• Journal of anesthesia · Aug 2016

    Case Reports

    Emergency bronchoscopy for foreign-body aspiration in a child with type I mucopolysaccharidosis: a challenging airway management experience.

    • Pinar Kendigelen, Yusuf Tunali, Ayse Tutuncu, Gulruh Ashyralyyeva, Senol Emre, and Guner Kaya.
    • Department of Anesthesiology and Intensive Care, Cerrahpasa Medical Faculty, Istanbul University, Kocamustafapasa, Fatih, 34098, Istanbul, Turkey. pinarken@gmail.com.
    • J Anesth. 2016 Aug 1; 30 (4): 696-8.

    AbstractThe 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.

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