Skip to main content

Advertisement

SpringerLink
Log in

Chiari type I and hydrocephalus

  • Special Annual Issue
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

The association between Chiari type I malformation (CIM) and hydrocephalus raises a great interest because of the still unclear pathogenesis and the management implications. The goal of this paper is to review the theories on the cause-effect mechanisms of such a relationship and to analyze the results of the management of this condition.

Methods

A review of the literature has been performed, focusing on the articles specifically addressing the problem of CIM and hydrocephalus and on the series reporting about its treatment. Also, the personal authors’ experience is briefly discussed.

Results

As far as the pathogenesis is concerned, it seems clear that raised intracranial pressure due to hydrocephalus can cause a transient and reversible tonsillar caudal ectopia (“pressure from above” hypothesis), which is something different from CIM. A “complex” hypothesis, on the other hand, can explain the occurrence of hydrocephalus and CIM because of the venous engorgement resulting from the hypoplasia of the posterior cranial fossa (PCF) and the occlusion of the jugular foramina, leading to cerebellar edema (CIM) and CSF hypo-resorption (hydrocephalus). Nevertheless, such a mechanism can be advocated only in a minority of cases (syndromic craniosynostosis). In non-syndromic CIM subjects, the presence of hydrocephalus could be explained by an occlusion of the basal CSF pathways, which would occur completely in a minority of cases (only 7–10% of CIM patients show hydrocephalus) while it would be partial in the remaining cases (no hydrocephalus). This hypothesis still needs to be demonstrated. As far as the management is concerned, the strategy to treat the hydrocephalus first is commonly accepted. Because of the “obstructive” origin of CIM-related hydrocephalus, the use of endoscopic third ventriculostomy (ETV) is straightforward. Actually, the analysis of the literature, concerning 63 cases reported so far, reveals very high success rates of ETV in treating hydrocephalus (90.5%), CIM (78.5%), and syringomyelia symptoms (76%) as well as in giving a radiological improvement of both CIM (74%) and syringomyelia (89%). The failures of ETV were not attributable to CIM or syringomyelia. Only 11% of cases required PCF decompression after ETV.

Conclusions

The association between CIM and hydrocephalus probably results from different, multifactorial, and not yet completely understood mechanisms, which place the affected patients in a peculiar subgroup among those constituting the heterogeneous CIM population. ETV is confirmed as the best first approach for this subset of patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Amacher AL, Page LK (1971) Hydrocephalus due to membranous obstruction of the fourth ventricle. J Neurosurg 35:672–676

    Article  CAS  PubMed  Google Scholar 

  2. Antonucci MU, Drohan A (2019) Dynamic cerebellar tonsils in Chiari malformation. J Pediatr 206:295

    Article  PubMed  Google Scholar 

  3. Badie B, Mendoza D, Batzdorf U (1995) Posterior fossa volume and response to suboccipital decompression in patients with Chiari I malformation. Neurosurgery 37:214–218

    Article  CAS  PubMed  Google Scholar 

  4. Banik R, Lin D, Miller NR (2006) Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci 247:71–75

    Article  PubMed  Google Scholar 

  5. Bartoli A, Ghinda CD, Radovanovic I, Momjian S (2012) Hidden aqueductal stenosis associated to bilateral idiopathic foramina of Monro stenosis mimicking a Chiari I malformation? Case report. Acta Neurochir 154:2109–2013

    Article  PubMed  Google Scholar 

  6. Batzdorf U (2002) Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations. Comment Neurosurg 51:503

    Google Scholar 

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

    Article  PubMed  Google Scholar 

  8. Bejjani GK (2003) Association of the adult Chiari malformation and idiopathic intracranial hypertension: more than a coincidence. Med Hypotheses 60:859–863

    Article  PubMed  Google Scholar 

  9. Buell TJ, Heiss JD, Oldfield EH (2015) Pathogenesis and cerebrospinal fluid hydrodynamics of the Chiari I malformation. Neurosurg Clin N Am 26:495–499

