title: Emergency bronchoscopy for foreign-body aspiration in a child with type I mucopolysaccharidosis: a challenging airway management experience.
abstract: The mucopolysaccharidosis (MPS) is a rare lysosomal storage disease. Glycosaminoglycans (GAG) accumulate in musculoskeletal system, connective tissues. Enlarged tongue, short immobile neck, and limited mobility of the cervical spine and temporomandibular joints render the airway management potentially risky. MPS children have high anesthetic risks, especially in airway management of emergency situations. The foreign-body aspiration requiring intervention with rigid bronchoscopy is an urgent and risky clinical situation. We present our experience with a challenging airway management with a three-year-old child with MPS who needed emergency bronchoscopy due to peanut aspiration.
authors.affiliation: Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey. pinarken@gmail.com.
authors.forename: Pinar
authors.initials: P
authors.lastname: Kendigelen
authors.affiliation: Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey.
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Tunali
authors.affiliation: Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey.
authors.forename: Ayse
authors.initials: A
authors.lastname: Tutuncu
authors.affiliation: Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey.
authors.forename: Gulruh
authors.initials: G
authors.lastname: Ashyralyyeva
authors.affiliation: Department of Pediatric Surgery, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey.
authors.forename: Senol
authors.initials: S
authors.lastname: Emre
authors.affiliation: Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey.
authors.forename: Guner
authors.initials: G
authors.lastname: Kaya
mesh_terms: D058109:Airway Management / Q000379:methods*; D001980:Bronchi; D001999:Bronchoscopy / Q000379:methods*; D002675:Child, Preschool; D005547:Foreign Bodies / Q000150:complications*; D006801:Humans; D008260:Macroglossia / Q000150:complications / Q000151:congenital / Q000209:etiology; D008297:Male; D008059:Mucopolysaccharidosis I / Q000150:complications*
keywords: Airway management; Mucopolysaccharidosis; Sugammadex