalus. Childs Nerv Syst 15:56–68CrossRefPubMedGoogle Scholar
  46. Oldfield EH, Muraszko K, Shawker TH et al (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15CrossRefPubMedGoogle Scholar
  47. Olivero WC, Rekate HL, Chizeck HJ et al (1988) Relationship between intracranial and sagittal sinus pressure in normal and hydrocephalic dogs. Pediatr Neurosci 14:196–201CrossRefPubMedGoogle Scholar
  48. Park JK, Gleason PL, Madsen JR et al (1997) Presentation and management of Chiari I malformation in children. Pediatr Neurosurg 26:190–196CrossRefPubMedGoogle Scholar
  49. Rauzzino M, Oakes WJ (1995) Chiari II malformation and syringomyelia. Neurosurg Clin N Am 6:293–309PubMedCrossRefGoogle Scholar
  50. Rekate HL (1985) To shunt or not to shunt: hydrocephalus and dysraphism. Clin Neurosurg 32:593–607PubMedGoogle Scholar
  51. Smith BW, Strahle J, Bapuraj JR et al (2013) Distribution of cerebellar tonsil position: implications for understanding Chiari malformation. J Neurosurg 119:812–819CrossRefPubMedGoogle Scholar
  52. Steinbok P, Hall J, Flodmark O (1989) Hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension. J Neurosurg 71:42–48CrossRefPubMedGoogle Scholar
  53. Stevenson KL (2004) Chiari type II malformation: past, present, and future. Neurosurg Focus 16:E5CrossRefPubMedGoogle Scholar
  54. Suehiro T, Inamura T, Natori Y et al (2000) Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report. J Neurosurg 93:326–329CrossRefPubMedGoogle Scholar
  55. Taylor WJ, Hayward RD, Lasjaunias P et al (2001) Enigma of raised intracranial pressure in patients with complex craniosynostosis: the role of abnormal intracranial venous drainage. J Neurosurg 94:377–385CrossRefPubMedGoogle Scholar
  56. Teo C, Jones R (1996) Management of hydrocephalus by endoscopic third ventriculostomy in patients with myelomeningocele. Pediatr Neurosurg 25:57–63; discussion 63CrossRefPubMedGoogle Scholar
  57. Tubbs RS, Oakes WJ (2004) Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:375–381CrossRefPubMedGoogle Scholar
  58. Tubbs RS, Elton S, Grabb P et al (2001) Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurgery 48:1050–1054; discussion 1054–1055PubMedGoogle Scholar
  59. Tubbs RS, Smyth MD, Wellons JC 3rd et al (2004) Arachnoid veils and the Chiari I malformation. J Neurosurg 100:465–467PubMedGoogle Scholar
  60. Tubbs RS, Beckman J, Naftel RP et al (2011) Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr 7:248–256CrossRefPubMedGoogle Scholar
  61. Tulipan N, Wellons JC 3rd, Thom EA et al (2015) Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr 16:613–620CrossRefPubMedPubMedCentralGoogle Scholar
  62. Venes JL, Black KL, Latack JT (1986) Preoperative evaluation and surgical management of the Arnold-Chiari II malformation. J Neurosurg 64:363–370CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryUniversity of Iowa Hospitals and ClinicsIowa CityUSA
  2. 2.Pappajohn Biomedical InstituteUniversity of Iowa Carver College of MedicineIowa CityUSA

Section editors and affiliations

  • Giuseppe Cinalli
    • 1
  1. 1.Department of Pediatric NeurosurgerySantobono-Pausilipon Children’s HospitalNaplesItaly

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