Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II
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Case Report
VOLUME: 6 ISSUE: 2
P: 113 - 116
August 2019

Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II

J Pediatr Emerg Intensive Care Med 2019;6(2):113-116
1. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Çocuk Acil Bilim Dalı, Ankara, Türkiye
2. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Ankara, Türkiye
3. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Metabolizma ve Beslenme Ünitesi, Ankara, Türkiye
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Received Date: 22.02.2018
Accepted Date: 15.05.2018
Publish Date: 17.06.2019
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ABSTRACT

Mucopolysaccharidosis type II (MPS II) is a rare, X-linked recessive disease characterized by deficiency of lysosomal iduronate-2-sulfatase. Progressive upper airway obstruction is common in patients with MPS II. Tracheostomy is an effective way of managing airway obstruction when less invasive interventions are no longer adequate. Here, we report a patient with MPS II who developed sudden respiratory distress in the emergency department and required tracheostomy as endotracheal intubation and laryngeal mask airway (LMA) failed to secure the airway.

Keywords:
Mucopolysaccharidosis, Hunter syndrome, airway, tracheostomy

References

1
Schwartz IV, Ribeiro MG, Mota JG, Toralles MB, Correia P, Horovitz D, et al. A clinical study of 77 patients with mucopolysaccharidosis type II. Acta Paediatr. 2007;96:63-70.
2
Tuschl K, Gal a, Paschke E, Kircher S, Bodamer OA. Mucopolysaccharidosis type II in females: case report and review of literature. Pediatr Neurol. 2005;32:270-2.
3
Neufeld E and Muenzer J. The mucopolysaccharidoses. In Scriver CR, Beaudet AL, Sly WS, Valle D, eds. Childs B, Kinzler KW, Vogelstein B, assoc eds. The Metabolic and Molecular Basis of Inherited Disease, 8th ed. New york: Mc Graw-Hill, 2001. p.3421-54.
4
Herrick LA, Rhine EJ. The Mucopolysaccharidoses and anaesthesia: A report of clinical experience. Can J Anaesth. 1988;35:67-73.
5
Donoghue A, Nagler J, Yamamato LG: Chapter 3 Airway. In: Bachur RG and Shaw KN (Ed.): Fleisher & Ludwig's textbook of pediatric emergency medicine. Baltimore, Lippincott Williams & Wilkins, 2015;7:94-109.
6
Walker RW, Darowski M, Morris P, Wraith JE. Anaesthesia and mucopolysaccharidoses. A review of airway problems in children. Anaesthesia. 1994;49:1078-84.
7
Chen CH, Huang GS, Lee CK, Huang YS, Wong CS. Use of Laryngeal Mask airway for the resuscitation of a Hunter Syndrome patient during general anaesthesia induction. J Med Sci. 2003;23:351-4.
8
Busoni P, Fognani G. Failure of the laryngeal mask to secure the airway in a patient with Hunter's syndrome (mucopolysaccharidosis type II) Pediatr Anaesth. 1999;9:153-5.
9
Malik V, Nichani J, Rothera MP, Wraith JE, Jones SA, et al. Tracheostomy in mucopolysaccharidosis type {II} (Hunter's syndrome) Int. J. Pediatr. Otorhinolaryngol. 2013;77:1204-8.
10
Gönüldaş B, Yılmaz T, Sivri HS, Güçer KŞ, Kılınç K, et al. Mucopolysaccharidosis: Otolaryngologic findings, obstructive sleep apnea and accumulation of glucosaminoglycans in lymphatic tissue of the upper airway. Int J Pediatr Otorhinolaryngol. 2014;78:944-9.
11
Diaz JH, Belani KG. Perioperative management of children with Mucopolysaccharidoses. Anesth Analg. 1993;77:1261-70.
12
Taguchi S, Kusunoki S, Fukuda H, Hamada H, Kawamoto M. Difficult tracheal intubation using airway scope in a pediatric patient with Hunter syndrome. Masui. 2009;58:1278-81.