    Article  PubMed  PubMed Central  Google Scholar 

  10. Caldarelli M, Novegno F, Di Rocco C (2009) A late complication of CSF shunting: acquired Chiari I malformation. Childs Nerv Syst 25:443–452

    Article  PubMed  Google Scholar 

  11. Chiari H (1891) Uber Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17:1172–1175

    Article  Google Scholar 

  12. Chiari H (1987) Concerning alterations in the cerebellum resulting from cerebral hydrocephalus. Pediatr Neurosci 13:3–8 [Translated by A. Radkowski from the original paper in German]

    Article  CAS  PubMed  Google Scholar 

  13. Cinalli G, Sainte-Rose C, Kollar E, Arnaud E, Renier D (1998) Hydrocephalus and craniosynostosis. J Neurosurg 88:209–214

    Article  CAS  PubMed  Google Scholar 

  14. Cinalli G, Spennato P, Sainte-Rose C, Arnaud E, Aliberti F, Brunelle F, Cianciulli E, Renier D (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21:889–901

    Article  PubMed  Google Scholar 

  15. De Vlieger J, Dejaegher J, Van Calenbergh F (2019) Posterior fossa decompression for Chiari malformation type I: clinical and radiological presentation, outcome and complications in a retrospective series of 105 procedures. Acta Neurol Belg 2019:245–252. https://doi.org/10.1007/s13760-019-01086-7

    Article  Google Scholar 

  16. Decq P, Le Guérinel C, Sol JC, Brugières P, Djindjian M, Nguyen JP (2001) Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy. J Neurosurg 95:783–790

    Article  CAS  PubMed  Google Scholar 

  17. Deng X, Wu L, Yang C, Tong X, Xu Y (2013) Surgical treatment of Chiari I malformation with ventricular dilatation. Neurol Med Chir (Tokyo) 53:847–852

    Article  Google Scholar 

  18. Di Rocco C, Frassanito P, Massimi L, Peraio S (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst 27:1653–1664

    Article  PubMed  Google Scholar 

  19. Duddy JC, Allcutt D, Crimmins D, O'Brien D, O'Brien DF, Rawluk D, Sattar MT, Young S, Caird J (2014) Foramen magnum decompression for Chiari I malformation: a procedure not to be underestimated. Br J Neurosurg 28:330–334

    Article  PubMed  Google Scholar 

  20. Entezami P, Gooch MR, Poggi J, Perloff E, Dupin M, Adamo MA (2019) Current management of pediatric chiari type 1 malformations. Clin Neurol Neurosurg 176:122–126

    Article  PubMed  Google Scholar 

  21. Erşahin Y, Gökçay A (2002) Acquired Chiari I malformation changes postendoscopic third ventriculostomy. Pediatr Neurosurg 36:54

    Article  PubMed  Google Scholar 

  22. Fagan LH, Ferguson S, Yassari R, Frim DM (2006) The Chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I. Pediatr Neurosurg 42:14–19

    Article  PubMed  Google Scholar 

  23. Feng H, Huang G, Liao X, Fu K, Tan H, Pu H, Cheng Y, Liu W, Zhao D (2004) Endoscopic third ventriculostomy in the management of obstructive hydrocephalus: an outcome analysis. J Neurosurg 100:626–633

    Article  PubMed  Google Scholar 

  24. Francis PM, Beals S, Rekate HL, Pittman HW, Manwaring K, Reiff J (1992) Chronic tonsillar herniation and Crouzon’s syndrome. Pediatr Neurosurg 18:202–206

    Article  CAS  PubMed  Google Scholar 

  25. Fuentes S, Métellus P, Adetchessi T, Dufour H, Grisoli F (2006) Idiopathic acute obstructive hydrocephalus. Case report. Neurochirurgie 52(French):47–51

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  27. Greenberg JK, Olsen MA, Yarbrough CK, Ladner TR, Shannon CN, Piccirillo JF, Anderson RC, Wellons JC 3rd, Smyth MD, Park TS, Limbrick DD Jr (2016) Chiari malformation type I surgery in pediatric patients. Part 2: complications and the influence of comorbid disease in California, Florida, and New York. J Neurosurg Pediatr 17:525–532

