General anesthesia with a native airway for patients with mucopolysaccharidosis type III.

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 ) was administered over 5 min followed by a continuous infusion at 0.5 μg·kg ·h . A continuous infusion of propofol was started at 150 μg·kg ·min . A bolus dose of propofol (1 mg·kg ) 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.