Chiari Malformations Types II, III, IV, and V

  • Luca MassimiEmail author
  • Massimo Caldarelli
  • Concezio Di Rocco
Living reference work entry


This chapter is addressed to the description of the forms of caudal herniation through the foramen magnum different from Chiari type I malformation, which is largely the most common variant of hindbrain herniation. Among the here-illustrated conditions, Chiari type II malformation is the most frequently encountered in the clinical practice, following the epidemiological distribution of the open spina bifida to which it is strictly related. Chiari II malformation provides diagnostic and management challenges because it is not a single malformation but a constellation of malformations involving either the neural (brain, cerebellum, spinal cord) and the bony structures (skull, spine). The clinical picture can vary significantly according to the age at onset and the severity of the brainstem dysfunction. On the other hand, the other variants are very rare, like Chiari type III malformation, or exceptional and nosographically still debated, like Chiari types IV and V malformations. These rare forms are quite often burdened by a dismal prognosis.


  1. Aaronson OS, Hernanz-Schulman N, Bruner JP, Reed GW, Tulipan NB (2003) Myelomeningocele: prenatal evaluation – comparison between transabdominal US and MR imaging. Radiology 227:839–843PubMedCrossRefPubMedCentralGoogle Scholar
  2. Abd-El-Barr MM, Strong CI, Groff MW (2014) Chiari malformations: diagnosis, treatments and failures. J Neurosurg Sci 58:215–221PubMedPubMedCentralGoogle Scholar
  3. Abele TA, Lee SL, Twickler DM (2013) MR imaging quantitative analysis of fetal Chiari II malformations and associated open neural tube defects: balanced SSFP versus half-Fourier RARE and interobserver reliability. J Magn Reson Imaging 38:786–793PubMedCrossRefPubMedCentralGoogle Scholar
  4. Adzick NS (2013) Fetal surgery for spina bifida: past, present, future. Semin Pediatr Surg 22:10–17PubMedCrossRefPubMedCentralGoogle Scholar
  5. Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D'Alton ME, Farmer DL, Investigators MOMS (2011) A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 364:993–1004PubMedCrossRefPubMedCentralGoogle Scholar
  6. Agrawal A, Mittal A, Kohali GB, Sampley S, Gupta A (2011) Chiari III malformation. Pediatr Neurosurg 47:309–310PubMedCrossRefPubMedCentralGoogle Scholar
  7. Aguilera S, Soothill P, Denbow M, Pople I (2009) Prognosis of spina bifida in the era of prenatal diagnosis and termination of pregnancy. Fetal Diagn Ther 26:68–74PubMedCrossRefPubMedCentralGoogle Scholar
  8. Akbari SH, Limbrick DD Jr, Kim DH, Narayan P, Leonard JR, Smyth MD, Park TS (2013) Surgical management of symptomatic Chiari II malformation in infants and children. Childs Nerv Syst 29:1143–1154PubMedCrossRefPubMedCentralGoogle Scholar
  9. Alkan O, Kizilkilic O, Yildirim T (2009) Malformations of the midbrain and hindbrain: a retrospective study and review of the literature. Cerebellum 8:355–365PubMedCrossRefPubMedCentralGoogle Scholar
  10. Alsaadi MM, Iqbal SM, Elgamal EA, Gozal D (2012) Sleep-disordered breathing in children with Chiari malformation type II and myelomeningocele. Pediatr Int 54:623–626PubMedCrossRefPubMedCentralGoogle Scholar
  11. Aribal ME, Gurcan F, Aslan B (1996) Chiari III malformation: MRI. Neuroradiology 38:S184–S186PubMedCrossRefPubMedCentralGoogle Scholar
  12. Arnold J (1894) Myelocyste, Transposition von Gewebskeimen und Sympodie. Beitr Path Anat 16:1–28Google Scholar
  13. Atlas SW (2009) Magnetic resonance imaging of the brain and spine, vol. 2. Lippincott Williams & Wilkins, Philadelphia, p 233Google Scholar
  14. Awad A-W, Aleck KA, Bhardwaj RD (2014) Concomitant achondroplasia and Chiari malformation: a double-hit at the cervicomedullary junction. World J Clin Cases 2:711–716PubMedCrossRefPubMedCentralGoogle Scholar
  15. Bakim B, Goksan YB, Yilmaz A, Karamustafalioglu O, Akbiyik M, Yayla S, Yuce I, Alpak G, Tankaya O (2013) The quality of life and psychiatric morbidity in patients operated for Arnold- Chiari malformation type I. Int J Psychiatry Clin Pract 17:259–263PubMedCrossRefPubMedCentralGoogle Scholar
  16. Barf HA, Verhoef M, Jennekens-Schinkel A, Post MW, Gooskens RH, Prevo AJ (2003) Cognitive status of young adults with spina bifida. Dev Med Child Neurol 45:813–820PubMedCrossRefPubMedCentralGoogle Scholar
  17. Barry A, Bm P, Stewart BH (1957) Possible factors in the development of the Arnold Chiari malformation. J Neurosurg 14:285–301PubMedCrossRefPubMedCentralGoogle Scholar
  18. Bianchi F, Frassanito P, Tamburrini G, Caldarelli M, Massimi L (2016) Shunt malfunction mimicking a cystic tumour. Br J Neurosurg 6:1–3Google Scholar
  19. Boltshauser E, Schneider J, Kollias S, Waibel P, Weissert M (2002) Vanishing cerebellum in myelomeningocele. Eur J Paediatr Neurol 6:109–113PubMedCrossRefPubMedCentralGoogle Scholar
  20. Bouchard S, Davey MG, Rintoul NE (2003) Correction of hindbrain herniation and anatomy of the vermis after in utero repair of myelomeningocele in sheep. J Pediatr Surg 38:451–458PubMedCrossRefPubMedCentralGoogle Scholar
  21. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA (2001) Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34:114–120PubMedCrossRefPubMedCentralGoogle Scholar
