Anesthesia and Ullrich Congenital Muscular Dystrophy: Comment.

Abstract

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 muscle membrane fragility and an associated mitochondrial dysfunction, which can be decreased with cyclosporine A. As collagen 6 is close to the dystrophin-glycoprotein complex, this muscle disease could be at risk of anesthesia-induced rhabdomyolysis in the presence of halogenated agents or succinylcholine, as are children with Duchenne or Becker progressive muscular dystrophy. Very few reports on the anesthetic management of patients with this type of myopathy have been published so far and all except two5,6 report using total intravenous anesthesia.2–4 Carefully titrated intravenous anesthesia to maintain spontaneous ventilation and using either dexmedetomidine, propofol, and/or ketamine could therefore be a safe alternative.Dr. Veyckemans reports a financial relationship with European Journal of Anaesthesiology’s Editorial Board and Pediatric Anesthesia’s Editorial Board.