Sanfilippo syndrome patients are typically considered at high risk when undergoing anesthesia because of the possibility of airway obstruction, but the use of two specific anesthetics, along with adequate airway positioning, appears to be an effective regimen for these patients, a study reports.
The study, “General anesthesia with a native airway for patients with mucopolysaccharidosis type III” was published in the journal Pediatric Anesthesia.
Airway involvement in Sanfilippo syndrome, also known as mucopolysaccharidosis type III, or MPS III, is milder than what is seen in other MPS disorders. However, patients are still at risk when they undergo anesthesia due to the possibility of the airway becoming obstructed. This can occur as a result of excessive airway secretions, abnormal airway anatomy, nerve-related abnormalities, or an increase in respiratory infections.
Procedures such as magnetic resonance imaging (MRI) or lumbar puncture (LP), which are commonly used to track Sanfilippo progression, often require general anesthesia, but given the risk of airway and respiratory system obstruction in these patients, it can be difficult to achieve the necessary level of sedation to prevent movement and minimize the potential for respiratory instability.
Researchers used data from the Natural History Studies of Mucopolysaccharidosis III (NCT02037880), a one-year natural history study of MPS IIIA and IIIB, to evaluate the incidence of airway management complications during sedation or anesthesia.
A total of 25 patients underwent anesthesia for MRI or LP procedures twice over the course of the study; once initially and again at 12 months.
Following anesthesia, none of the patients required an airway device to maintain ventilation and none failed sedation. The researchers found that airway obstruction issues were more severe during the so-called inhalation induction stage of anesthesia, which is the period between the administration of induction agents and loss of consciousness, using sevoflurane. However, there were fewer issues during the maintenance phase of anesthesia, when the patient is unconscious, using a combination of dexmedetomidine and propofol.
Furthermore, the use of a shoulder roll seemed to work for decreasing airway obstruction. However, its size varied among patients, indicating that airway positioning needs to be tailored to individual needs to manage airway obstruction during anesthesia.
“We found that a combination of dexmedetomidine and propofol was an effective regimen for general anesthesia with a native airway in mucopolysaccharidosis type III patients. Although upper airway obstruction was noted, it was generally easily resolved with simple airway maneuvers,” the researchers wrote.
“The potential for airway obstruction and respiratory compromise mandates the need for appropriate monitoring during anesthesia/sedation until the patient has fully recovered,” they concluded.