  22. Brownlee R, Myles T, Hamilton MG (1997) The Chiari III and IV malformations. In: Anson JA, Benzel EC, Awad IA (eds) Syringomyelia and the Chiari malformations. AANS, Park Ridge, pp 88–89Google Scholar
  23. Bruner JP, Tulipan N, Paschall R, 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 282:1819–1825PubMedCrossRefPubMedCentralGoogle Scholar
  24. Bruner JP, Tulipan N, Drabowiak ME, Walsh DS, Rorke LB, Adzick NS (2004) Upper level of the spina bifida defect: how good are we? Ultrasound Obstet Gynecol 24:612–617PubMedCrossRefPubMedCentralGoogle Scholar
  25. Bulut MD, Yavuz A, Bora A, Gulsen I, Ozkacmaz S, Sosuncu E (2013) Chiari III malformation with a giant encephalocele sac: case report and a review of the literature. Pediatr Neurosurg 49:316–319PubMedCrossRefPubMedCentralGoogle Scholar
  26. Cakirer S (2003) Chiari III malformation varieties of MRI appearances in two patients. J Clin Imag 27:1–4CrossRefGoogle Scholar
  27. Caldarelli M, Di Rocco C (2010) Chiari malformations. In: Lumenta CB, Di Rocco C, Haase J, Mooij JJA (eds) Neurosurgery. Springer, Berlin/Heidelberg, pp 311–314Google Scholar
  28. Caldarelli M, Di Rocco C, Colosimo C Jr, Fariello G, Di Gennaro M (1995) Surgical treatment of late neurological deterioration in children with myelodysplasia. Acta Neurochir 137:199–206PubMedCrossRefPubMedCentralGoogle Scholar
  29. Caldarelli M, Di Rocco C, La Marca F (1998) Treatment of hydromyelia in spina bifida. Surg Neurol 50:411–420PubMedCrossRefPubMedCentralGoogle Scholar
  30. Caldarelli M, Rea G, Cincu R, Di Rocco C (2002) Chiari type III malformation. Childs Nerv Syst 18:207–210PubMedCrossRefPubMedCentralGoogle Scholar
  31. Caldarelli M, Boscarelli A, Massimi L (2013) Recurrent tethered cord: radiological investigation and management. Childs Nerv Syst 29:1601–1609PubMedCrossRefPubMedCentralGoogle Scholar
  32. Callen AL, Filly RA (2008) Supratentorial abnormalities in the Chiari II malformation. I – the ventricular point. J Ultrasound Med 27:33–38PubMedCrossRefPubMedCentralGoogle Scholar
  33. Callen AL, Stengel JW, Filly RA (2009) Supratentorial abnormalities in the Chiari II malformation. II – Tectal morphological changes. J Ultrasound Med 28:29–35PubMedCrossRefPubMedCentralGoogle Scholar
  34. Cama A, Tortori-Donati P, Piatelli GL, Fondelli MP, Andreussi L (1995) Chiari complex in children. Neuroradiological diagnosis, neurosurgical treatment and proposal of a new classification (312 cases). Eur J Pediatr Surg 5(Suppl 1):35–38PubMedCrossRefPubMedCentralGoogle Scholar
  35. Cameron AH (1957) The Arnold-Chiari and other neuroanatomical malformations associated with spina bifida. J Pathol 73:195–211CrossRefGoogle Scholar
  36. Campbell J, Gilbert WM, Nicolaides KH, Campbell S (1987) Ultrasound screening for spina bifida: cranial and cerebellar signs in a high-risk population. Obstet Gynecol 70:247–250PubMedPubMedCentralGoogle Scholar
  37. Castillo M, Quencer RM, Dominguez R (1992) Chiari III malformation: imaging features. Am J Neuroradiol 13:107–113PubMedPubMedCentralGoogle Scholar
  38. Center for Disease Control and Prevention-(1992) (1995) Economic costs of birth defects and cerebral palsy – United States. MMWR 44:694–699Google Scholar
  39. Cesmebasi A, Loukas M, Hogan E, Kralovic S, Tubbs RS, Cohen-Gadol AA (2015) The Chiari malformations: a review with emphasis on anatomical traits. Clin Anat 28:184–194PubMedCrossRefPubMedCentralGoogle Scholar
  40. Chakraborty A, Crimmins D, Hayward R, Thompson D (2008) Toward reducing shunt placement rates in patients with myelomeningocele. J Neurosurg Pediatr 1:361–365PubMedCrossRefPubMedCentralGoogle Scholar
  41. Chambers CD, Johnson KA, Dick LM, Felix RJ, Jones KL (1998) Maternal fever and birth outcome: a prospective study. Teratology 58:251–257PubMedCrossRefPubMedCentralGoogle Scholar
  42. Chaudhari IM, Karas CS, Baig MN, Elton SW (2008) Unique defect representing features of Chiari type III and IV malformations. Pediatr Neurosurg 44:513–514PubMedCrossRefPubMedCentralGoogle Scholar
  43. Chiari H (1891) Uber Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wschr 17:1172–1175CrossRefGoogle Scholar
  44. Chiari H (1896) Uber die Veränderungen des Kleinhirns, der Pons und der Medulla oblongata in Folge von congenitaler Hydrocephalie des Grosshirns. Denkschr Akad Wissensch Math Nature 63:71–116Google Scholar
  45. Chikkannaiah P, Srinivasamurthy V, Satish Prasad BS, Lalyanayak P, Shivaram DN (2014) Iniencephaly: radiological and pathological features of a series of three cases. J Neurosci Rural Pract 5:389–393PubMedCrossRefPubMedCentralGoogle Scholar
  46. Cleland J (1883) Contribution to the study of spina bifida, encephalocele, and anencephalus. J Anat Physiol 17:257–292PubMedPubMedCentralGoogle Scholar
  47. Coniglio SJ, Anderson SM, Ferguson JE (1997) Developmental outcomes of children with myelomeningocele: prenatal predictors. Am J Obstet Gynecol 177:319–324PubMedCrossRefPubMedCentralGoogle Scholar
  48. De Chalain T, Hudgins R, Burstein F, Armfield K (1994) Combination of unilateral polydactyly, syndactyly, and clinodactyly with occipitocervical encephalocele and vertebral fusion. Ann Plast Surg 44:86–89CrossRefGoogle Scholar
  49. De Reuck J, Thienpont L (1976) Fetal Chiari’s type III malformation. Childs Brain 2:85–91PubMedPubMedCentralGoogle Scholar
