Arnold-Chiari malformation: etiology, pathogenesis, diagnosis, and surgical treatment

DOI: https://doi.org/10.29296/25877305-2022-04-05
Issue: 
4
Year: 
2022

A. Yarikov(1, 2), Candidate of Medical Sciences, M. Ostapyuk(2, 3), I. Lobanov(2),
A. Ermolaev(2, 3), I. Smirnov(2), I. Medyanik(3), MD; K. Yashin(3), Candidate of Medical Sciences, Professor A. Fraerman(2), MD; Professor O. Perlmutter(2), MD;
D. Godanyuk(4), Candidate of Medical Sciences; A. Kalinkin(5), Candidate of Medical Sciences
1-Volga District Medical Center, Federal Biomedical Agency of Russia, Nizhny Novgorod
2-City Clinical Hospital Thirty-Nine, Nizhny Novgorod
3-Volga Research Medical University, Nizhny Novgorod
4-V.A. Almazov National Medical Research Center, Saint Petersburg
5-Federal Siberian Research and Clinical Center, Federal Biomedical Agency
of Russia, Krasnoyarsk

Arnold-Chiari malformation (ACM) is a group of congenital developmental anomalies in the hindbrain and bones of the skull base, in which there is displacement of the cerebellar tonsils, in a number of cases the trunk and the fourth ventricle below the level of the foramen magnum. The paper describes the etiology and pathogenesis of this disease. It presents a detailed classification of ACM. Types I and II are most common among the presented ones of malformations. The clinical presentations of ACM are described in detail: the symptoms associated with impaired cerebrospinal fluid circulation, with compression/dysfunction of the brain stem or cerebellum, with syringomyelia/myelopathy, with compression of the upper spinal cord. Particular attention is paid to diagnostic techniques, such as computed tomography, magnetic resonance imaging (MRI), cardiac synchronized phase contrast MRI. Modern methods of and technical approaches to surgical treatment are also presented.
Keywords: 
neurosurgery
Arnold-Chiari malformation
Chiari malformation
syringomyelia
craniovertebral anomalies
hydrocephalus
cerebellar tonsillar ectopia
posterior cranial fossa decompression

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