Visual function abnormalities in Arnold-Chiari syndrome: Analysis of two cases
DOI:
https://doi.org/10.31288/oftalmolzh202566066Keywords:
Arnold-Chiari syndrome, head MRI, optic nerve, neuritis, comprehensive treatmentAbstract
Purpose: To analyze clinical manifestations of visual function abnormalities in Arnold-Chiari syndrome.
Material and Methods: Two patients with Arnold-Chiari syndrome (ACS) were under our observation at SI “The Filatov Institute of Eye Diseases and Tissue Therapy of the National Academy of Medical Sciences of Ukraine” and Eye Microsurgery Center, Multipurpose Medical Center, Odesa National Medical University. They underwent general clinical eye examinations.
Results: We reviewed the cases of two patients with ACS. Major manifestations of the disease and the potential for treatment for preserving visual functions were determined.
Conclusion: Taking a comprehensive approach to medical issues (especially those related to comorbid conditions) was always important. There are several ophthalmological issues that require consultations with neuropathologists and neurosurgeons for making a correct diagnosis and starting a subsequent treatment early. The cases presented showed that possible ACS manifestations may include e.g., strabismus and papilledema. Knowing these manifestations is a prerequisite for solving the diagnostic and treatment issues of the disease. There is no specific pathognomonic clinical sign or group of symptoms for the clinical diagnosis of ACS, and the disease can be diagnosed only by neuroimaging. Early identification of these ocular abnormalities allows suspecting and detecting this malformation early. Comprehensive treatment over many years can stabilize visual functions in patients with ACS.
References
Liao C, Visocchi M, Zhang W, Li S, Yang M, Zhong W, Liu P. The Relationship Between Basilar Invagination and Chiari Malformation Type I: A Narrative Review. Acta Neurochir Suppl. 2019;125:111-118. https://doi.org/10.1007/978-3-319-62515-7_16
Brito JNPO, Santos BAD, Nascimento IF, Martins LA, Tavares CB. Basilar invagination associated with chiari malformation type I: A literature review. Clinics (Sao Paulo). 2019;74:e653. https://doi.org/10.6061/clinics/2019/e653
Klekamp J, Alves OL, Zileli M, Oertel J, Yaman O, Sharif S, Visocchi M, Goel A, Botelho R. Incidence and Management of Basilar Invagination With Associated Chiari I Malformation: WFNS Spine Committee Recommendations. Spine (Phila Pa 1976). 2025 Jun 1;50(11):786-791. https://doi.org/10.1097/BRS.0000000000005293
Wang J, Alotaibi NM, Samuel N, Ibrahim GM, Fallah A, Cusimano MD. Acquired Chiari Malformation and Syringomyelia Secondary to Space-Occupying Lesions: A Systematic Review. World Neurosurg. 2017 Feb;98:800-808.e2. https://doi.org/10.1016/j.wneu.2016.11.080
Ciaramitaro P, Massimi L, Bertuccio A, et al. International Experts Jury of the Chiari Syringomyelia Consensus Conference, Milan, November 11-13, 2019. Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci. 2022 Feb;43(2):1327-1342. https://doi.org/10.1007/s10072-021-05347-3
Arnold J. Myelicyste. Transposition von Gewebskeinmen und sympoide. Beirt Path Anat 1894; 16: 1; 2. Chiari H. Uber veränderugen des kleinhirns infolge von hydrocephalie des grosshirns. Deutsche Medicinische Wocherschrif 1891; 17: 1172. 3.https://doi.org/10.1055/s-0029-1206803
Chiari H. Uber veränderungen des kleinhirns, des pons and der medulla oblongata in folge von congenitaler hydrocephalie des grosshirns. Deutsch Akad Wiss Wien (Peach B 1965) 1895; 63: 71
Coria F, et al. Occipital dysplasia and Chiari type I deformity in a family: clinical and radiological study of three generations. J Neurol Sci 1983; 62: 147-158.https://doi.org/10.1016/0022-510X(83)90195-8
Onafowokan OO, Das A, Mir JM, Alas H, Williamson TK, et al. Predictors of reoperation for spinal disorders in Chiari malformation patients with prior surgical decompression. J Craniovertebr Junction Spine. 2023 Oct-Dec;14(4):336-340. https://doi.org/10.4103/jcvjs.jcvjs_140_23
Elbaroody M, Mostafa HE, Alsawy MFM, Elhawary ME, Atallah A, Gabr M. Outcomes of Chiari Malformation III: A Review of Literature. J Pediatr Neurosci. 2020 Oct-Dec;15(4):358-364. https://doi.org/10.4103/JPN.JPN_135_19
Chatterjee S, Shivhare P, Verma SG. Chiari malformation and atlantoaxial instability: problems of co-existence. Childs Nerv Syst. 2019 Oct;35(10):1755-1761. https://doi.org/10.1007/s00381-019-04284-z
Ivashchuk G, Loukas M, Blount JP, Tubbs RS, Oakes WJ. Chiari III malformation: a comprehensive review of this enigmatic anomaly. Childs Nerv Syst. 2015 Nov;31(11):2035-40. https://doi.org/10.1007/s00381-015-2853-9
Häberle J, Hülskamp G, Harms E, Krasemann T. Cervical encephalocele in a newborn--Chiari III malformation. Case report and review of the literature. Childs Nerv Syst. 2001 May;17(6):373-5. https://doi.org/10.1007/s003810000434