    Article  PubMed  PubMed Central  Google Scholar 

  28. Greitz D (2004) Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev 27:145–165

    PubMed  Google Scholar 

  29. Gripp KW, Hopkins E, Doyle D, Dobyns WB (2010) High incidence of progressive postnatal cerebellar enlargement in Costello syndrome: brain overgrowth associated with HRAS mutations as the likely cause of structural brain and spinal cord abnormalities. Am J Med Genet 152A:1161–1168

    Article  PubMed  Google Scholar 

  30. Guan J, Riva-Cambrin J, Brockmeyer DL (2016) Chiari-related hydrocephalus: assessment of clinical risk factors in a cohort of 297 consecutive patients. Neurosurg Focus 41:E2

    Article  PubMed  Google Scholar 

  31. Hackel M, Benes V, Mohapl M (2001) Simultaneous cerebral and spinal fluid pressure recordings in surgical indications of the Chiari malformation without myelodysplasia. Acta Neurochir 143:909–918

    Article  CAS  PubMed  Google Scholar 

  32. Hashish H, Guenot M, Mertens P, Sindou M (1999) Chronic hydrocephalus in an adult due to congenital membranous occlusion of the apertura mediana ventriculi quartii (foramen of Magendie). Report of two cases and review of the literature. Neurochirurgie 45:232–236 (French)

    CAS  PubMed  Google Scholar 

  33. Hayhurst C, Osman-Farah J, Das K, Mallucci C (2008) Initial management of hydrocephalus associate with Chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg 108:1211–1214

    Article  PubMed  Google Scholar 

  34. Hoffman HJ, Tucker WS (1976) Cephalocranial disproportion. A complication of the treatment of hydrocephalus in children. Childs Brain 2:167–176

    CAS  PubMed  Google Scholar 

  35. Hofmann E, Warmuth-Metz M, Bendszus M, Solymosi L (2000) Phase-contrast MR imaging of the cervical CSF and spinal cord: volumetric motion analysis in patients with Chiari I malformation. AJNR 21:151–158

    CAS  PubMed  PubMed Central  Google Scholar 

  36. Holder-Espinasse M, Winter RM (2003) Type 1 Chiari malformation and Noonan syndrome: a new diagnostic feature? Clin Dysmorphol 12:275

    Article  PubMed  Google Scholar 

  37. Horn SR, Shepard N, Vasquez-Montes D, Bortz CA, Segreto FA, De La Garza Ramos R, Goodwin CR, Passias PG (2018) Chiari malformation clusters describe differing presence of concurrent anomalies based on Chiari type. J Clin Neurosci 58:165–171

    Article  PubMed  Google Scholar 

  38. Kandasamy J, Kneen R, Gladstone M, Newman W, Mohamed T, Mallucci C (2008) Chiari I malformation without hydrocephalus: acute intracranial hypertension managed with endoscopic third ventriculostomy. Childs Nerv Syst 24:1493–1497

    Article  PubMed  Google Scholar 

  39. Kim H, Itamoto K, Watanabe M, Nakaichi M, Taura Y (2006) Application of ventriculoperitoneal shunt as a treatment for hydrocephalus in a dog with syringomyelia and Chiari I malformation. J Vet Sci 7:203–206

    Article  PubMed  PubMed Central  Google Scholar 

  40. Kline-Fath BM, Arroyo MS, Calvo-Garcia MA, Horn PS, Thomas C (2018) Congenital aqueduct stenosis: progressive brain findings in utero to birth in the presence of severe hydrocephalus. Prenat Diagn 38:706–712

    Article  PubMed  Google Scholar 

  41. Konar SK, Shukla D, Devi BI (2018) Neuroendoscopic management of coexisting congenital agenesis of bilateral foramen of Monro with aqueductal stenosis and Chiari malformation: case report and review of the literature. World Neurosurg 118:55–58

    Article  PubMed  Google Scholar 

  42. Kratz CP, Zampino G, Kriek M, Kant SG, Leoni C, Pantaleoni F, Oudesluys-Murphy AM, Di Rocco C, Kloska SP, Tartaglia M, Zenker M (2009) Craniosynostosis in patients with Noonan syndrome caused by germline KRAS mutations. Am J Med Genet A 149A:1036–1040

