title: Anesthetic care and perioperative complications in children with Sanfilipo Syndrome Type A.
abstract: 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.
  
  
  OBJECTIVE
  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%).
  
  
  CONCLUSIONS
  We found no change in the modified Cormack-Lehane intubation grades in 25 Sanfilippo syndrome type A children over the 2-year study period.
authors.affiliation: Department of Anesthesiology, University of Minnesota, Minneapolis, MN, USA.
authors.forename: Elif C
authors.initials: EC
authors.lastname: Cingi
authors.affiliation: Department of Anesthesiology, University of Minnesota, Minneapolis, MN, USA.
authors.forename: David S
authors.initials: DS
authors.lastname: Beebe
authors.affiliation: Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA.
authors.forename: Chester B
authors.initials: CB
authors.lastname: Whitley
authors.affiliation: Department of Anesthesiology, University of Minnesota, Minneapolis, MN, USA.|Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA.
authors.forename: Kumar G
authors.initials: KG
authors.lastname: Belani
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000768:Anesthesia, General; D002648:Child; D002675:Child, Preschool; D016292:Procedural Sedation; D004452:Echocardiography; D005260:Female; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000453:epidemiology*; D007442:Intubation, Intratracheal; D017214:Laryngeal Masks; D007826:Laryngismus / Q000453:epidemiology / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009084:Mucopolysaccharidosis III / Q000150:complications* / Q000628:therapy*; D011183:Postoperative Complications / Q000453:epidemiology*; D012135:Respiratory Sounds / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D013129:Spinal Puncture; D016896:Treatment Outcome; D055815:Young Adult
keywords: Sanfilippo syndrome type A sedation/intubation/extubation procedures; lysosomal storage disease; mucopolysaccharidosis IIIA; postoperative airway problems