  50. Del Casale A, Serata D, Rapinesi C, Simonetti A, Tamorri SM, Comparelli A, De Carolis A, Savoja V, Kotzalidis GD, Sani G, Tatarelli R, Girardi P (2012) Psychosis risk syndrome comorbid with panic attack disorder in a cannabis abusing patient affected by Arnold-Chiari malformation type I. Gen Hosp Psychiatry 34:702–707PubMedPubMedCentralGoogle Scholar
  51. Dhandapani S, Srinivasan A (2016) Contiguous triple spinal dysraphism associated with Chiari malformation type II and hydrocephalus: an embryological conundrum between the unified theory of pang and the unified theory of McLone. J Neurosurg Pediatr 17:103–106PubMedCrossRefPubMedCentralGoogle Scholar
  52. Di Rocco C, Rende M (1989) Neural tube defects: considerations on the pathogenesis and correlation with myelodysplasia in man. II. An experimental model. Fetal Ther 4:99–103PubMedCrossRefPubMedCentralGoogle Scholar
  53. Dias MS (1999) Myelomeningocele. In: Choux M, Di Rocco C, Hockley A, Walker M (eds) Pediatric neurosurgery. Churchill Livingstone, London, pp 33–59Google Scholar
  54. Dias MS (2005) Neurosurgical causes of scoliosis in patients with myelomeningocele: an evidence-based literature review. J Neurosurg 103(1 Suppl):24–35PubMedPubMedCentralGoogle Scholar
  55. Dionigi B, Ahmed A, Brazzo J 3rd, Connors JP, Zurakowski D, Fauza DO (2015a) Partial or complete coverage of experimental spina bifida by simple intra-amniotic injection of concentrated amniotic mesenchymal stem cells. J Pediatr Surg 50:69–73PubMedCrossRefPubMedCentralGoogle Scholar
  56. Dionigi B, Brazzo JA III, Ahmed A, Feng C, Wu Y, Zurakowski D, Fauza DO (2015b) Trans-amniotic stem cells therapy (TRSCET) minimizes Chiari II malformation in experimental spina bifida. J Pediatr Surg 50:1037–1041PubMedCrossRefPubMedCentralGoogle Scholar
  57. Drake JM (2008) The surgical management of pediatric hydrocephalus. Neurosurgery 62(Suppl 2):633–640PubMedCrossRefPubMedCentralGoogle Scholar
  58. Elster AD, Chen MY (1992) Chiari I malformations: clinical and radiological reappraisal. Radiology 183:347–353CrossRefGoogle Scholar
  59. Emery JL, MacKenzie N (1973) Medullo-cervical dislocation deformity (Chiari II deformity) related to neurospinal dysraphism (myelomeningocele). Brain 96:155–162PubMedCrossRefPubMedCentralGoogle Scholar
  60. Erdincler P, Kaynar MY, Canbaz B, Kocer N, Kuday C, Çiplak N (1998) Iniencephaly: neuroradiological and surgical features. J Neurosurg 89:317–320PubMedCrossRefPubMedCentralGoogle Scholar
  61. Erol FS, Ucler N, Yakar H (2011) The association of Chiari type III malformation and Klippel-Feil syndrome with mirror movement: a case report. Turk Neurosurg 21:655–658PubMedPubMedCentralGoogle Scholar
  62. Filho PSAH, Pratesi R (2009) A possible cause of attention deficit in children and adolescents with Chiari malformation type II. Arq Neuropsiquiatr 67:29–34CrossRefGoogle Scholar
  63. Filly MR, Filly RA, Barkovich AJ, Goldstein RB (2010) Supratentorial abnormalities in the Chiari II malformation. IV – the too-far-back ventricle. J Ultrasound Med 29:243–248PubMedCrossRefPubMedCentralGoogle Scholar
  64. Fujii M, Tomita T, McLone DG, Grant JA, Stack CV, Mori K (1997) Developmental normo-maturation of brainstem auditory evoked potentials in children with asymptomatic meningo-myelocele during the first year of life. Childs Nerv Syst 13:147–153PubMedCrossRefPubMedCentralGoogle Scholar
  65. Furtado SV, Anantharam BA, Reddy K, Hegde A (2009) Repair of Chiari III malformation using cranioplasty and occipital rotation flap: technical note and review of literature. Surg Neurol 72:414–417PubMedCrossRefPubMedCentralGoogle Scholar
  66. Ganesh D, Sagayaraj BM, Barua RK, Sharma N, Ranga U (2014) Arnold Chiari malformation with spina bifida: a lost opportunity of folic acid supplementation. J Clin Diagn Res 8:OD01–OD03PubMedPubMedCentralGoogle Scholar
  67. Gardner WJ (1965) Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 28:247–259PubMedCrossRefPubMedCentralGoogle Scholar
  68. Garg K, Malik N, Jaiswal AK, Behari S (2008) Chiari III malformation with hypertelorism and microcephaly in a neonate: case report and review of literature. J Pediatr Neurosci 3:169–171CrossRefGoogle Scholar
  69. Garg K, Tandon V, Mahapatra AK (2011) Chiari III malformation with proatlas abnormality. Pediatr Neurosurg 47:295–298PubMedCrossRefPubMedCentralGoogle Scholar
  70. Garne E, Loane M, Dolk H, De Vigan C, Scarano G, Tucker D, Stoll C, Gener B, Pierini A, Nelen V, Rösch C, Gillerot Y, Feijoo M, Tincheva R, Queisser-Luft A, Addor MC, Mosquera C, Gatt M, Barisic I (2005) Prenatal diagnosis of severe structural congenital malformations in Europe. Ultrasound Obstet Gynecol 25:6–11PubMedCrossRefPubMedCentralGoogle Scholar
  71. Geerdink N, van der Vliet T, Rotteveel JJ, Feuth T, Roeleveld N, Mullaart RA (2012) Essential features of Chiari II malformation in MR imaging: an interobserver reliability study – part 1. Childs Nerv Syst 28:977–985PubMedCrossRefPubMedCentralGoogle Scholar
  72. Geiger F, Parsch D, Carstens C (1999) Complications of scoliosis surgery in children with myelomeningocele. Eur Spine J 8:22–26PubMedCrossRefPubMedCentralGoogle Scholar
  73. Ghi T, Pilu GL, Falco P, Segata M, Carletti A, Cocchi G, Santini D, Bonasoni P, Tani G, Rizzo N (2006) Prenatal diagnosis of open and closed spina bifida. Ultrasound Obstet Gynecol 28:899–903PubMedCrossRefPubMedCentralGoogle Scholar
