Skip to main content
SpringerLink
Log in

Chiari III malformation: a comprehensive review of this enigmatic anomaly

  • Review Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Introduction

Chiari III is one of the rarest of the Chiari malformations and is characterized by a high cervical or low occipital encephalocele and osseous defect with or without spinal cord involvement and may include many of the anatomical characteristics seen in the Chiari II malformation. Herein, we provide a comprehensive review of this rare anomaly as well as a translation of Chiari’s original description.

Methods

We review all reported cases of Chiari III malformation found in the extant literature.

Conclusions

Out of 57 reported cases of Chiari III malformation, encephaloceles were in a high cervical/low occipital position in 23, 8 were in a high cervical position, 17 were in low occipital position, and the position in 9 cases was not reported. The pathogenesis of Chiari III malformation remains unclear. The majority of patients have concomitant hydrocephalus. Brain parts occurring in the sac from the most to least common include the following: cerebellum, occipital lobe, and parietal lobe. The severity of symptoms is correlated with the amount of brain structures within the encephalocele. Neurologic functional outcomes have been varied and depend on the neurological status of the patient before surgery.

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
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Chiari H (1891) Ueber Veranderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch Med Wochenschr 17:1172–1175

    Article  Google Scholar 

  2. Caldarelli M, Rea G, Cincu R, Rocco CD (2002) Chiari type III malformation. Child’s Nerv Syst 18:207–210

    Article  Google Scholar 

  3. 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 29:147–150

    Article  Google Scholar 

  4. Haberle J, Hulskamp G, Harms E, Krasemann T (2001) Cervical encephalocele in a newborn—Chiari III malformation. Child’s Nerv Syst 17:373–375

    Article  CAS  Google Scholar 

  5. Cakirer S (2003) Chiari III malformation varieties of MRI appearances in two patients. J Clin Imag 27:1–4

    Article  CAS  Google Scholar 

  6. Brownlee R, Myles T, Hamilton M (1997) The Chiari II and IV malformations. In: Anson JA et al (eds) Syringomyelia and the Chiari malformation, 1st edn. Wiley-Blackwell, New York, pp 83–90

    Google Scholar 

  7. Isik N, Elmaci I, Silva G, Celik M, Kalelioglu M (2009) Chiari malformation type III and results of surgery: a clinical study. Pediatr Neurosurg 45:19–28

    Article  CAS  PubMed  Google Scholar 

  8. Kiymaz N, Yilmaz N, Demir I, Keskin S (2010) Prognostic factors in patients with occipital encephalocele. Pediatr Neurosurg 46:6–11

    Article  PubMed  Google Scholar 

  9. McLone DG, Knepper PA (1989) The cause of Chiari II malformation: a unified theory. Pediatr Neurosci 15:1–12

    Article  CAS  PubMed  Google Scholar 

  10. Rani H, Kulkarni AV, Rao RV, Patil P (2013) Chiari III malformation: a rare case with review of literature. Fetal Pediatr Pathol 32:169–174

    Article  PubMed  Google Scholar 

  11. 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 11:509–516

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. 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 37:1035–1038

    Article  PubMed  Google Scholar 

  13. De Reuck J, Thienpont L (1976) Fetal Chiari’s type III malformation. Childs Brain 2:85–91

    PubMed  Google Scholar 

  14. Lee R, Tay KS, Cheng PW, Lui WM, Chan FL (2002) Chiari III malformation: antenatal MRI diagnosis. Clin Radiol 57:759–767

    Article  CAS  PubMed  Google Scholar 

  15. Mayr U, Aichner F, Menardi Hager J (1986) Computer-tomographic appearances of the Chiari malformations of the posterior fossa. Z Kinderchir 41:33–35

    PubMed  Google Scholar 

  16. 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 5:35–38

    Article  PubMed  Google Scholar 

  17. Dyste GN, Menezes A, Van Gildrer JC (1989) Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome. J Neurosurg 71:159–168

    Article  CAS  PubMed  Google Scholar 

  18. Castillo M, Quencer RM, Dominguez R (1992) Chiari III malformation: imaging features. AJNR 13:107–113

    CAS  PubMed  Google Scholar 

  19. 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 72:414–417

    Article  PubMed  Google Scholar 

  20. 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 49:316–319

    Article  PubMed  Google Scholar 

  21. Jaggi RS, Premsagar IC (2007) Chiari malformation type III treated with primary closure. Pediatr Neurosurg 43:424–427

