I read with great interest the short report published in the Images in Anesthesiology section about difficult intubation in a 2-yr-old patient with Ullrich congenital muscular dystrophy.1 This disease is well known to carry a risk of difficult intubation.2–6 The authors nicely described how they used a nasopharyngeal airway to administer a volatile anesthetic and oxygen through one nostril while performing nasotracheal fiberoptic intubation via the other. I am however surprised that the choice of the anesthetic agent(s) used was not discussed. Ullrich congenital muscular dystrophy indeed belongs to the subgroup of the collagen type 6–related myopathies occurring after a mutation of the COL6A1, COL6A2, or COL6A3 gene. Collagen type 6 is part of the large complex that anchors the basal lamina and the interstitium in muscle cells. The myopathy is probably caused by the...
Anesthesia and Ullrich Congenital Muscular Dystrophy: Comment
(Accepted for publication January 6, 2021. Published online first on February 2, 2021.)
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Francis Veyckemans; Anesthesia and Ullrich Congenital Muscular Dystrophy: Comment. Anesthesiology 2021; 134:813 doi: https://doi.org/10.1097/ALN.0000000000003698
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