• A&A practice · Apr 2022

    Case Reports

    Anesthetic Management of a Patient With S-Adenosylhomocysteine Hydrolase Deficiency: A Case Report.

    • Cori R Van Gorkom, Emily D Black, and Joelle B Karlik.
    • From the Departments of Anesthesiology and Pain Medicine.
    • A A Pract. 2022 Apr 8; 16 (4): e01578.

    AbstractS-adenosylhomocysteine hydrolase (AHCY) deficiency is a rare congenital disorder in methionine metabolism with minimal guidelines regarding anesthetic management. This case report describes a 19-year-old man presenting for a liver biopsy in interventional radiology due to a history of elevated aminotransferases and creatine kinase. He received dextrose-containing fluids and a total intravenous anesthetic to avoid rhabdomyolysis and hyperkalemia. Anesthetic goals for patients with AHCY deficiency should focus on avoiding rhabdomyolysis, minimizing postoperative ventilatory compromise, monitoring for potential coagulopathy, and providing anxiolysis.Copyright © 2022 International Anesthesia Research Society.

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