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Dandy–Walker malformation is a rare cause of syringomyelia in adults

https://doi.org/10.14412/2074-2711-2017-3-62-67

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Abstract

Neural tube defects are the most common central nervous system malformation. Dandy–Walker malformation (DWM) is a rare abnormality of the posterior cranial fossa, which is generally diagnosed in the prenatal period or early infancy.
The paper describes a case of the late clinical manifestation of DWM, which has caused syringomyelia. All variants of pathological changes within the Dandy–Walker complex very rarely become a cause of syringomyelia. Only four cases of DWM, in which syringomyelia was found in adults, are known.
The authors believe that formation of syringomyelia cysts in these patients is associated with the impaired circulation of normal cerebrospinal fluid between the superior cistern and the subarachnoid spaces of the spinal cord due to the caudal dislocation of the cyst formed in the fourth ventricle.
Decompression of the craniovertebral junction, by resecting the caudal portion of the cyst in the foramen magnum, and repair of the free communication between the superior cistern and the cerebrospinal fluid spaces of the spinal cord, which is complemented by duroplasty, are pointed out to be an optimal treatment in these patients.

About the Authors

G. Yu. Evzikov
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Moscow 119021
Department of Nervous System Diseases and Neurosurgery



M. G. Bashlachev
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Moscow 119021
Department of Nervous System Diseases and Neurosurgery



K. A. Belozerskikh
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Moscow 119021
Department of Nervous System Diseases and Neurosurgery



V. A. Parfenov
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia
Russian Federation

11, Rossolimo St., Moscow 119021
Department of Nervous System Diseases and Neurosurgery



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For citation:


Evzikov G.Y., Bashlachev M.G., Belozerskikh K.A., Parfenov V.A. Dandy–Walker malformation is a rare cause of syringomyelia in adults. Neurology, Neuropsychiatry, Psychosomatics. 2017;9(3):62-67. (In Russ.) https://doi.org/10.14412/2074-2711-2017-3-62-67

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ISSN 2074-2711 (Print)
ISSN 2310-1342 (Online)