Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2021.03.018
Brazilian Journal of Anesthesiology
Case Report

A difficult airway approach in a merosin-deficient congenital muscular dystrophy patient: a case report

Abordagem de via aérea difícil em paciente com distrofia muscular congênita deficiente em merosina: relato de caso

Jorge Pelicano Paulos, Vanessa Artilheiro, Catarina Cruz, Ana Pinto Carneiro

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Abstract

Merosin-deficient muscular dystrophy is caused by an autosomal recessive mutation on laminin-α2 gene characterized by severe progressive muscle weakness associated with neuromuscular scoliosis and restrictive lung disease. In this case report, we describe an alternative airway approach performed in a child with anticipated difficult airway and merosin-deficient muscular dystrophy. Significant anesthetic implications may increase the perioperative risk, requiring accurate knowledge to anticipate an adequate management and provide patient-safety strategies.

Keywords

Pediatrics,  Orthopedics,  Airway management,  Intravenous anesthesia,  Muscular dystrophy congenital, merosin negative

Resumo

A distrofia muscular deficiente em merosina é causada por uma mutação autossômica recessiva no gene da laminina-α2, caracterizada por fraqueza muscular progressiva grave associada à escoliose neuromuscular e doença pulmonar restritiva. Neste relato de caso, descrevemos uma abordagem alternativa das vias aéreas realizada em uma criança com via aérea difícil prevista e distrofia muscular deficiente em merosina. Implicações anestésicas significativas podem aumentar o risco perioperatório, exigindo conhecimento preciso para antecipar um manejo adequado e fornecer estratégias de segurança ao paciente.

Palavras-chave

Pediatria; Ortopedia; Gerenciamento de vias aéreas; Anestesia intravenosa; Distrofia muscular congênita, merosina negativa

References

1. N. Jimenez, K. Song, A.M. Lynn Hemodynamic instability during prone spine surgery in a patient with merosin-deficient congenital muscular dystrophy Paediatr Anaesth, 23 (2013), pp. 294-296

2. C. Pregardien, T. Pirotte, F. Veyckemans Pressure-support ventilation in a child with merosin-deficient congenital muscular dystrophy under sevoflurane anesthesia Acta Anaesthesiol Belg, 67 (2016), pp. 139-141

3. P.C. Echeverry-Marín, Á.M. Bustamante-Vega Anesthetic implications of muscular dystrophies Rev Colomb Anestesiol, 46 (2018), pp. 228-239

4. A.A. Matioc Use of the Airtraq® with a fibreoptic bronchoscope in a difficult intubation outside the operating room Can J Anesth, 55 (2008), pp. 561-562

5. F. Sgalambro, F. Sanfilippo, C. Santonocito, C. Caltavuturo, C. Grillo Virtual laryngoscopy and combined laryngoscopic-bronchoscopic approach for safe management of obstructive upper airways lesions Br J Anaesth, 113 (2014), pp. 304-306
 

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