• Med Princ Pract · Jan 2019

    Case Reports

    General Anesthesia Management for Adult Mucopolysaccharidosis Patients Undergoing Major Spine Surgery.

    • Masahiko Tsuchiya, Hidetomi Terai, Koh Mizutani, Yusuke Funai, Katsuaki Tanaka, Tokuhiro Yamada, Takashi Mori, and Kiyonobu Nishikawa.
    • Department of Anesthesiology, Osaka City University Graduate School of Medicine, Osaka, Japan, oxymasa@ea.mbn.or.jp.
    • Med Princ Pract. 2019 Jan 1; 28 (6): 581-585.

    ObjectiveMucopolysaccharidosis (MPS) are a group of rare systemic lysosomal storage diseases associated with severe airway obstruction and cardiac disease, making anesthesia management difficult. Contemporary treatment extends the lifespan of affected individuals, increasing the need for major surgery in adulthood.Clinical Presentation And InterventionWe provided general anesthesia for 6 adult MPS patients undergoing spine surgery. The airway was assessed as difficult in all, with 2 receiving awake fiberoptic intubation and 1 successfully undergoing video-laryngoscopy, while 3 video-laryngoscopy procedures failed and required conversion to fiberoptic intubation. One patient developed ventricular fibrillation.ConclusionAdult MPS patients have substantial anesthesia risk.© 2019 The Author(s) Published by S. Karger AG, Basel.

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