  74. Gilbert JN, Jones KL, Rorke LB, Chernoff GF, James HE (1986) Central nervous system anomalies associated with meningomyelocele, hydrocephalus, and the Arnold-Chiari malformations: reappraisal of thesis regarding the pathogenesis of posterior neural tube closure defects. Neurosurgery 18:559–563PubMedCrossRefPubMedCentralGoogle Scholar
  75. Guille JT, Starwark JF, Sherk HH, Kumar SJ (2006) Congenital and developmental deformities of the spine in children with myelomeningocele. J Am Acad Orthop Surg 14:294–302PubMedCrossRefPubMedCentralGoogle Scholar
  76. Hall P, Lindseth R, Campbell R, Kalsbeck JE, Desousa A (1979) Scoliosis and hydrocephalus in myelocele patients. The effects of ventricular shunting. J Neurosurg 50:174–178PubMedCrossRefPubMedCentralGoogle Scholar
  77. Hashiguchi K, Morioka T, Murakami N, Togao O, Hiwatashi A, Ochiai M, Eriguchi G, Kishimoto J, Iihara K (2016) Sequential morphological change of Chiari malformation type II following surgical repair of myelomeningocele. Childs Nerv Syst 32:1069–1078PubMedCrossRefPubMedCentralGoogle Scholar
  78. Hoederath L, Jellestad L, Jenewein J, Boettger S (2014) Psychotic and major neurocognitive disorder secondary to Arnold-Chiari type II malformation. Psychiatr Danub 26:291–293PubMedPubMedCentralGoogle Scholar
  79. Hoffman HJ, Hendrick EB, Humphreys RP (1975) Manifestations and management of the Arnold-Chiari malformation in patients with myelomeningocele. Childs Brain 1:255–259PubMedPubMedCentralGoogle Scholar
  80. Hoffman HJ, Hendrick EB, Humphreys RP (1983) Manifestazioni e trattamento della malformazione di Arnold- Chiari nel bambino con mielomeningocele. In: Di Rocco C, Caldarelli M (eds) Mielomeningocele. Casa del Libro, Rome, pp 251–260Google Scholar
  81. Hol FA, Geurds MP, Chatkupt S et al (1996) PAX genes and human neural tube defects: an amino acid substitution in PAX 1 in a patient with spina bifida. J Med Gen 33:655–660CrossRefGoogle Scholar
  82. Isik N, Elmaci I, Silva G, Celik M, Kalelioglu M (2009) Chiari malformation type III and results of surgery: a clinical study. Pediatr Neurosurg 45:19–28PubMedCrossRefPubMedCentralGoogle Scholar
  83. Iskandar BJ, Oakes WJ (1999) Chiari malformations. In: Albright L, Pollack I, Adelson D (eds) Principles and practice of pediatric neurosurgery. Thieme, New York, pp 165–187Google Scholar
  84. Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ (1998) The resolution of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 89:212–216PubMedCrossRefPubMedCentralGoogle Scholar
  85. Ivashchuk G, Loukas M, Blount JP, Tubbs RS, Oakes WJ (2015) Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst 31:2035–2040PubMedCrossRefPubMedCentralGoogle Scholar
  86. Jaggi RS, Premsagar IC (2007) Chiari malformation type III treated with primary closure. Pediatr Neurosurg 43:424–427PubMedCrossRefPubMedCentralGoogle Scholar
  87. Januschek E, Röhrig A, Kunze S, Fremerey C, Wiebe B, Messing-Jünger M (2016) Myelomeningocele – a single institute analysis of the years 2007 to 2015. Childs Nerv Syst 32(7):1281–1287. Epub 2016 Apr 16CrossRefPubMedPubMedCentralGoogle Scholar
  88. Jenkinson MD, Campbell S, Hayhurst C, Clark S, Kandasamy J, Lee MK, Flynn A, Murphy P, Mallucci CL (2011) Cognitive and functional outcome in spina bifida-Chiari II malformation. Childs Nerv Syst 27:967–974PubMedCrossRefPubMedCentralGoogle Scholar
  89. Jiang J, Zhang Y, Wei L, Sun Z, Liu Z (2014) Association between MTHDI G1958A polymorphism and neural tube defects susceptibility: a meta-analysis. PLoS One 9:e1011169Google Scholar
  90. Josan V, Morokoff MWJ (2008) Epidemiology and aetiological factors. In: Ozek MM, Cinalli G, Maixner WJ (eds) Spina bifida. Springer, Milan, pp 59–65CrossRefGoogle Scholar
  91. Juranek J, Dennis M, Cirino PT, El-Messidi L, Fletcher JM (2010) The cerebellum in children with spina bifida and Chiari II malformation: quantitative volumetrics by region. Cerebellum 9:240–248PubMedCrossRefPubMedCentralGoogle Scholar
  92. Kiymaz N, Yilmaz N, Demir I, Keskin S (2010) Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg 46:6–11PubMedCrossRefPubMedCentralGoogle Scholar
  93. Kulkarni PR, Rao RV, Alur MB, Joshi SK (2011) Iniencephaly clausus: a case report with review of literature. J Pediatr Neurosci 6:121–123PubMedPubMedCentralGoogle Scholar
  94. La Marca F, Herman R, Grant JA, McLone DG (1997) Presentation and management of hydromyelia in children with Chiari type II malformation. Pediatr Neurosurg 26:57–67PubMedCrossRefPubMedCentralGoogle Scholar
  95. Lee R, Tay KS, Cheng PW, Lui WM, Chan FL (2002) Chiari III malformation: antenatal MRI diagnosis. Clin Radiol 57:759–767PubMedCrossRefPubMedCentralGoogle Scholar
  96. Luigetti M, Losurdo A, Dittoni S, Testani E, Colicchio S, Gnoni V, Farina B, Scarano E, Zampino G, Mariotti P, Rendeli C, Di Rocco C, Massimi L, Della Marca G (2010) Improvement of obstructive sleep apneas caused by hydrocephalus associated with Chiari malformation type II following surgery. J Neurosurg Pediatrics 6:336–339CrossRefGoogle Scholar
  97. Mangels KJ, Tulipan N, Tsao LY (2000) Fetal MRI in the evaluation of intrauterine myelomeningocele. Pediatr Neurosurg 32:124–131PubMedCrossRefPubMedCentralGoogle Scholar
  98. Marin-Padilla M, Marin-Padilla TM (1981) Morphogenesis of experimentally induced Arnold-Chiari malformation. J Neurol Sci 50:29–55PubMedCrossRefPubMedCentralGoogle Scholar
  99. Massimi L, Novegno F, Di Rocco C (2011a) Chiari type I malformation in children. Advances and Technical Standards in Neurosurgery, Springer, Wien New York 37:143–211CrossRefGoogle Scholar
