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General anesthesia with a native airway for patients with mucopolysaccharidosis type III
Author(s) -
Kamata Mineto,
McKee Christopher,
Truxal Kristen V.,
Flanigan Kevin M.,
McBride Kim L.,
Aylward Shawn C.,
Tobias Joseph D.,
Corridore Marco
Publication year - 2017
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/pan.13108
Subject(s) - medicine , dexmedetomidine , anesthesia , airway , propofol , sevoflurane , sedation , airway obstruction , anesthetic , bolus (digestion) , airway management , surgery
Summary Background Mucopolysaccharidosis type III is a progressive disease with worsening airway, pulmonary, and cardiac involvement that may complicate anesthetic care. Aim To prospectively evaluate the incidence of airway issues and complications during magnetic resonance imaging (MRI) and lumbar puncture (LP) during general anesthesia with a native airway for patients with mucopolysaccharidosis type III . Method The study was a part of the natural history study. Anesthesia was induced with sevoflurane, which was discontinued after intravenous access was obtained. General anesthesia with a native airway was provided by dexmedetomidine and propofol. Dexmedetomidine (0.5 μg·kg −1 ) was administered over 5 min followed by a continuous infusion at 0.5 μg·kg −1 ·h −1 . A continuous infusion of propofol was started at 150 μg·kg −1 ·min −1 . A bolus dose of propofol (1 mg·kg −1 ) was administered and the propofol infusion was increased as needed. Airway management and vital signs were recorded for the entire procedure until discharge. Results Twenty‐five patients (6.9 ± 3.1 years) received total of 43 MRI and LP procedures in the cohort. No patient failed sedation. Although mask induction with sevoflurane was not clinically problematic, upper airway obstruction was noted during 14 procedures (33%). This required the application of continuous positive airway pressure, temporary oral airway placement, jaw thrust, or shoulder roll. Airway dynamics improved once the anesthesia was transitioned to intravenous anesthetic agents. Although a small shoulder roll was needed to improve airway patency for 11 cases (26%), a large shoulder roll tended to make the upper airway obstruction worse. Oxygen desaturation (≤90%) was noted during MRI in three cases (7%). Conclusion A combination of dexmedetomidine and propofol provided effective general anesthesia with a native airway during the procedures. Although upper airway obstruction was noted, it resolved with simple airway maneuvers without further airway intervention.