A team of researchers from Portugal reported the case of a 10-year-old male with very long-chain acyl-coenzyme a dehydrogenase (VLCAD) deficiency, a type of long-chain fatty acid oxidation disorder (LCFAOD), who was successfully managed with volatile anesthetics.
The team described the challenges that they encountered and the strategies that they used to deal with them.
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The study is published in Revista Española de Anestesiología y Reanimación (English Edition).
The patient was hospitalized due to rhabdomyolysis and was undergoing a totally implanted central venous catheter placement to allow the rapid start of treatment in decompensation events that he might encounter in the future. This decision was taken because he had had multiple previous hospitalizations requiring the placement of a temporary central venous catheter.
He was successfully managed with general anesthesia with nitrous oxide, sevoflurane, and remifentanil. He was also infused with glucose during the operation to avoid metabolic decompensation. Hypoglycemia did not occur and the levels of creatine kinase and lactate in his blood did not increase.
The authors concluded that volatile anesthetics seem to be a safe option and “may have been erroneously avoided in the past.” They added that more research is necessary to provide validated guidelines so that outcomes can be improved in patients with LCFAOD undergoing general anesthesia.
LCFAOD is a group of rare genetic diseases characterized by impaired fat metabolism leading to acute crises of energy production and chronic energy deficiency. There are 6 types of LCFAOD each caused by a mutation in genes coding for mitochondrial enzymes that are involved in energy metabolism during fasting and physiologic stress.
VLCAD deficiency is caused by a mutation in the ACADVL gene, which encodes the enzyme needed for the beta-oxidation of fatty acids inside the mitochondria.
Reference
Peixoto C, Dourado C, Santos H, Roberto P. Perioperative anesthetic management of very long-chain acyl-coenzyme a dehydrogenase deficiency. Rev Esp Anestesiol Reanim (EN). Published online February 25, 2023. doi:10.1016/j.redare.2021.12.005