    Article  CAS  PubMed  Google Scholar 

  43. Kreiborg S, Marsh JL, Cohen MM Jr, Liversage M, Pedersen H, Skovby F, Borgesen SE, Vannier MW (1993) Comparative three-dimensional analysis of CT-scans of the calvaria and cranial base in Apert and Crouzon syndrome. J Craniomaxillofac Surg 21:181–188

    Article  CAS  PubMed  Google Scholar 

  44. Lee M, Rezai AR, Wisoff JH (1995) Acquired Chiari I malformation and hydromyelia secondary to a giant craniopharingioma. Pediatr Neurosurg 22:251–254

    Article  CAS  PubMed  Google Scholar 

  45. Levine DN (2004) The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 220:3–21

    Article  PubMed  Google Scholar 

  46. LoPresti MA, Pan IW, Gadgil N, Wagner K, Lam S (2018) Outcomes and resource utilization in surgery for Chiari I malformation in a national network of children’s hospitals. Childs Nerv Syst 10:657–664. https://doi.org/10.1007/s00381-018-4012-6

    Article  Google Scholar 

  47. Loukas M, Shayota BJ, Oelhafen K, Miller JH, Chern JJ, Tubbs RS, Oakes WJ (2011) Associated disorders of Chiari type I malformations a review. Neurosurg Focus 31:E3

    Article  PubMed  Google Scholar 

  48. Martinez-Lage JF, Guillén-Navarro E, Almagro MJ, Felipe-Murcia M, López López-Guerrero A, Galarza M (2010) Hydrocephalus and Chiari type 1 malformation in macrocephaly-cutis marmorata telangiectatica congenita: a case-based update. Childs Nerv Syst 26:13–18

    Article  PubMed  Google Scholar 

  49. Massimi L, Novegno F, Di Rocco C (2011) Chiari type 1 malformation in children. Adv Tech Stand Neurosurg 37:143–211

    Article  Google Scholar 

  50. Massimi L, Pravatà E, Tamburrini G, Gaudino S, Pettorini B, Novegno F, Colosimo C Jr, Di Rocco C (2011) Endoscopic third ventriculostomy for the management of Chiari I and related hydrocephalus: outcome and pathogenetic implications. Neurosurgery 68:950–956

    Article  PubMed  Google Scholar 

  51. Metellus P, Dufour H, Levrier O, Grisoli F (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

    Article  PubMed  Google Scholar 

  52. Milhorat TH, Chou MW, Trinidad EM, Kula RW, Mandell M, Wolpert C, Speer MC (1999) Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients. Neurosurgery 44:1005–1017

    Article  CAS  PubMed  Google Scholar 

  53. Mohanty A, Suman R, Shankar SR, Satish S, Shankar Praharaj S (2005) Endoscopic third ventriculostomy in the management of Chiari I malformatgion and syringomyelia associated with hydrocephalus. Clin Neurol Neurosurg 108:87–92

    Article  PubMed  Google Scholar 

  54. Nishihara T, Hara T, Suzuki I, Kirino T, Yamakawa K (1996) Third ventriculostomy for symptomatic syringomyelia using flexible endoscope: case report. Minim Invasive Neurosurg 39:130–132

    Article  CAS  PubMed  Google Scholar 

  55. Nishikawa M, Sakamoto H, Hakuba A, Nakanishi N, Inoue Y (1997) Pathogenesis of Chiari malformation: a morphometric study of the posterior cranial fossa. J Neurosurg 86:40–47

    Article  CAS  PubMed  Google Scholar 

  56. Novegno F, Caldarelli M, Massa A, Chieffo D, Massimi L, Pettorini B, Tamburrini G, Di Rocco C (2008) The natural history of the Chiari type I anomaly. J Neurosurg Pediatrics 2:179–187

    Article  Google Scholar 

  57. Osuagwu FC, Lazareff JA, Rahman S, Bash S (2005) Chiari I anatomy after ventriculoperitoneal shunting:posterior fossa volumetric evaluation with MRI. Childs Nerv Syst 22:1451–1456