    Article  PubMed  Google Scholar 

  22. Ramdurg SR, Solpure S, Dubey S, Gubbi S (2013) Asymptomatic Chiari III malformation with tectal beaking and holocord syrinx. J Pediatr Neurosci 8:254–256

    Article  PubMed Central  PubMed  Google Scholar 

  23. Muzumdar D, Gandhi S, Fattepurkar S, Goel A (2007) Type III Chiari malformation presenting as intermittent respiratory stridor: a neurological image. Pediatr Neurosurg 43:446–448

    Article  CAS  PubMed  Google Scholar 

  24. Jeong DH, Kim CH, Kim MO, Chung H, Kim TH, Jung HY (2014) Arnold-Chiari malformation type III with meningoencephalocele: a case report. Ann Rehab Med 38:401–404

    Article  Google Scholar 

  25. Aribal ME, Gurcan F, Aslan B (1996) Chiari III malformation: MRI. Neuroradiology 38:S184–S186

    Article  PubMed  Google Scholar 

  26. Chaudhari AM, Karas CS, Baig MN, Elton SW (2008) Unique defect representing features of Chiari III and IV malformations. Pediatr Neurosurg 44:513–514

    Article  PubMed  Google Scholar 

  27. Kannegieter L, Dietrich RB, Pais MJ, Goldenberg TM (1994) Pediatric case of the day. Radiographics 14:452–454

    Article  CAS  PubMed  Google Scholar 

  28. Garg K, Tandon V, Mahapatra AK (2011) Chiari III malformation with proatlas abnormality. Pediatr Neurosurg 47:295–298

    Article  PubMed  Google Scholar 

  29. 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 3:169–171

    Article  Google Scholar 

  30. 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 44:86–89

    Article  Google Scholar 

  31. 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 21:655–658

    PubMed  Google Scholar 

  32. Raimondi AJ (1972) Pediatric neuroradiology. Saunders, Philadelphia, pp 275–343

    Google Scholar 

  33. Snyder WE Jr, Luerssen TG, Boaz JC, Kalsbeck JE (1998) Chiari III malformation treated with CSF diversion and delayed surgical closure. Pediatr Neurosurg 29:117–120

    Article  PubMed  Google Scholar 

  34. 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 26:867–870

    Article  PubMed  Google Scholar 

  35. Ambekhar S, Devi BI, Shukla D (2011) Large occipito-cervical encephalocele with Chiari III malformation. J Pediatr Neurosci 6:116–117

    Google Scholar 

  36. Agrawal A, Mittal A, Kohali GB, Sampley S, Gupta A (2011) Chiari III malformation. Pediatr Neurosurg 47:309–310

    Article  PubMed  Google Scholar 

  37. Pittman HW (1990) The Chiari crisis. BNI Q 6:10–16

    Google Scholar 

  38. Kernan JC, Horgan MA, Piatt JH (1996) Tethered hindbrain. J Neurosurg 85:713–715

    Article  CAS  PubMed  Google 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 28:184–194

    Article  PubMed  Google Scholar 

Download references

Conflicts of interest

The authors have not conflicts of interest to declare.

Author information

Authors and Affiliations

  1. Pediatric Neurosurgery, Children’s of Alabama, Birmingham, AL, USA

    Galyna Ivashchuk, Jeffrey P. Blount & W. Jerry Oakes

  2. Department of Anatomical Sciences, St. George’s University, St. George, Grenada

    Marios Loukas

  3. Seattle Science Foundation, Seattle, WA, USA

    R. Shane Tubbs

Authors
  1. Galyna Ivashchuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marios Loukas
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jeffrey P. Blount
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R. Shane Tubbs
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. W. Jerry Oakes
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to R. Shane Tubbs.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ivashchuk, G., Loukas, M., Blount, J.P. et al. Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst 31, 2035–2040 (2015). https://doi.org/10.1007/s00381-015-2853-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-015-2853-9

Keywords

Search

Navigation