An Extension Form of Chiari 1 malformation: A Case Report
DOI:
https://doi.org/10.19184/ams.v11i3.53709Keywords:
Case report, Chiari malformation, SyringomyeliaAbstract
The updated classification of Chiari malformation includes Chiari 1.5 malformation, which explicitly denotes the tonsillar herniation characteristic of Chiari 1 malformation, accompanied by an extended brainstem and a dilated fourth ventricle. We present a Chiari 1 malformation characterized by significant downward herniation of the tonsils and the development of syringomyelia, with no indication of elongation of the fourth ventricle. This example represents an extension of Chiari 1 malformation, which is currently not classified as Chiari 1.5 malformation. A 27-year-old female exhibited cephalic and cervical discomfort for two years. The initial MRI indicated a fall of the cerebellar tonsil beneath the foramen magnum. The midsagittal MRI assessed the degree of tonsillar herniation beneath the foramen magnum. Tonsillar herniation was observed at the C2 level; a spinal MRI revealed syringomyelia affecting the C2 to C3 levels. The patient received a suboccipital craniectomy and C1-C2 laminectomy. There was dural sac pulsation at the C2 level. The patient's cervical discomfort resolved entirely following the surgical procedure. This case represents an extension of Chiari 1 malformation, which is currently not classified as adenotes explicitly 1.5 malformation.
Downloads
References
Abd-El-Barr M.M., Strong C.I., Groff M.W. (2014). Chiari malformations: diagnosis, treatments and failures. J Neurosurg Sci. 58(4):215-21.
Arnautovic A., Pojskić M., Arnautović K.I. (2023). Adult Chiari Malformation Type I: Surgical Anatomy, Microsurgical Technique, and Patient Outcomes. Neurosurg Clin N Am. 34(1):91-104.
Azahraa Haddad, F., Qaisi, I., Joudeh, N., Dajani, H., Jumah, F., Elmashala, A., Adeeb, N., Chern, J. J., & Tubbs, R. S. (2018). The newer classifications of the chiari malformations with clarifications: An anatomical review. Clin Anat. 31(3), 314–322.
Cools M.J., Wellons J.C., Iskandar B.J. (2023). The Nomenclature of Chiari Malformations. Neurosurg Clin N Am. 34(1):1-7.
Greenberg J.K., Yarbrough C.K., Radmanesh A., Godzik J., Yu M., Jeffe DB, Smyth MD, Park TS, Piccirillo JF, Limbrick DD (2015). The Chiari Severity Index: a preoperative grading system for Chiari malformation type 1. Neurosurgery. 76(3):279-85; discussion 285
Ivashchuk, G., Loukas, M., Blount, J. P., Tubbs, R. S., & Oakes, W. J. (2015). Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 31(11), 2035–2040.
Lei, Z. W., Wu, S. Q., Zhang, Z., Han, Y., Wang, J. W., Li, F., & Shu, K. (2018). Clinical Characteristics, Imaging Findings and Surgical Outcomes of Chiari Malformation Type I in Pediatric and Adult Patients. Curr Med Sci., 38(2), 289–295.
Malik, A., Chandra, R., Misra, R., & Thukral, B. B. (2015). Chiari 1.5: A lesser known entity. Ann Indian Acad Neurol, 18(4), 449–450.
McVige J.W., Leonardo J. (2015) Neuroimaging and the clinical manifestations of Chiari Malformation Type I (CMI). Curr Pain Headache Rep. 19(6):18.
Moore H.E., Moore K.R. (2014) Magnetic resonance imaging features of complex Chiari malformation variant of Chiari 1 malformation. Pediatr Radiol.44:1403–11.
Shoja, M. M., Johal, J., Oakes, W. J., & Tubbs, R. S. (2018). Embryology and pathophysiology of the Chiari I and II malformations: A comprehensive review. Clin Anat. 31(2), 202–215.
Tubbs R.S., Elton S., Grabb P., Dockery S.E., Bartolucci A.A., Oakes W.J. (2001). Analysis of the posterior fossa in children with the Chiari 0 malformation. Neurosurg. 48:1050–5.
Tubbs R.S., Iskandar B.J., Bartolucci A.A., Oakes W.J. (2004). A critical analysis of the Chiari 1.5 malformation. J Neurosurg. 101:179–83.
Tubbs R.S., McGirt M.J., Oakes W.J. (2003). Surgical experience in 130 pediatric patients with Chiari I malformations. J Neurosurg.99:291–296.
Yan, H., Han, X., Jin, M., Liu, Z., Xie, D., Sha, S., Qiu, Y., & Zhu, Z. (2016). Morphometric features of posterior cranial fossa are different between Chiari I malformation with and without syringomyelia. Eur Spine J, 25(7), 2202–2209.
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Komang Yunita Wiryaning Putri, Novan Krisno Adji, Ulfa Elfiah, Muhammad Yuda Nugraha
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.
