Can inhalation agents be used in the presence of a child with myopathy?

Abstract

Only a few muscle diseases are really associated with a risk of malignant hyperthermia. The risk of rhabdomyolysis is more difficult to clarify and a multicentric database would be useful to evaluate the risk/benefit ratio of all anaesthetic drugs in patients with muscle diseases.

Anesthetic considerations in myofibrillar myopathy

“…Myofibrillar myopathy (MFM) is not associated with RYR1 or CACNA1S mutations, and MH rarely presents with a myopathic phenotype . Some ultrastructural findings do overlap between MFM and myopathies with MH risk: central core disease and a newly described recessive RYR1 mutation have ultrastructural features such as Z‐disk extensions and myofibrillar disorganization, but other ultrastructural findings are exclusive between these diseases and MFM .…”

“…Although the mechanism is unknown, volatile anesthetics have been implicated in a minority of cases of AIR, predominately in patients with muscular dystrophy . Thus, practitioners have classically avoided volatile anesthetics for patients with dystrophinopathies or in the setting of undiagnosed muscle weakness.…”

The syndromic child and anaesthesia

“…Two congenital myopathies, namely central core disease and a subset The page number in the footer is not for bibliographic referencing www.tandfonline.com/ojaa 17 200 of multiminicore myopathy, have an increased risk of malignant hyperthermia. 24 King-Denborough syndrome has a similar phenotype to Noonan syndrome, with the addition of congenital myopathy which causes proximal weakness and a high risk of malignant hyperthermia. 24,25 Native American myopathy has a similar phenotype, whereby patients present with a cleft palate, a higharched palate, micrognathia, myopathic facies, a short stature, scoliosis, cryptorchidism, arthrogryposis and clubbed feet.…”

“…24 King-Denborough syndrome has a similar phenotype to Noonan syndrome, with the addition of congenital myopathy which causes proximal weakness and a high risk of malignant hyperthermia. 24,25 Native American myopathy has a similar phenotype, whereby patients present with a cleft palate, a higharched palate, micrognathia, myopathic facies, a short stature, scoliosis, cryptorchidism, arthrogryposis and clubbed feet. Those affected are also at high risk of developing malignant hyperthermia.…”

“…24 Those with the enzymopathy, carnitine palmitoyltransferase II deficiency, may have acute rhabdomyolysis in response to exercise, fasting, stress and infection. Therefore, it is prudent to avoid inhalational agents and succinylcholine in these patients.…”