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

Endoscopia de vias aéreas com paciente acordado em mucopolissacaridose: relato de caso

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

Resumo

As mucopolissacaridoses (MPS) são um grupo de doenças hereditárias genéticas raras com curso progressivo devido ao acúmulo de glicosaminoglicanos resultando em anormalidades anatômicas e disfunção de órgãos, incluindo os sistemas respiratório, cardiovascular, esquelético e neurológico que podem aumentar o risco de complicações anestésicas. As manifestações clínicas são variáveis, multissistêmicas e incluem alterações morfológicas graves. O manejo anestésico desses pacientes é complexo, particularmente o manejo das vias aéreas, que pode ser planejado para incluir uma investigação das vias aéreas com fibra óptica antes da cirurgia. Apresentamos dois casos de pacientes com MPS tipo VI e VII submetidos ao mapeamento de vias aéreas por fibra óptica sob sedação consciente, sem complicações. Como a MPS é uma doença rara, mas desafiadora no que diz respeito ao manejo das vias aéreas, propomos uma técnica anestésica segura e eficaz que pode ser usada para fibrobroncoscopia e permite a intubação traqueal assistida por fibra óptica no momento da cirurgia.

Palavras-chave

Mucopolissacaridose; Via aérea; Anestesia; Relato de caso

References

1. Muenzer J. Overview of the mucopolysaccharidoses. Rheumatology (Oxford). 2011;50:v4–12.

2. Gönüldas¸ B, Yılmaz T, Sivri HS, et al. Mucopolysaccharidosis: otolaryngologic findings, obstructive sleep apnea and accumulation of glycosaminoglycans in lymphatic tissue of the upper airway. Int J Pediatr Otorhinolaryngol. 2014;78:944.

3. Walker R, Belani KG, Braunlin EA, et al. Anaesthesia and airway management in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36:211.

4. National Library of Medicine. https://ghr.nlm.nih.gov/ condition/mucopolysaccharidosis-type-vi#statistics, 2020.

5. National Library of Medicine. https://ghr.nlm.nih.gov/ condition/mucopolysaccharidosis-type-vii#statistics, 2020.

6. Berger KI, Fagondes SC, Giugliani R, et al. Respiratory and sleep disorders in mucopolysaccharidosis. J Inherit Metab Dis. 2013;36:201–10.

7. Clark BM, Sprung J, Weingarten TN, et al. Anesthesia for patients with mucopolysaccharidoses: comprehensive review of the literature with emphasis on airway management. Bosn J Basic Med Sci. 2018;18:1–7.

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