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CC BY 4.0 · J Neuroanaesth Crit Care
DOI: 10.1055/s-0044-1787879
Case Report

Anesthetic Challenges in Hirayama Disease Patients Undergoing Cervical Spine Surgery—A Case Series

Sreyashi Naskar 
1   Department of Neuroanaesthesiology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
,
Soumya Chakrabarti
1   Department of Neuroanaesthesiology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
,
Dipanjan Dawn
1   Department of Neuroanaesthesiology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
,
Amita A. Pahari
1   Department of Neuroanaesthesiology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
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Abstract

Hirayama disease (HD) is a rare disease, resulting from cervical compressive myelopathy, manifesting as upper limb muscular atrophy, and rarely autonomic and upper motor neuron signs. Anesthesia management is challenging—careful neck positioning during bag-mask ventilation and endotracheal intubation, avoidance of drugs that release histamine, multimodal monitoring to avoid delayed recovery, anticipation of hypotension, and blood loss due to autonomic dysfunction—all this is necessary for successful outcome of general anesthesia in HD patients. This case series demonstrates that preexisting autonomic dysfunction in HD patients should alert the anesthesiologists regarding higher likelihood of hemodynamic perturbations and blood loss, compared with patients who have normal autonomic functions, and henceforth take appropriate precautionary measures.

Keywords

Hirayama disease - motor neuron disease - spinal - muscular atrophy - nonprogressive


Publication History

Article published online:
08 August 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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