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Child's Nervous System

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01-11-2018 | Original Paper

Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia

Authors: Masakazu Sano, Nao Takahashi, Keisuke Nagasaki, Makoto Oishi, Junichi Yoshimura, Yukihiko Fujii

Published in: Child's Nervous System | Issue 11/2018

Abstract

Objective

Management of cervicomedullary compression due to foramen magnum stenosis in achondroplasia remains controversial, especially for patients with no symptoms or mild symptoms. We examined the effectiveness of polysomnography (PSG) as an indicator for cervicomedullary decompression treatment.

Methods

We retrospectively reviewed nine achondroplasia cases (mean age 1 year and 9 months) treated from 2008 to 2015. All patients were examined by PSG, magnetic resonance imaging (MRI), and otolaryngeal fibroscopy. We analyzed demographic data, clinical presentation, degree and type of respiratory impairment, severity of foramen magnum stenosis and concomitant cervicomedullary compression, treatment (conservative or surgical), and clinical outcome.

Results

Eight of nine patients presented with no severe symptoms in the daytime. However, MRI revealed four severe, four moderate, and one mild case of cervicomedullary compression, and PSG demonstrated severe sleep apnea in four cases and moderate sleep apnea in five cases. All sleep apnea cases were obstructive or obstructive-dominant. Fibroscopy revealed no upper airway stenosis in six cases and mild stenosis in three cases. Four patients who had severe sleep-related respiratory disturbance on PSG and severe or moderate cervicomedullary compression were treated by cervicomedullary decompression. Three of these patients demonstrated improved sleep respiration soon after surgery, while one required temporary tracheostomy due to bilateral vocal cord paralysis caused by compression during intratracheal intubation.

Conclusion

Polysomnography can be a useful indicator for cervicomedullary decompression surgery, especially in cases of seemingly asymptomatic achondroplasia with severe foramen magnum stenosis.

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Title: Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia
Authors: Masakazu Sano
Nao Takahashi
Keisuke Nagasaki
Makoto Oishi
Junichi Yoshimura
Yukihiko Fujii
Publication date: 01-11-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 11/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3880-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia

Abstract

Objective

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusion

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