title: Management of an anticipated difficult airway in Hurler's syndrome.
abstract: Management of an anticipated difficult airway in Hurler syndrome. Hurler syndrome is a subtype of Mucopolysaccharidosis (MPS) type 1. Mucopolysaccharidosis (lysosomal storage diseases) are a group of inherited disorders caused by deficiency of specific lysosomal enzyme required for a normal degradation of glycosaminoglycons (GAGs). Administration of general anaesthesia in patients who have congenital syndromes such as Hurler's is often a challenge because of progressive airway, craniofacial and skeletal abnormalities that may make both the ventilation and intubation difficult. We encountered difficult mask ventilation and endotracheal intubation was not possible and finally ventilated with laryngeal mask airway in a known case of Hurler syndrome posted for umbilical hernia repair.
authors.affiliation: Department of Anaesthesiology, Father Muller Medical College, Mangalore, Karnataka, India.
authors.forename: T
authors.initials: T
authors.lastname: Gurumurthy
authors.affiliation: Department of Anaesthesiology, Father Muller Medical College, Mangalore, Karnataka, India.
authors.forename: S
authors.initials: S
authors.lastname: Shailaja
authors.affiliation: Department of Anaesthesiology, Father Muller Medical College, Mangalore, Karnataka, India.
authors.forename: Shetty
authors.initials: S
authors.lastname: Kishan
authors.affiliation: Department of Anaesthesiology, Father Muller Medical College, Mangalore, Karnataka, India.
authors.forename: Madona
authors.initials: M
authors.lastname: Stephen
keywords: Anesthesia; Hurler syndrome; difficult airway; mucopolysaccharidoses