Furtado SV, Anantharam BA, Reddy K, Hegde AS. Repair of Chiari III malformation using cranioplasty and an occipital rotation flap: technical note and review of literature. Surg Neurol. 2009 Oct;72(4):414-7; discussion 417. https://doi.org/10.1016/j.surneu.2008.09.008
Afshari FT, Solanki GA, Lo WB, Rodrigues D. Posterior Calvarial Augmentation for Chiari Malformation Type 1 Refractory to Foramen Magnum Decompression. World Neurosurg. 2020 Jul;139:70-74. https://doi.org/10.1016/j.wneu.2020.03.218
Beretta E, Vetrano IG, Curone M, Chiapparini L, Furlanetto M, Bussone G, Valentini LG. Chiari malformation-related headache: outcome after surgical treatment. Neurol Sci. 2017 May;38(Suppl 1):95-98. https://doi.org/10.1007/s10072-017-2950-5
Messing-Jünger M, Röhrig A. Primary and secondary management of the Chiari II malformation in children with myelomeningocele. Childs Nerv Syst. 2013 Sep;29(9):1553-62. https://doi.org/10.1007/s00381-013-2134-4
Ciaramitaro P, Massimi L, Bertuccio A, et al. International Experts Jury of the Chiari Syringomyelia Consensus Conference, Milan, November 11-13, 2019. Diagnosis and treatment of Chiari malformation and syringomyelia in adults: international consensus document. Neurol Sci. 2022 Feb;43(2):1327-1342. https://doi.org/10.1007/s10072-021-05347-3
Saletti V, Farinotti M, Peretta P, Massimi L, Ciaramitaro P, Motta S, Solari A, Valentini LG. The management of Chiari malformation type 1 and syringomyelia in children: a review of the literature. Neurol Sci. 2021 Dec;42(12):4965-4995. https://doi.org/10.1007/s10072-021-05565-9
Sun Y, Fang J, Wang S, Luan J, Wang N, Lv L, Xin C, Luo P, Yue Y, Cheng Z, Yang Z, Chen L. Clinical analysis of atlanto-occipital decompression in the management of chiari malformation with multi-segmental syringomyelia. Front Pediatr. 2024 Aug 29;12:1432706.
https://doi.org/10.3389/fped.2024.1432706
Gepp R, Lee JH, Parthiban J, Costa F, Dantas F, Sampaio F, Chen Z, Oertel J, Sharif S, Klekamp J, Botelho R, Zileli M, Alves ÓL. Pediatric Chiari Malformation Management: WFNS Spine Committee Recommendations. Spine (Phila Pa 1976). 2025 Jun 1;50(11):E208-E212. https://doi.org/10.1097/BRS.0000000000005290
Goel A. Is atlantoaxial instability the cause of Chiari malformation? Outcome analysis of 65 patients treated by atlantoaxial fixation. J Neurosurg Spine. 2015 Feb;22(2):116-27. https://doi.org/10.3171/2014.10.SPINE14176
Joaquim AF. Atlantoaxial instability and Chiari malformation. J Neurosurg Spine. 2015 May;22(5):55. https://doi.org/10.3171/2014.12.SPINE141194
Chotai S, Aum DJ, Strahle JM. Role of Chiari Decompression in Managing Spinal Deformity Associated with Chiari I Malformation and Syringomyelia. Neurosurg Clin N Am. 2023 Jan; 34(1):159-166. https://doi.org/10.1016/j.nec.2022.09.007
Guzun OV, Zadorozhnyy OS, Konovalova NV, Khramenko NI, Vasiuta VA, Slobodianyk SB. Reduction of the risk of glaucomatous optic neuropathy progression after complex neuroprotection. Odesa Medical Journal. 2025; 2(193):31-37.https://doi.org/10.32782/2226-2008-2025-2-5
Visocchi M, Signorelli F, Alves ÓL, Goel A, Parthiban J, Baeesa S, Sharif S, Sampaio F, Ben Ali S, Lee JH, Oertel J, Zileli M, Botelho R. Indications for Surgery and Surgical Options in Chiari Malformation: WFNS Spine Committee Recommendations. Spine (Phila Pa 1976). 2025 Jun 1;50(11):760-766. https://doi.org/10.1097/BRS.0000000000005288
Brito JNPO, Santos BAD, Nascimento IF, Martins LA, Tavares CB. Basilar invagination associated with chiari malformation type I: A literature review. Clinics (Sao Paulo). 2019;74:e653. https://doi.org/10.6061/clinics/2019/e653
Symeou S, Lampros M, Zagorianakou P, Voulgaris S, Alexiou GA. Applications of Machine Learning in the Diagnosis and Prognosis of Patients with Chiari Malformation Type I: A Scoping Review. Children (Basel). 2025 Feb 18;12(2):244. https://doi.org/10.3390/children12020244
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2025 Konovalova N. V., Zborovska O. V., Bushuieva N. M., Goranskyi Yu. I., Guzun O. V., Zhuravok Yu. O., Serebrina T. M.
This work is licensed under a Creative Commons Attribution 4.0 International License.
This work is licensed under a Creative Commons Attribution 4.0 International (CC BY 4.0) that allows users to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author as long as they cite the source.
COPYRIGHT NOTICE
Authors who publish in this journal agree to the following terms:
- Authors hold copyright immediately after publication of their works and retain publishing rights without any restrictions.
- The copyright commencement date complies the publication date of the issue, where the article is included in.
DEPOSIT POLICY
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) during the editorial process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work with an acknowledgement of its initial publication in this journal.
- Post-print (post-refereeing manuscript version) and publisher's PDF-version self-archiving is allowed.
- Archiving the pre-print (pre-refereeing manuscript version) not allowed.
