Anesthetic care and perioperative complications in children with Sanfilipo Syndrome Type A

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10 Scopus citations


Background Patients with mucopolysaccharidoses (MPS) are generally considered high risk for anesthesia care, owing to disease-related factors. Sanfilippo syndrome type A (MPS IIIA) is the most frequently occurring MPS. Anesthesia-specific information for MPS IIIA is not readily available in the literature. Objectives To report post hoc analyses on anesthesia care and outcomes from a 2-year study of the natural history of patients with untreated MPS IIIA (NCT01047306). Methods Subjects were ≥1 year of age, developmental age ≥1 year, and without significant central nervous system impairment (other than that due to MPS IIIA) or issues that would preclude study procedures. Procedures requiring general anesthesia included brain/abdominal magnetic resonance imaging, lumbar puncture, and echocardiography. Sedation, intubation, and extubation procedures as well as postoperative airway problems were recorded at baseline and 6, 12, and 24 months of age. Results Twenty-five patients (baseline age, 13-220 months) received a total of 94 general anesthetics. Patients successfully received oral sedation prior to 76 of 94 anesthetics. No patients required airway intervention or oxygen supplementation during sedation. All anesthesia providers described facemask ventilation and endotracheal intubations as 'easy'. All subjects were successfully extubated after completion of the procedures. No patients required reintubation. Six (24%) patients had episodes of postoperative airway problems: wheezing (7/94, 7.4%), croup (6/94, 6.4%), and laryngospasm (2/94, 2.1%). Conclusion We found no change in the modified Cormack-Lehane intubation grades in 25 Sanfilippo syndrome type A children over the 2-year study period.

Original languageEnglish (US)
Pages (from-to)531-538
Number of pages8
JournalPaediatric anaesthesia
Issue number5
StatePublished - May 1 2016


  • Sanfilippo syndrome type A sedation/intubation/extubation procedures
  • lysosomal storage disease
  • mucopolysaccharidosis IIIA
  • postoperative airway problems


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