Email this article Tonsillectomy in a pediatric patient with propionic acidemia: Anesthesia management and potential perioperative challenges
DOI: https://doi.org/10.20528/cjpm.2025.02.006
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Propionic acidemia (PA) is a rare metabolic disorder caused by a deficiency in the propionyl-CoA carboxylase enzyme, leading to the accumulation of toxic metabolites. Surgical procedures pose a risk of metabolic decompensation in patients with PA, requiring careful anesthesia management. A four-year-old male patient with propionic acidemia was scheduled for tonsillectomy due to recurrent upper respiratory tract infections. Midazolam was administered for premedication, and anesthesia induction was achieved with thiopental and rocuronium. Anesthesia maintenance was provided with sevoflurane, and fentanyl was used for analgesia. Postoperative pain management included paracetamol. The procedure was completed without complications, and the patient was transferred to the ward in stable condition. Anesthesia management in PA patients should focus on maintaining metabolic stability. This case demonstrates that with proper preoperative preparation, careful anesthesia management, and close perioperative monitoring, surgical interventions can be safely performed in pediatric patients with PA.
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