    Article  Google Scholar 

  58. Passias PG, Pyne A, Horn SR, Poorman GW, Janjua MB, Vasquez-Montes D, Bortz CA, Segreto FA, Frangella NJ, Siow MY, Sure A, Zhou PL, Moon JY, Diebo BG, Vira SN (2018) Developments in the treatment of Chiari type 1 malformations over the past decade. J Spine Surg 4:45–54

    Article  PubMed  PubMed Central  Google Scholar 

  59. Rifkinson-Mann S, Sachdev VP, Huang YP (1987) Congenital fourth ventricular midline outlet obstruction. Report of two cases. J Neurosurg 67:595–599

    Article  CAS  PubMed  Google Scholar 

  60. Rijken BF, Lequin MH, Van Veelen ML, de Rooi J, Mathijssen IM (2015) The formation of the foramen magnum and its role in developing ventriculomegaly and Chiari I malformation in children with craniosynostosis syndromes. J Craniomaxillofac Surg 43:1042–1048

    Article  PubMed  Google Scholar 

  61. Sacko O, Boetto S, Lauwers-Cances V, Dupuy M, Roux FE (2010) Endoscopic third ventriculostomy: outcome analysis in 368 procedures. J Neurosurg Pediatr 5:68–74

    Article  PubMed  Google Scholar 

  62. Salvador SF, Oliveira J, Pereira J, Barros H, Vaz R (2014) Endoscopic third ventriculostomy in the management of hydrocephalus: outcome analysis of 168 consecutive procedures. Clin Neurol Neurosurg 126:130–136

    Article  PubMed  Google Scholar 

  63. Schijman E, Steinbok P (2004) International survey on the management of Chiari I malformation and syringomyelia. Childs Nerv Syst 20:341–348

    Article  PubMed  Google Scholar 

  64. Sgouros S, Goldin JH, Hockley AD, Wake MJ (1996) Posterior skull surgery in craniosynostosis. Childs Nerv Syst 12(11):727–733

    Article  CAS  PubMed  Google Scholar 

  65. Sgouros S, Kountouri M, Natarajan K (2007) Skull base growth in children with Chiari malformation type I. J Neurosurg 107(3 Suppl):188–192

    PubMed  Google Scholar 

  66. Sgulò FG, Spennato P, Aliberti F, Di Martino G, Cascone D, Cinalli G (2017) Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature. Childs Nerv Syst 33:187–192

    Article  PubMed  Google Scholar 

  67. Sheehan JM, Jane JA Sr (2000) Resolution of tonsillar herniation and syringomyelia after supratentorial tumor resection: case report and review of the literature. Neurosurgery 47:233–235

    CAS  PubMed  Google Scholar 

  68. Suheiro T, Inamura T, Natori Y, Sasaki M, Fukui M (2000) Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report. J Neurosurg 93:326–329

    Article  Google Scholar 

  69. Teo C, Nakaji P, Serisier D, Coughlan M (2005) Resolution of trigeminal neuralgia following third ventriculostomy for hydrocephalus associated with Chiari I malformation: case report. Minim Invasive Neurosurg 48:302–305

    Article  CAS  PubMed  Google Scholar 

  70. Thotakura AK, Marabathina NR (2017) Acquired Chiari I malformation with syringomyelia secondary to colloid cyst with hydrocephalus-case report and review of literature. World Neurosurg 108(995):995.e1–995.e4

    Article  Google Scholar 

  71. Tubbs RS, Oakes WJ (2003) Costello syndrome and Chiari I malformation: apropos of a case with a review of the literature regarding a potential association. J Child Neurol 18:496–498

    Article  PubMed  Google Scholar 

  72. Tubbs RS, Rutledge SL, Kosentka A, Bartolucci AA, Oakes WJ (2004) Chiari I malformation and neurofibromatosis type 1. Pediatr Neurol 30:278–280

    Article  PubMed  Google Scholar 

  73. Tubbs RS, Lyerly MJ, Loukas M, Shoja M, Oakes WJ (2007) The pediatric Chiari I malformation: a review. Childs Nerv Syst 23:1239–1250

