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Predictors of cervical myelopathy and hydrocephalus in young children with achondroplasia

Cited 4 time in Web of Science Cited 3 time in Scopus
Authors

Shim, Youngbo; Ko, Jung Min; Cho, Tae-Joon; Kim, Seung‐Ki; Phi, Ji Hoon

Issue Date
2021-02-12
Publisher
BMC
Citation
Orphanet Journal of Rare Diseases. 2021 Feb 12;16(1):81
Keywords
AchondroplasiaCervical myelopathyForamen magnum decompressionHydrocephalusPredictorVentriculo-peritoneal shunt
Abstract
Background
Cervical myelopathy and hydrocephalus occasionally occur in young children with achondroplasia. However, these conditions are not evaluated in a timely manner in many cases. The current study presents significant predictors for cervical myelopathy and hydrocephalus in young children with achondroplasia.

Methods
A retrospective analysis of 65 patients with achondroplasia who visited Seoul National University Childrens Hospital since 2012 was performed. The patients were divided into groups according to the presence of cervical myelopathy and hydrocephalus, and differences in foramen magnum parameters and ventricular parameters by magnetic resonance imaging between groups were analyzed. Predictors for cervical myelopathy and hydrocephalus were analyzed, and the cut-off points for significant ones were calculated.

Results
The group with cervical myelopathy showed foramen magnum parameters that indicated significantly lower cord thickness than in the group without cervical myelopathy, and the group with hydrocephalus showed significantly higher ventricular parameters and Posterior indentation grade than the group without hydrocephalus. Cord constriction ratio (OR 5199.90, p = 0.001) for cervical myelopathy and Frontal horn width (OR 1.14, p = 0.001) and Posterior indentation grade (grade 1: OR 9.25, p = 0.06; grade 2: OR 18.50, p = 0.01) for hydrocephalus were significant predictors. The cut-off points for cervical myelopathy were Cord constriction ratio of 0.25 and FM AP of 8mm (AUC 0.821 and 0.862, respectively) and Frontal horn width of 50mm and Posterior indentation grade of 0 (AUC 0.788 and 0.758, respectively) for hydrocephalus.

Conclusion
Cord constriction ratio for cervical myelopathy and Frontal horn width and Posterior indentation grade for hydrocephalus were significant predictors and may be used as useful parameters for management. Posterior indentation grade may also be used to determine the treatment method for hydrocephalus.
ISSN
1750-1172
Language
English
URI
https://hdl.handle.net/10371/174230
DOI
https://doi.org/10.1186/s13023-021-01725-4
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