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CC BY-NC-ND 4.0 · J Neuroanaesth Crit Care 2021; 08(03): 214-216
DOI: 10.1055/s-0040-1714451
Case Report

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

Keta D. Thakkar 
1   Department of Anaesthesiology, Division of Neuroanesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
,
Ajay P. Hrishi 
1   Department of Anaesthesiology, Division of Neuroanesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
,
Manikandan Sethuraman 
1   Department of Anaesthesiology, Division of Neuroanesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
,
Smita Vimala 
1   Department of Anaesthesiology, Division of Neuroanesthesia, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India
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Abstract

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.

Keywords

Hurler’s syndrome - pediatric difficult airway - D-blade C-MAC


Publication History

Article published online:
10 August 2020

© 2020. Indian Society of Neuroanaesthesiology and Critical Care. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

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  • References

  • 1 Spinello CM, Novello LM, Pitino S. et al Anesthetic management in mucopolysaccharidoses. ISRN Anesthesiol 2013; Doi: DOI: 10.1155/2013/791983.
    CrossrefPubMedGoogle Scholar
  • 2 Clark BM, Sprung J, Weingarten TN, Warner ME. Anesthesia for patients with mucopolysaccharidoses: Comprehensive review of the literature with emphasis on airway management. Bosn J Basic Med Sci 2018; 18 (01) 1-7
    CrossrefPubMedGoogle Scholar
  • 3 Gupta AK, Kharde V, Gawer V, Sidhaye RV, Divekar DS. Hurler’s syndrome: anaesthetic challenges and management. Anestesia Pediatrica e Neonatale 2010; 8 (02) 1-8
    PubMedGoogle Scholar
  • 4 Nicolson SC, Black AE, Kraras CM. Management of a difficult airway in a patient with Hurler-Scheie syndrome during cardiac surgery. Anesth Analg 1992; 75 (05) 830-832
    CrossrefPubMedGoogle Scholar
  • 5 Gupta N, Rath GP, Bala R, Reddy BK, Chaturvedi A. Anesthetic management in children with Hurler’s syndrome undergoing emergency ventriculoperitoneal shunt surgery. Saudi J Anaesth 2012; 6 (02) 178-180
    CrossrefPubMedGoogle Scholar
  • 6 Busoni P, Fognani G. Failure of the laryngeal mask to secure the airway in a patient with Hunter’s syndrome (mucopolysaccharidosis type II). Paediatr Anaesth 1999; 9 (02) 153-155
    CrossrefPubMedGoogle Scholar
  • 7 Kılıçaslan A, Topal A, Erol A, Uzun ST, Tuncer S, Uzen ST. Comparison of the C-MAC D-Blade, conventional C-MAC, and Macintosh laryngoscopes in simulated easy and difficult airways. Turk J Anaesthesiol Reanim 2014; 42 (04) 182-189
    CrossrefPubMedGoogle Scholar
  • 8 Frerk C, Mitchell VS, McNarry AF. et al Difficult Airway Society intubation guidelines working group. Difficult Airway Society 2015 guidelines for management of unanticipated difficult intubation in adults. Br J Anaesth 2015; 115 (06) 827-848
    CrossrefPubMedGoogle Scholar