Complicated peripartum course in a patient with very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency

Neuromuscul Disord. 2021 Jun;31(6):566-569. doi: 10.1016/j.nmd.2021.03.015. Epub 2021 Apr 9.


Very long-chain acyl-coenzyme A (CoA) dehydrogenase (VLCAD) deficiency is an autosomal recessive fatty acid oxidation disorder characterized by rhabdomyolysis, hypoglycemia and cardiomyopathy. The general treatment approach in adult patients is based on the prevention of catabolism. High carbohydrate, low fat diet and supplementation of medium-chain triglycerides are essential in the treatment. There is little experience with pregnancy follow-up in this patient group. We present a complicated peripartum course and successful management in a patient with VLCAD deficiency. Although high-dose glucose infusion was initiated, creatine kinase levels significantly increased in the immediate postpartum period, but the patient remained asymptomatic and rhabdomyolysis resolved rapidly after increasing the glucose infusion rate.

Keywords: Creatine kinase; Pregnancy; Rhabdomyolysis; VLCAD; Very long-chain acyl-coenzyme A dehydrogenase deficiency.

Publication types

  • Case Reports

MeSH terms

  • Acyl-CoA Dehydrogenase, Long-Chain / deficiency
  • Adult
  • Congenital Bone Marrow Failure Syndromes / therapy*
  • Female
  • Humans
  • Lipid Metabolism, Inborn Errors / therapy*
  • Mitochondrial Diseases / therapy*
  • Muscular Diseases / therapy*
  • Peripartum Period*
  • Pregnancy
  • Rhabdomyolysis / therapy


  • Acyl-CoA Dehydrogenase, Long-Chain

Supplementary concepts

  • VLCAD deficiency