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Anesthetic Management for Pediatric Liver Transplantation in a Patient With Propionic Acidemia: A Case Report

Luis I Rodriguez1, Erica J Tainsh1, Eliane Varga1

  • 1University of Miami, Miami, FL, USA.

Journal of Investigative Medicine High Impact Case Reports
|May 12, 2021
PubMed

Insights

Orthotropic liver transplantation offers improved outcomes for propionic acidemia, a metabolic disorder. Monitoring metabolic markers during surgery and early extubation are key for successful recovery in these patients.

Area of Science:

  • Biochemistry
  • Genetics
  • Pediatrics

Background:

  • Propionic acidemia is an inherited metabolic disorder caused by propionyl-CoA carboxylase deficiency, leading to toxic propionic acid accumulation.
  • Current management primarily involves dietary protein restriction, which has limitations in preventing severe metabolic crises.

Observation:

  • A case report details a 4-year-old boy with propionic acidemia treated with orthotropic liver transplantation.
  • Intraoperative monitoring of urine ketones, arterial ammonia, and lactate levels was crucial due to the patient's risk of hyperammonemia and metabolic acidosis.

Findings:

  • Orthotropic liver transplantation was performed to manage propionic acidemia.
  • The study emphasizes the importance of vigilant intraoperative metabolic monitoring in patients with propionic acidemia undergoing transplantation.
  • Early extubation and fast-tracking recovery were associated with positive outcomes.

Implications:

  • Liver transplantation represents a viable therapeutic option for propionic acidemia, potentially improving long-term prognosis.
  • Close monitoring of metabolic parameters during surgical procedures is essential for managing patients with inborn errors of metabolism.
  • Optimized perioperative care, including early extubation, can enhance recovery trajectories in pediatric transplant recipients.

Keywords:
pediatric anesthesiapediatric liver transplantationpediatric metabolic disorderpropionic acidemia

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