  100. Massimi L, Pravatà E, Tamburrini G, Gaudino S, Pettorini B, Novegno F, Colosimo C Jr, Di Rocco C (2011b) Management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery 68:950–956PubMedCrossRefPubMedCentralGoogle Scholar
  101. Mc Lone DG (2011) Comment on Salman M: posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study. Childs Nerv Syst 27:465PubMedCrossRefPubMedCentralGoogle Scholar
  102. McLone DG (1992) Continuing concepts in the management of spina bifida. Pediatr Neurosurg 18:254–256PubMedCrossRefPubMedCentralGoogle Scholar
  103. McLone DG (1998) Care of the neonate with myelomeningocele. Neurosurg Clin N Am 9:111–120PubMedCrossRefPubMedCentralGoogle Scholar
  104. McLone DG, Dias MS (2003) The Chiari II malformation: cause and impact. Childs Nerv Syst 19:540–550PubMedCrossRefPubMedCentralGoogle Scholar
  105. McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12PubMedCrossRefPubMedCentralGoogle Scholar
  106. McLone DG, Naidich TP (1992) Developmental morphology of the subarachnoid space, brain vasculature and contiguous structures, and the cause of the Chiari II malformation. Am J Neuroradiol 13:463–482PubMedPubMedCentralGoogle Scholar
  107. Mehta VA, Bettegowda C, Amin A, El-Gassim M, Jallo G, Ahn ES (2011) Impact of tethered cord release on symptoms of Chiari II malformation in children born with a myelomeningocele. Childs Nerv Syst 27:975–978PubMedCrossRefPubMedCentralGoogle Scholar
  108. Meijer WM, de Walle HE (2005) Differences in folic acid policy and the prevalence of neural-tube defects in Europe; recommendations for food fortification in a EUROCAT report. Ned Tijdschr Genneeskd 149:2561–2564 (article in Dutch)Google Scholar
  109. Melvin EC, George TM, Worley G, Franklin A, Mackey J, Viles K, Shah N, Drake CR, Enterline DS, McLone D, Nye J, Oakes WJ, McLaughlin C, Walker ML, Peterson P, Brei T, Buran C, Aben J, Ohm B, Bermans I, Qumsiyeh M, Vance J, Pericak-Vance MA, Speer MC (2000) Genetic studies in neural tube defects. NTD collaborative group. Pediatr Neurosurg 32:1–9PubMedCrossRefPubMedCentralGoogle Scholar
  110. Messing-Jünger M, Röhrig A (2013) Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 29:1553–1562PubMedCrossRefPubMedCentralGoogle Scholar
  111. Meuli M, Moehrlen U (2014) Fetal surgery for myelomeningocele is effective: a critical look at the whys. Pediatr Surg Int 30:689–697PubMedCrossRefPubMedCentralGoogle Scholar
  112. Mignone Philpott C, Shannon P, Chitayat D, Ryan G, Raybaud CA, Blaser CI (2013) Diffusion-weighted imaging of the cerebellum in the fetus with Chiari II malformation. Am J Neuroradiol 34:1656–1660PubMedCrossRefPubMedCentralGoogle Scholar
  113. Mikulis DJ, Diaz O, Egglin TK, Sanchez R (1992) Variance of the position of the cerebellar tonsils with age: preliminary report. Radiology 183:725–728PubMedCrossRefPubMedCentralGoogle Scholar
  114. Milhorat TH, Miller JI, Johnson WD, Adler DE, Heger IM (1993) Anatomical basis of syringomyelia occurring with hindbrain lesions. Neurosurgery 32:748–754PubMedCrossRefPubMedCentralGoogle Scholar
  115. Mirzai H, Ersahin Y, Mutluer S, Kayahan A (1998) Outcome of patients with myelomeningocele: the Ege University experience. Childs Nerv Syst 14:120–123PubMedCrossRefPubMedCentralGoogle Scholar
  116. MRC Vitamin Study Research Group (1991) Prevention of neural tube defects: results of the Medical Research Council vitamin study. Lancet 338:131–137CrossRefGoogle Scholar
  117. Muzumdar D, Gandhi S, Fattepurkar S, Goel A (2007) Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg 43:446–448PubMedCrossRefPubMedCentralGoogle Scholar
  118. Naidich TP, Pudlowski RM, Naidich JB, Gornish M, Rodriguez FJ (1980) Computed tomography signs of the Chiari II malformation. Part I: skull and dural positions. Radiology 134:65–71PubMedCrossRefPubMedCentralGoogle Scholar
  119. Nishimura T, Mori K (1996) Blink reflex in meningomyelocele, with special reference to its usefulness in the evaluation of brainstem dysfunction. Childs Nerv Syst 12:2–12PubMedCrossRefPubMedCentralGoogle Scholar
  120. Nishimura T, Mori K, Uchida Y, Ohira T, Tamura K (1991) Brain stem auditory-evoked potentials in meningomyelocele. Natural history of Chiari II malformations. Childs Nerv Syst 7:316–326PubMedCrossRefPubMedCentralGoogle Scholar
  121. Oakeshott P, Hunt GM (2003) Long-term outcome in open spina bifida. Br J Gen Pract 53:632–636PubMedPubMedCentralGoogle Scholar
  122. O'Brien DF, Javadpour M, Collins DR, Spennato P, Mallucci CL (2005) Endoscopic third ventriculostomy: an outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction. J Neurosurg 103(5 Suppl):393–400PubMedPubMedCentralGoogle Scholar
  123. Ogiwara H, Morota N (2013) Surgical decompression without dural opening for symptomatic Chiari type II malformation in young infants. Childs Nerv Syst 29:1563–1567PubMedCrossRefPubMedCentralGoogle Scholar
  124. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15PubMedCrossRefPubMedCentralGoogle Scholar
  125. Osaka K, Tanimura T, Hirayama A, Matsumoto S (1978) Myelomeningocele before birth. J Neurosurg 49:711–724PubMedCrossRefPubMedCentralGoogle Scholar
  126. Otera Y, Morokuma S, Fukushima K, Anami A, Yumoto Y, Ito Y, Ochiai M, Hashiguchi K, Wake N, Sago H, Kato K (2014) Neurological outcomes in Chiari type II malformations and their correlation to morphological findings and fetal heart rate patterns: a retrospective study. BMC Res Notes 8:57CrossRefGoogle Scholar
