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

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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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Authors and Affiliations

  1. Department of Neurosurgery, Brain Research Institute, University of Niigata, 1-757, Asahimachi-dori, Chuo-ku,, Niigata City, Niigata, 951-8585, Japan

    Masakazu Sano, Makoto Oishi, Junichi Yoshimura & Yukihiko Fujii

  2. Department of Otolaryngology Head and Neck surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan

    Nao Takahashi

  3. Division of Pediatrics, Department of Homeostatic Regulation and Development, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan

    Keisuke Nagasaki

Authors
  1. Masakazu Sano
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  2. Nao Takahashi
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  3. Keisuke Nagasaki
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  4. Makoto Oishi
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  5. Junichi Yoshimura
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  6. Yukihiko Fujii
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Correspondence to Masakazu Sano.

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The authors declare that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.

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Sano, M., Takahashi, N., Nagasaki, K. et al. Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia. Childs Nerv Syst 34, 2275–2281 (2018). https://doi.org/10.1007/s00381-018-3880-0

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  • DOI: https://doi.org/10.1007/s00381-018-3880-0

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