Open AccessGeneral Anesthesia Management for Adult Mucopolysaccharidosis Patients Undergoing Major Spine Surgery
Author(s) -
Tsuchiya Masahiko,
Terai Hidetomi,
Mizutani Koh,
Funai Yusuke,
Tanaka Katsuaki,
Yamada Tokuhiro,
Mori Takashi,
Nishikawa Kiyonobu
Publication year - 2019
Publication title -
medical principles and practice
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.426
H-Index - 45
eISSN - 1423-0151
pISSN - 1011-7571
DOI - 10.1159/000503051
Subject(s) - case report
Objective: Mucopolysaccharidosis (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 Intervention: We 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. Conclusion: Adult MPS patients have substantial anesthesia risk.