  127. Paek BW, Farmer DL, Wilkinson CC, Albanese CT, Peacock W, Harrison MR, Jennings RW (2000) Hindbrain herniation develops in surgically created myelomeningocele but is absent after repair in fetal lambs. Am J Obstet Gynecol 183:1119–1123PubMedCrossRefPubMedCentralGoogle Scholar
  128. Papp C, Adam Z, Toth-Pal E, Török O, Váradi V, Papp Z (1997) Risk of recurrence of craniospinal anomalies. J Matern Fetal Med 6:53–57PubMedPubMedCentralGoogle Scholar
  129. Patel DM, Rocque BG, Hopson B, Arynchyna A, Bishop ER, Lozano D, Blount JP (2015) Sleep-disordered breathing in patients with myelomeningocele. J Neurosurg Pediatr 16:30–35PubMedCrossRefPubMedCentralGoogle Scholar
  130. Penfield W, Coburn DF (1938) Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiatr 40:328–336CrossRefGoogle Scholar
  131. Penso C, Redline RW, Benacerraf BR (1987) A sonographic sign which predicts which fetuses with hydrocephalus have an associated neural tube defect. J Ultrasound Med 6:307–311PubMedCrossRefPubMedCentralGoogle Scholar
  132. Perez da Rosa S, Millward CP, Chiappa V, Martinez de Leon M, Ibáñez Botella G, Ros López B (2015) Endoscopic third Ventriculostomy in children with myelomeningocele: a case series. Pediatr Neurosurg 50:113–118PubMedCrossRefPubMedCentralGoogle Scholar
  133. Piatt JH Jr (2004) Syringomyelia complicating myelomeningocele: review of the evidence. J Neurosurg 100(2 Suppl Pediatrics):101–109PubMedPubMedCentralGoogle Scholar
  134. Pitkin RM (2007) Folate and neural tube defects. Am J Clin Nutr 85:285S–288SPubMedCrossRefPubMedCentralGoogle Scholar
  135. Pollack IF, Pang AD, Albright AL, Krieger D (1992) Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77:881–888PubMedCrossRefPubMedCentralGoogle Scholar
  136. Pollack IF, Kinnunen D, Albright LA (1996) The effect of early craniocervical decompression on functional outcome in neonates and young infants with myelodysplasia and symptomatic Chiari II malformations: results from a prospective series. Neurosurgery 38:703–710PubMedCrossRefPubMedCentralGoogle Scholar
  137. Raimondi AJ (1972) Pediatric neurosurgery. Saunders, Philadelphia, pp 235–237, 339–343Google Scholar
  138. Ramdurg SR, Solpure S, Dubey S, Gubbi S (2013) Asymptomatic Chiari III malformation with tectal beaking and holocord syrinx. J Pediatr Neurosci 8:254–256PubMedCrossRefPubMedCentralGoogle Scholar
  139. Rani H, Kulkarni AV, Rao RV, Patil P (2013) Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol 32:169–174PubMedCrossRefPubMedCentralGoogle Scholar
  140. Ray JG, Meier C, Vermeulen MJ, Boss S, Wyatt PR, Cole DE (2002) Association of neural tube defects and folic acid fortification in Canada. Lancet 360:2047–2048PubMedCrossRefPubMedCentralGoogle Scholar
  141. Raybaud C, Miller E (2008) Radiological evaluation of myelomeningocele-Chiari II malformation. In: Ozek MM, Cinalli G, Maixner WJ (eds) Spina bifida. Springer Verlag, Milan, pp 111–142CrossRefGoogle Scholar
  142. Righini A, Parazzini C, Doneda C, Arrigoni F, Rustico M, Re TJ, Triulzi F (2011) Fetal MRI features related to the Chiari malformations. Neurol Sci 32(Suppl 3):S279–S281PubMedCrossRefPubMedCentralGoogle Scholar
  143. Roldan O, Wieben O, Haughton V, Osswald T, Chesler N (2009) Characterization of CSF hydrodynamics in the presence and absence of tonsillar ectopia by means of computational flow analysis. AJNR 30:941–946PubMedCrossRefPubMedCentralGoogle Scholar
  144. Salman MS (2011) Posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study. Childs Nerv Syst 27:457–462PubMedCrossRefPubMedCentralGoogle Scholar
  145. Salman MS, Blaser SE, Sharpe JA, Dennis M (2006) Cerebellar vermis morphology in children with spina bifida and Chiari type II malformation. Childs Nerv Syst 22:385–393PubMedCrossRefPubMedCentralGoogle Scholar
  146. Sav A (2008) Pathological anatomy of spina bifida. In: Ozek MM, Cinalli G, Maixner WJ (eds) Spina bifida. Springer, Milan, pp 43–57CrossRefGoogle Scholar
  147. Schwahn B, Rozen R (2001) Polymorphisms in the methylenetetrahydrofolate reductase gene: clinical consequences. Am J Pharmacogenomics 1:189–201PubMedCrossRefPubMedCentralGoogle Scholar
  148. Schwalbe E, Gredig M (1907) Ueber Entwicklungstströrungen des Kleihirns, Hirnstamms und Halsmarks bei Spina bifida (Arnold’sche und Chiari’sche Missbildung). Beitr Path Anat 40:132–194Google Scholar
  149. Sebire NJ, Noble PI, Thorpe-Beeston JG, Snijders RJ, Nicolaides KH (1997) Presence of the lemon sign in fetuses with spina bifida at the 12-14 week scan. Ultrasound Obstet Gynecol 10:403–407PubMedCrossRefPubMedCentralGoogle Scholar
  150. Sener RN, Dzelzite S (2003) Rhombencephalosynapsis and a Chiari II malformation. J Comput Assist Tomogr 27:257–259PubMedCrossRefPubMedCentralGoogle Scholar
  151. Sgouros S (2008) Chiari II malformation and syringomyelia. In: Ozek MM, Cinalli G, Maixner WJ (eds) Spina bifida. Springer, Milan, pp 237–248CrossRefGoogle Scholar
  152. Sgouros S, Kountouri M, Natarajan K (2006) Posterior fossa volume in children with Chiari malformation type I. J Neurosurg 105:101–106PubMedPubMedCentralGoogle Scholar
  153. Shankar P, Zamora C, Castillo M (2016) Congenital malformations of the brain and spine. Handb Clin Neurol 136:1121–1137PubMedCrossRefPubMedCentralGoogle Scholar
