CC BY-NC-ND 4.0 · Asian J Neurosurg 2023; 18(02): 258-264
DOI: 10.1055/s-0043-1768572
Original Article

Hundred Pediatric Cases Treated for Chiari Type II Malformation with Hydrocephalus and Myelomeningocele

Daniel Encarnacion
1   Department of Neurosurgery, Morozovskaya Children City Clinic Hospital, Moscow, Russia
Gennady Chmutin
1   Department of Neurosurgery, Morozovskaya Children City Clinic Hospital, Moscow, Russia
Bipin Chaurasia
2   Department of Neurosurgery, Nilkantha Hospital and Research Centre, Birgunj, Nepal
3   Department of Neurosurgery, Medical Park Ankara Hospital, Ankara, Turkey
› Institutsangaben


Background Chiari malformation type II (CM-II) may not always present as an asymptomatic disorder but prove to be difficult in managing. This is especially true for neonates who show the worst prognosis. There is confounding data over whether shunting or craniocervical junction (CVJ) decompression should be employed. This retrospective analysis summarizes the results of 100 patients diagnosed and treated for CM-II along with hydrocephalus and myelomeningocele.

Methods We reviewed all the children who were diagnosed and surgically treated for CM-II at the Moscow Regional Hospital. Surgical timing was decided on the clinical conditions of each patient. Urgent surgery in the more compromised patients (usually infants) and elective surgery for patients with less severe conditions was performed. All patients first underwent CVJ decompression.

Results The retrospective review yielded 100 patients operated on for CM-II with concomitant hydrocephalus and myelomeningocele. The average herniation was 11.2 ± 5.1 mm. However, herniation level did not correlate with clinical findings. Concomitant syringomyelia was observed in 60% of patients. More severe spinal deformity was observed in patients with widespread syringomyelia (p = 0.04). In children of the younger age group, cerebellar symptoms and bulbar disorders were more frequently observed (p = 0.03), and cephalic syndrome was noted much less frequently (p = 0.005). The severity of scoliotic deformity correlated with the prevalence of syringomyelia (p = 0.03). Satisfactory results were significantly more often observed in patients of the older age group (p = 0.02). Patients with unsatisfactory results at the time of treatment were significantly younger (p = 0.02).

Conclusion If CM-II is asymptomatic, then no specific treatment is prescribed. If the patient develops pain in the occiput and neck, then pain relievers are prescribed. If a patient has neurological disorders or concomitant syringomyelia, hydrocephalus or myelomeningocele, surgical intervention is indicated. The operation is also performed if the pain syndrome cannot be overcome within the framework of conservative therapy.

Ethical Approval

The ethical principles outlined by the Helsinki Declaration have been followed.


Artikel online veröffentlicht:
06. Juni 2023

© 2023. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

  • References

  • 1 Kuhn J, Emmady PD. Chiari II Malformation. [Updated 2022 May 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. . Accessed March 17, 2023 at:
  • 2 Talamonti G, Marcati E, Mastino L, Meccariello G, Picano M, D'Aliberti G. Surgical management of Chiari malformation type II. Childs Nerv Syst 2020; 36 (08) 1621-1634
  • 3 Shoja MM, Johal J, Oakes WJ, Tubbs RS. Embryology and pathophysiology of the Chiari I and II malformations: a comprehensive review. Clin Anat 2018; 31 (02) 202-215
  • 4 Thiébaut M, Le Bigot P, Besson G, Dorval MP, Martin C. [Arnold-Chiari II malformation and basilar impression. Report of one case (a literature review)]. Ann Readapt Med Phys 2002; 45 (03) 131-138
  • 5 Heinrich G, Bollmann R. [Prenatal diagnosis of type II Arnold Chiari malformation]. Ultraschall Med 1993; 14 (04) 193-199
  • 6 Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 2013; 29 (09) 1553-1562
  • 7 Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst 2013; 29 (09) 1553-1562
  • 8 Tubbs RS, Oakes WJ. Treatment and management of the Chiari II malformation: an evidence-based review of the literature. Childs Nerv Syst 2004; 20 (06) 375-381
  • 9 Masur H, Oberwittler C, Reuther G, Heyen P. Cerebellar herniation in syringomyelia: relation between tonsillar herniation and the dimensions of the syrinx and the remaining spinal cord. A quantitative MRI study. Eur Neurol 1995; 35 (03) 162-167
  • 10 Godzik J, Kelly MP, Radmanesh A. et al. Relationship of syrinx size and tonsillar descent to spinal deformity in Chiari malformation type I with associated syringomyelia. J Neurosurg Pediatr 2014; 13 (04) 368-374
  • 11 Hung CF. The relationship between hydrocephalus and Chiari type II malformation in the experimental rat fetuses with Arnold-Chiari malformation. Proc Natl Sci Counc Repub China B 1986; 10 (02) 118-126
  • 12 Lacy M, DeDios-Stern S, Fredrickson S, Parikh S, Nader T, Frim DM. Prevalence of psychiatric diagnoses in pediatric Chiari malformation type 1. Pediatr Neurosurg 2018; 53 (06) 371-378
  • 13 Lázaro E, Garca M, Amayra I. et al. Anxiety and depression in Chiari malformation. J Integr Neurosci 2018; 17 (04) 343-348
  • 14 Agnoli AL, Neuhäuser G, Lochner B. [Abnormalities of the central nervous system in the area of the posterior cranial fossa–neuroradiologic and clinical aspects]. Klin Padiatr 1986; 198 (02) 96-102
  • 15 Henriques Filho PS, Pratesi R, Pratesi A. et al. Abnormalities in auditory evoked potentials of 75 patients with Arnold-Chiari malformations types I and II. Arq Neuropsiquiatr 2006; 64 (3A): 619-623