    Article  PubMed  Google Scholar 

  74. Tubbs RS, Beckman J, Naftel RP, Chern JJ, Wellons JC III, Rozzelle CJ, Blount JP, Oakes WJ (2011) Institutional experience with 500 cases of surgically treated pediatric Chiari malformation type I. J Neurosurg Pediatr 7:248–256

    Article  PubMed  Google Scholar 

  75. Vega RA, Harrison JF, Scott Graham R (2014) Tectal plate glioma: lessons learned from a patient with hydrocephalus, Chiari malformation-1 and concomitant aqueductal stenosis. Clin Neurol Neurosurg 119:50–53

    Article  PubMed  Google Scholar 

  76. Venes JL (1988) Arnold-Chiari malformation in an infant with Kleeblattschadel: an acquired malformation. Neurosurgery 23:360–362

    Article  CAS  PubMed  Google Scholar 

  77. Vivas AC, Shimony N, Jackson EM, Xu R, Jallo GI, Rodriguez L, Tuite GF, Carey CM (2018) Management of hydrocephalus and subdural hygromas in pediatric patients after decompression of Chiari malformation type I: case series and review of the literature. J Neurosurg Pediatr 22:426–438

    Article  PubMed  Google Scholar 

  78. Wen L, Ma C, Wang H, Hu Z (2014) The role of hydrocephalus in the development of Chiari I malformation and syringomyelia. J Neurol Sci 344:240–242

    Article  PubMed  Google Scholar 

  79. Williams H (2008) A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida. Cerebrospinal Fluid Res 11:7

    Article  Google Scholar 

  80. Williams H (2008) The venous hypothesis of hydrocephalus. Med Hypotheses 70:743–747

    Article  PubMed  Google Scholar 

  81. Wu Y, Li C, Zong X, Wang X, Gui S, Gu C, Zhang Y (2018) Application of endoscopic third ventriculostomy for treating hydrocephalus-correlated Chiari type I malformation in a single Chinese neurosurgery centre. Neurosurg Rev 41:249–254

    Article  PubMed  Google Scholar 

  82. Yoshimoto S, Takai K, Takahashi K, Yasui T, Taniguchi M (2018) Intracranial hypotension and hypertension: reversible Chiari malformation due to dynamic cerebrospinal fluid abnormalities in Gorham-stout disease. J Neurosurg Pediatr 22:508–512

    Article  PubMed  Google Scholar 

  83. Zampino G, Pantaleoni F, Carta C, Cobellis G, Vasta I, Neri C, Pogna EA, DeFeo E, Delogu A, Sarlozy A, Atzeri F, Selicorni A, Rauen KA, Cytrymbaum CS, Weksberg R, Dallapiccola B, Ballabio A, Gelb BD, Neri G, Tartaglia M (2007) Diveristy, parental germline origin, and phenotypic spectrum of de novo HRAS missense changes in Costello syndrome. Hum Mutat 28:265–272

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Fondazione Policlinico Gemelli IRCCS, Neurochirurgia Infantile, Roma, Italy

    Luca Massimi, Gianpiero Tamburrini & Massimo Caldarelli

  2. Istituto di Neurochirurgia, Università Cattolica del Sacro Cuore, Roma, Italy

    Luca Massimi, Giovanni Pennisi, Paolo Frassanito, Gianpiero Tamburrini & Massimo Caldarelli

  3. International Neuroscience Institute, Hannover, Germany

    Luca Massimi & Concezio Di Rocco

Authors
  1. Luca Massimi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Giovanni Pennisi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Paolo Frassanito
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Gianpiero Tamburrini
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Concezio Di Rocco
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Massimo Caldarelli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Luca Massimi.

Ethics declarations

Conflict of interest

No conflict of interest to disclose.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Massimi, L., Pennisi, G., Frassanito, P. et al. Chiari type I and hydrocephalus. Childs Nerv Syst 35, 1701–1709 (2019). https://doi.org/10.1007/s00381-019-04245-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-019-04245-6

Keywords

Search

Navigation