Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2021.06.005
Brazilian Journal of Anesthesiology
Case Report

Awake airway endoscopy in mucopolysaccharidosis: a case report

Sofia Fernandes; João Borges; Marta Martins

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Abstract

Mucopolysaccharidosis (MPS) are a group of rare genetic inherited diseases with a progressive course due to the accumulation of glycosaminoglycans resulting in anatomic abnormalities and organ dysfunction, including the respiratory, cardiovascular, skeletal, and neurological systems that can increase the risk of anesthesia complications. Clinical manifestations are variable, multisystemic, and include severe morphological changes. The anesthetic management of these patients is complex, particularly airway management, which can be planned to include a fiberoptic airway investigation prior to surgery. We present two cases of patients with MPS type VI and VII who underwent fiberoptic airway mapping under conscious sedation, with no complications. Since MPS is a rare but challenging disease concerning the airway management, we propose a safe and effective anesthetic technique that could be used for fiberoptic bronchoscopy and allow fiberoptic-assisted tracheal intubation at the time of surgery.

Keywords

Mucopolysaccharidosis;  Airway;  Anesthesia;  Case report

References

J. Muenzer Overview of the mucopolysaccharidoses Rheumatology (Oxford), 50 (2011), pp. v4-v12

2 B. Gönüldaş, T. Yılmaz, H.S. Sivri, et al. Mucopolysaccharidosis: otolaryngologic findings, obstructive sleep apnea and accumulation of glycosaminoglycans in lymphatic tissue of the upper airway Int J Pediatr Otorhinolaryngol, 78 (2014), p. 944

3 R. Walker, K.G. Belani, E.A. Braunlin, et al. Anaesthesia and airway management in mucopolysaccharidosis J Inherit Metab Dis, 36 (2013), p. 211

4 National Library of Medicine Mucopolysaccharidosis type VI https://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi#statistics (2020) . (Accessed 23 May 2020)

5 National Library of Medicine Mucopolysaccharidosis type VII https://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vii#statistics (2020) . (Accessed 23 May 2020)

6 K.I. Berger, S.C. Fagondes, R. Giugliani, et al. Respiratory and sleep disorders in mucopolysaccharidosis J Inherit Metab Dis, 36 (2013), pp. 201-210

7 B.M. Clark, J. Sprung, T.N. Weingarten, et al. Anesthesia for patients with mucopolysaccharidoses: comprehensive review of the literature with emphasis on airway management Bosn J Basic Med Sci, 18 (2018), pp. 1-7
 

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Braz J Anesthesiol

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