Management of a Difficult Airway Scenario in a Case of Hurler’s Syndrome with a D-Blade Video Laryngoscope

Hurler’s syndrome is a rare genetic disease characterized by progressive multiorgan accumulation of glycosaminoglycans. It is associated with progressive craniofacial, skeletal, and cardiac involvement, which increases the risk of anesthesia. Patients with Hurler’s syndrome could present as the wors...

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Bibliographic Details
Main Authors: Keta D. Thakkar, Ajay P. Hrishi, Manikandan Sethuraman, Smita Vimala
Format: article
Language:EN
Published: Thieme Medical and Scientific Publishers Pvt. Ltd. 2020
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Online Access:https://doaj.org/article/b71780bc834a4cee88c674b3fa258c06
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Summary:Hurler’s syndrome is a rare genetic disease characterized by progressive multiorgan accumulation of glycosaminoglycans. It is associated with progressive craniofacial, skeletal, and cardiac involvement, which increases the risk of anesthesia. Patients with Hurler’s syndrome could present as the worst airway management problem an anesthesiologist could deal with due to abnormal upper airway anatomy and limited neck manipulation, owing to the atlantoaxial instability. We report a case of difficult airway scenario of a child with Hurler’s syndrome, leading to an apparent cannot ventilate cannot intubate scenario, which was managed successfully with the help of a C-MAC video laryngoscope with unique D-blade. In Hurler’s syndrome, C-MAC with D-blade is an excellent tool in establishing an airway in a pediatric difficult airway scenario. Moreover, D-blade C-MAC could be considered as the primary tool for establishing an airway in pediatric patients with Hurler’s syndrome.