title: Delivery of anesthesia for children with Mucopolysaccharidosis Type III (Sanfilippo syndrome): a review of 86 anesthetics.
abstract: BACKGROUND
  Sanfilippo syndrome (MPS III) is rare, with 97 cases in the United Kingdom between 1988 and 1998. Mucopolysaccharide infiltration of tissues in mucopolysaccharidosis (MPS) causes multi-systemic pathology including difficult airways and cardiac disease. Published anesthesia case reviews of Sanfilippo syndrome have included limited numbers of patients to date.
  
  
  OBJECTIVE
  To identify the perioperative management and complications of anesthesia in children with mucopolysaccharidosis Type III at Great Ormond Street Hospital.
  
  
  METHODS
  A retrospective case note review of all children with MPS III in our institution was undertaken. All medical notes and anesthetic charts were analyzed, and conduct of anesthesia, airway management, perioperative complications, and associated comorbidities were identified.
  
  
  RESULTS
  There were 43 patients with MPS III, of which 34 required anesthesia, on 86 occasions for 156 procedures between 1993 and 2015. Dental extraction was the likeliest indication for anesthesia (34%) (general surgery [30%]; ear, nose, and throat [26%]; other [10%]). Thirteen of 34 patients had cardiac pathology (valvular [n = 6], functional [n = 6], electrophysiological [n = 1]). Ten of 34 patients had evidence of clotting abnormality (mild prolonged clotting time [n = 5], low von Willebrand factor [n = 2], thrombocytopenia [n = 3]). The majority of intubations were Cormack-Lehane Grade 1 (n = 47) (Grade 2 [n = 14], Grade 3 [n = 1], Grade 4 [n = 1]). In 86 anesthetics, there were 0 cases of difficulty with mask ventilation. There was 1 case of failed intubation. They were subsequently anesthetized by a different operator uneventfully at a later date. Two perioperative complications occurred: a failed intubation and bleeding during adenoidectomy.
  
  
  CONCLUSIONS
  We demonstrate a difficult airway is unlikely when anesthetizing an MPS III patient although a risk does remain. A significant proportion of MPS III have cardiac involvement although no perioperative complications were described. With associated coagulation issues, bleeding tendency, while uncommon, can occur in this group.
authors.affiliation: Department of Anaesthesia, Great Ormond Street Hospital NHS Foundation Trust, London, UK.
authors.forename: Marc A
authors.initials: MA
authors.lastname: Cohen
authors.affiliation: Department of Anaesthesia, Great Ormond Street Hospital NHS Foundation Trust, London, UK.|Portex Department of Paediatric Anaesthesia, UCL Great Ormond Street Institute of Child Health, University College London, London, UK.
authors.forename: Grant M
authors.initials: GM
authors.lastname: Stuart
mesh_terms: D000293:Adolescent; D000328:Adult; D058109:Airway Management / Q000379:methods*; D000758:Anesthesia / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009084:Mucopolysaccharidosis III / Q000601:surgery*; D012189:Retrospective Studies; D006113:United Kingdom; D055815:Young Adult
keywords: Mucopolysaccharidosis; anesthesia; difficult airway; metabolic diseases; pediatric; perioperative care