  154. Shaw GM, Velie EM, Shaffer D (1996) Risk of neural tube defect-affected pregnancies among obese women. JAMA 275:1093–1096PubMedCrossRefPubMedCentralGoogle Scholar
  155. Sim KB, Hong SK, Cho BK, Choi DY, Wang KC (1996) Experimentally induced Chiari-like malformation with myeloschisis in chick embryos. J Korean Med Sci 11:509–516PubMedCrossRefPubMedCentralGoogle Scholar
  156. Sim K-B, Lee JY, Phi JH, Kim S-K, Wang KC (2013) Experimental models of spinal open neural tube defect and Chiari type II malformation. Childs Nerv Syst 29:1435–1449PubMedCrossRefPubMedCentralGoogle Scholar
  157. Simon EM (2004) MRI of the fetal spine. Pediatr Radiol 34:712–719PubMedCrossRefPubMedCentralGoogle Scholar
  158. Sirikci A, Bayazit YA, Bayram M (2001) The Chiari III malformation: an unusual and asymptomatic variant in an 11-year old child. Eur J Radiol 29:147–150CrossRefGoogle Scholar
  159. Smith AB, Gupta N, Otto C, Glenn OA (2007) Diagnosis of Chiari III malformation by second trimester fetal MRI with postnatal MRI and CT correlation. Pediatr Radiol 37:1035–1038PubMedCrossRefPubMedCentralGoogle Scholar
  160. Snyder WE Jr, Luerssen TG, Boaz JC, Kalsbeck JE (1998) Chiari III malformation treated with CSF diversion and delayed surgical closure. Pediatr Neurosurg 29:117–120PubMedCrossRefPubMedCentralGoogle Scholar
  161. Soare PL, Ramondi AJ (1977) Intellectual and perceptual-motor characteristics of treated myelomeningocele children. Am J Dis Child 131:199–204PubMedPubMedCentralGoogle Scholar
  162. Spennato P, Ruggiero C, Aliberti F, Nastro A, Mirone G, Cinalli G (2013) Third ventriculostomy in shunt malfunction. World Neurosurg 79(2 Suppl):e21–e26Google Scholar
  163. Stritzke AI, Dunham CP, Smyth JA, Steinbok P (2011) Congenital stridor in the context of Chiari malformation type II: the etiological role of vernix caseosa granulomatous meningitis. J Neurosurg Pediatrics 8:372–376CrossRefGoogle Scholar
  164. Sutton L, Adzik NS, Johnson MP (2003) Fetal surgery of myelomeningocele. Childs Nerv Syst 19:587–591PubMedCrossRefPubMedCentralGoogle Scholar
  165. Talamonti G, Zella S (2011) Surgical treatment of CM2 and syringomyelia in a series of 231 myelomeningocele patients. Neurol Sci 32(Suppl 3):S331–S333PubMedCrossRefPubMedCentralGoogle Scholar
  166. Taylor DG, Mastorakos P, Jane JA Jr, Oldfield EH (2017) Two distinct populations of Chiari I malformation based on presence or absence of posterior fossa crowdedness on magnetic resonance imaging. J Neurosurg 126:1934–1940PubMedCrossRefPubMedCentralGoogle Scholar
  167. Tekin D, Uysal S, Iyigun O (2002) Primary cerebellar agenesis – Chiari IV malformation. MOU Tip Dergisi 19:213–216Google Scholar
  168. Testoni S, Pria AD, Gentile A (2010) Imaging diagnosis-cerebellar displacement and spina bifida in a calf. Vet Radiol Ultrasound 51:162–164PubMedCrossRefPubMedCentralGoogle Scholar
  169. The EUROCAT Working Group (1987) Prevalence of neural tube defects in 16 regions of Europe, 1980–1983. Int J Epidemiol 16:246–251CrossRefGoogle Scholar
  170. The EUROCAT Working Group (1991) Prevalence of neural tube defects in 20 regions of Europe and the impact of prenatal diagnosis, 1980-1986. J Epidemiol Comm heatlh 45:52–58CrossRefGoogle Scholar
  171. Titomanlio L, Romano A, Del Giudice E (2005) Cerebellar agenesis. Neurology 64:E21PubMedCrossRefPubMedCentralGoogle Scholar
  172. 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 42:471–491PubMedCrossRefPubMedCentralGoogle Scholar
  173. Trigylidas T, Baronia B, Vassyliadi M, Ventureyra ECG (2008) Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformations. Childs Nerv Syst 24:329–336PubMedCrossRefPubMedCentralGoogle Scholar
  174. Tubbs RS, Oakes WJ (2004) Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 20:375–381PubMedCrossRefPubMedCentralGoogle Scholar
  175. Tubbs RS, Oakes WJ (2012) The Chiari V malformation. Childs Nerv Syst 28:339CrossRefGoogle Scholar
  176. Tubbs RS, Muhleman M, Loukas M, Oakes WJ (2012) A new form of herniation: the Chiari V malformation. Childs Nerv Syst 28:305–307PubMedCrossRefPubMedCentralGoogle Scholar
  177. Tubbs RS, Demerdash A, Vahedi P, Griessenauer CJ, Oakes WJ (2016) Chiari IV malformation: correcting an over one century long historical error. Childs Nerv Syst 32:1175–1179PubMedCrossRefPubMedCentralGoogle Scholar
  178. Tude Melo JR, Pacheco P, Nunes de Melo E, Vasconcellos A, Klein Passos R (2015) Clinical and ultrasonographic criteria for using ventriculoperitoneal shunts in newborns with myelomeningocele. Arq Neuropsiquiatr 73:759–763CrossRefGoogle Scholar
  179. Tulipan N, Hernanz-Schulman M, Lowe LH, Bruner JP (1999) Intrauterine myelomeningocele repair reverses pre-existing hindbrain herniation. Pediatr Neurosurg 31:137–142PubMedCrossRefPubMedCentralGoogle Scholar
  180. Tulipan N, Wellons JC 3rd, Thom EA, Gupta N, Sutton LN, Burrows PK, Farmer D, Walsh W, Johnson MP, Rand L, Tolivaisa S, D'alton ME, Adzick NS, MOMS Investigators (2015) Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr 16:613–620PubMedCrossRefPubMedCentralGoogle Scholar
  181. Udayakumaran S (2012) Chiari V or Chiari II plus? Childs Nerv Syst 28:337–338PubMedCrossRefPubMedCentralGoogle Scholar
  182. Udayakumaran S, Ben Sira L, Constantini S (2010) Temporal lobe herniation of developmental origin: a novel radiological association with open spina bifida and Chiari II malformation. Childs Nerv Syst 26:277–278PubMedCrossRefPubMedCentralGoogle Scholar
