title: Perioperative complications in patients diagnosed with mucopolysaccharidosis and the impact of enzyme replacement therapy followed by hematopoietic stem cell transplantation at early age.
abstract: BACKGROUND
  Mucopolysaccharidoses (MPS) are hereditary storage diseases; airway management typically worsens in these patients with the progression of the disease.
  
  
  OBJECTIVE
  To assess the incidence of perioperative complications in children with MPS and the impact of enzyme replacement therapy (ERT) followed by hematopoietic stem cell transplantation (HSCT).
  
  
  METHODS
  The records of patients with MPS treated with ERT followed by HSCT, who received anesthesia at the Wilhelmina Children's Hospital between 2003 and 2012, were reviewed. Data were collected on incidence of perioperative respiratory and cardiovascular complications and the impact of treatment and age.
  
  
  RESULTS
  Nineteen children with MPS were identified (including 17 Hurler patients), who received ERT treatment followed by HSCT. Median age at start of treatment was 14 (range: 7-43) months. Patients were anesthetized 136 times. The incidence of respiratory and cardiovascular complications was 24% and 4%, respectively. Airway management by face mask was difficult in 7%. There were no problems with the laryngeal mask airway. Tracheal intubation was difficult in 25% and failed in 10%; using a video laryngoscope was most successful (89%), followed by classic laryngoscope (67%) and fiber-optic scope (20%). Multivariate logistic regression analyses showed that the incidence of perioperative respiratory problems did not increase with age or decrease after start of treatment.
  
  
  CONCLUSIONS
  Perioperative airway management was most successful using a laryngeal mask airway or video laryngoscope. Treatment with ERT followed by HSCT and patient age did not influence the incidence of perioperative respiratory problems.
authors.affiliation: Department of Anesthesiology, Intensive Care and Emergency Medicine, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: Johanna H A M
authors.initials: JH
authors.lastname: Megens
authors.forename: Michel
authors.initials: M
authors.lastname: de Wit
authors.forename: Peter M
authors.initials: PM
authors.lastname: van Hasselt
authors.forename: Jaap Jan
authors.initials: JJ
authors.lastname: Boelens
authors.forename: Desiree B M
authors.initials: DB
authors.lastname: van der Werff
authors.forename: Jurgen C
authors.initials: JC
authors.lastname: de Graaff
mesh_terms: D000367:Age Factors; D000758:Anesthesia / Q000379:methods; D002318:Cardiovascular Diseases / Q000453:epidemiology*; D002675:Child, Preschool; D015331:Cohort Studies; D056947:Enzyme Replacement Therapy / Q000379:methods*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods*; D006801:Humans; D015994:Incidence; D007223:Infant; D007828:Laryngoscopy / Q000379:methods; D008297:Male; D009083:Mucopolysaccharidoses / Q000453:epidemiology / Q000601:surgery / Q000628:therapy*; D009426:Netherlands / Q000453:epidemiology; D059035:Perioperative Period / Q000379:methods*; D012140:Respiratory Tract Diseases / Q000453:epidemiology*; D012189:Retrospective Studies
keywords: airway management; anesthesia; complication; hematopoietic stem cell transplantation; mucopolysaccharidosis; pediatric