title: Management of difficult airway with laryngeal mask in a child with mucopolysaccharidosis and mitral regurgitation: a case report.
abstract: BACKGROUND
  Mucopolysaccharidoses (MPSs) are a group of heredity storage diseases, transmitted in an autosomal recessive manner, associated with the accumulation of glycosaminoglycans (GAGs) in various tissues and organs. The concerned patients have multiple concomitant hereditary anomalies. Considering the craniofacial abnormality in these patients, airway management may be difficult for anesthesiologists. In these patients, preanesthetic assessment is necessary and performed with the accurate assessment of airways, consisting of the physical exam and radiography, MRI or CT of head and neck. An anesthesiologist should set up a "difficult intubation set" with a flexible fiber-optic bronchoscope and also, it may be necessary to discuss with an ear-nose and throat (ENT) specialist if required, for unpredicted emergency situations.
  
  
  METHODS
  In this case-report we presented a 2-year-old boy with known MPSs with psychomotor retardation, bilateral corneal opacities, impaired hearing and vision, inguinal hernia, severe mitral regurgitation, micrognathia, coarse facial feature, stiff and short neck and restricted mouth opening. He scheduled for left inguinal hernia repair surgery.
  
  
  CONCLUSIONS
  The patient's difficult airway was managed successfully and the anesthesia of his surgical procedure had an uneventful course.
authors.affiliation: Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.
authors.forename: Mohsen
authors.initials: M
authors.lastname: Ziyaeifard
authors.affiliation: Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.
authors.forename: Rasoul
authors.initials: R
authors.lastname: Azarfarin
authors.affiliation: Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.
authors.forename: Rasoul
authors.initials: R
authors.lastname: Ferasatkish
authors.affiliation: Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, IR Iran.
authors.forename: Majid
authors.initials: M
authors.lastname: Dashti
keywords: Airway Management; Anesthesia; Laryngeal Masks; Mucopolysaccharidosis