  183. Van den Hof MC, Nicolaides KH, Campbell J, Campbell S (1990) Evaluation of the lemon and banana signs in one hundred and thirty foetuses with open spina bifida. Am J Obstet Gynecol 162:322–327PubMedCrossRefPubMedCentralGoogle Scholar
  184. Van der Linden IJ, Afman LA, Heil SG, Blom HJ (2006) Genetic variations in gene of folate metabolism and neural tube defect risk. Proc Nutr Soc 65:204–215PubMedCrossRefPubMedCentralGoogle Scholar
  185. Van Der Vossen S, Pistorius LR, Mulder EJ, Platenkamp M, Stoutenbeek P, Visser GH, Gooskens RH (2009) Role of prenatal ultrasound in predicting survival and mental and motor functioning in children with spina bifida. Ultrasound Obstet Gynecol 34:253–258CrossRefGoogle Scholar
  186. Van Roost D, Solymosi L, Funke K (1995) A characteristic ventricular shape in myelomeningocele-associated hydrocephalus? A CT stereology study. Neuroradiology 37:412–417PubMedCrossRefPubMedCentralGoogle Scholar
  187. Vandertop WP, Asai A, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Becker LE (1992) Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele. J Neurosurg 77:541–544PubMedCrossRefPubMedCentralGoogle Scholar
  188. Velie E, 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 144:473–479PubMedCrossRefPubMedCentralGoogle Scholar
  189. Verma R, Pratiaraj HN (2012) Unusual association of Arnold Chiari malformation and vitamin B12 deficiency. BMJ Case Rep Jul 9:2012Google Scholar
  190. Vernet O, Farmer JP, Montes JL (1996) Comparison of syringopleural and syringosubarachnoid shunting in the treatment of syringomyelia in children. J Neurosurg 84:624–628PubMedCrossRefPubMedCentralGoogle Scholar
  191. Vinchon M (2011) Comment on Salman M: posterior fossa decompression and the cerebellum in Chiari type II malformation: a preliminary MRI study. Childs Nerv Syst 27:463–464PubMedCrossRefPubMedCentralGoogle Scholar
  192. Vinck A, Maassen B, Mullaart R, Rotteveel J (2006) Arnold-Chiari-II malformation and cognitive functioning in spina bifida. J Neurol Neurosurg Psychiatry 77:1083–1086PubMedCrossRefPubMedCentralGoogle Scholar
  193. von Koch CS, Compagnone N, Hirose S, Yoder S, Harrison MR, Farmer DL (2005a) Myelomeningocele: characterization of a surgically induced sheep model and its central nervous system similarities and differences to the human disease. Am J Obstet Gynecol 193:1456–1462CrossRefGoogle Scholar
  194. Von Koch CS, Glenn OA, Goldstein RB, Barkovic AJ (2005b) Fetal magnetic resonance imaging enhances detection of spinal cord anomalies in patients with sonographically detected bony anomalies of the spine. J Ultrasound Med 24:781–789CrossRefGoogle Scholar
  195. Waters KA, Forbes P, Morielli A, Hum C, O'Gorman AM, Vernet O, Davis GM, Tewfik TL, Ducharme FM, Brouillette RT (1998) Sleep-disordered breathing in children with myelomeningocele. J Pediatr 132:672–881PubMedCrossRefPubMedCentralGoogle Scholar
  196. Weber Guimarães Barreto M, Ferro MM, Guimarães Bittencourt D, Violin Pereira LA, Barini R, Sbragia L (2005) Arnold-Chiari in a fetal rat model of dysraphism. Fetal Diagn Ther 20:437–441PubMedCrossRefPubMedCentralGoogle Scholar
  197. Welsch M, Antes S, Kiefer M, Meyer S, Eymann R (2013) Association of Chiari malformation and vitamin B12 deficiency in a family. Childs Nerv Syst 29:1193–1198PubMedCrossRefPubMedCentralGoogle Scholar
  198. Williams B (1975) Cerebrospinal fluid pressure gradients in spina bifida cystica, with special reference to the Arnold-Chiari malformation and aqueductal stenosis. Dev Med Child Neurol 35:138–150Google Scholar
  199. Williams B (1990) Syringomyelia. Neurosurg Clin North Am 1:653–685CrossRefGoogle Scholar
  200. Williams H (2008) A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida. Cerebrospinal Fluid Res 5:7PubMedCrossRefPubMedCentralGoogle Scholar
  201. Woitek R, Prayer D, Weber M, Amann G, Seidl R, Bettelheim D, Schöpf V, Brugger PC, Furtner J, Asenbaum U, Kasprian G (2016) Fetal diffusion tensor quantification of brainstem pathology in Chiari II malformation. Eur Radiol 26:1274–1283PubMedCrossRefPubMedCentralGoogle Scholar
  202. Wong SK, Barkovich AJ, Callen AL, Filly RA (2009) Supratentorial abnormalities in the Chiari II malformation. III – the interhemispheric cyst. J Ultrasound Med 28:999–1006PubMedCrossRefPubMedCentralGoogle Scholar
  203. Worley G, Erwin CW, Schuster JM, Park Y, Boyko OB, Griebel ML, Weidman ER, Radtke RA, Oakes WJ (1994) BAEPs in infants with myelomeningocele and later development of Chiari II malformation-related brainstem dysfunction. Dev Med Child Neurol 36:707–715PubMedCrossRefPubMedCentralGoogle Scholar
  204. Zolal A, Vachata P, Hefcl A, Malucelli A, Bartos R, Sames M (2010) Identification of the large descending tracts using diffusion tensor imaging in Chiari III malformation. Childs Nerv Syst 26:867–870PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Luca Massimi
    • 1
    Email author
  • Massimo Caldarelli
    • 1
  • Concezio Di Rocco
    • 2
  1. 1.Pediatric NeurosurgeryA. Gemelli HospitalRomeItaly
  2. 2.Department of NeurosurgeryInternational Neuroscience InstituteHannoverGermany

Section editors and affiliations

  • Concezio Di Rocco
    • 1
  • G. Tamburrini
    • 2
  1. 1.Department of NeurosurgeryInternational Neuroscience InstituteHannoverGermany
  2. 2.Pediatric NeurosurgeryCatholic University Medical SchoolRomeItaly

Personalised recommendations