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Published in: Child's Nervous System 1/2020

01-01-2020 | Trisomy 21 | Case-Based Update

Atlantoaxial dislocation due to os odontoideum in patients with Down’s syndrome: literature review and case reports

Authors: Olga M. Sergeenko, Konstantin A. Dyachkov, Sergey O. Ryabykh, Alexander V. Burtsev, Alexander V. Gubin

Published in: Child's Nervous System | Issue 1/2020

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Abstract

Purpose

To clarify etiology, clinical features, and diagnostic and treatment options of atlantoaxial dislocation (AAD) due to os odontoideum (OsO) in patients with Down’s syndrome (DS).

Methods

We described and analyzed three clinical cases of AAD due to OsO in DS patients and reviewed descriptions of similar cases in the scientific sources.

Results

According to literature review, more than 80% of DS patients with odontoid ossicles had atlantoaxial instability (AAI). AAI in DS patients with OsO is more often manifested in childhood and adolescence, rarely in adults when ligament relaxation is reduced. Some patients had acute clinical manifestation after a minor trauma without any precursors; in some of the cases, neurological deterioration increased during several years. We found that the earlier surgical treatment of AAD due to OsO in DS patients carries the higher recovery potential.

Conclusions

Most patients with DS and OsO had AAI. The method of appropriate treatment in such cases is a posterior screw fixation. Preoperative halo traction and posterior fusion have proved to be a very useful tool in the treatment of AAD due to OsO in DS patients. Even if irreducibility of the AAD established preoperatively, it should not be an absolute indication for anterior decompression. In such cases, an attempt to reduce the AAD should be made under general anesthesia during posterior fixation.
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Metadata
Title
Atlantoaxial dislocation due to os odontoideum in patients with Down’s syndrome: literature review and case reports
Authors
Olga M. Sergeenko
Konstantin A. Dyachkov
Sergey O. Ryabykh
Alexander V. Burtsev
Alexander V. Gubin
Publication date
01-01-2020
Publisher
Springer Berlin Heidelberg
Keyword
Trisomy 21
Published in
Child's Nervous System / Issue 1/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-019-04401-y

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