Anesthetic management of a patient with isovaleric acidemia

A A Case Rep. 2015 Feb 1;4(3):37-8. doi: 10.1213/XAA.0000000000000096.


A 3-year-old male with isovaleric acidemia presented for dental rehabilitation under general anesthesia. In times of stress, such as in the perioperative period, patients with isovaleric acidemia are at greater risk for morbidity and mortality from disordered metabolism, including glucose disturbances, hyperammonemia, hypocalcemia, and non-anion gap metabolic acidosis. Communication between the anesthesiology, dental, and endocrine teams allowed for safe and successful care of the patient.

Publication types

  • Case Reports

MeSH terms

  • Amino Acid Metabolism, Inborn Errors / complications*
  • Amino Acid Metabolism, Inborn Errors / diagnosis
  • Amino Acid Metabolism, Inborn Errors / metabolism
  • Amino Acid Metabolism, Inborn Errors / therapy
  • Anesthesia, General* / adverse effects
  • Child, Preschool
  • Dental Caries / complications
  • Dental Caries / diagnosis
  • Dental Caries / surgery*
  • Humans
  • Interdisciplinary Communication
  • Isovaleryl-CoA Dehydrogenase / deficiency*
  • Isovaleryl-CoA Dehydrogenase / metabolism
  • Male
  • Oral Surgical Procedures* / adverse effects
  • Patient Care Team
  • Preoperative Care
  • Risk Assessment
  • Risk Factors
  • Treatment Outcome


  • Isovaleryl-CoA Dehydrogenase

Supplementary concepts

  • Acidemia, isovaleric