Anesthetic Management for orthotopic liver transplantation in a patient with Glycogen Storage Disease Type IIIa: A Case Report
Denada Haka
Başkent Universitesi Hastanesi
Giriş: Glycogen storage disease (GSD) type IIIa is a metabolic disorder caused by a deficiency of amyl-1,6- glucosidase enzyme, which is responsible for the breakdown of the glycogen molecule. As a result, glycogen accumulates in the organs and hypoglycemia, muscle weakness, liver dysfunction, delayed anesthetic recovery, excessive surgical bleeding, cardiomyopathy and end-organ dysfunction may happen. A multidisciplinary approach should be provided to optimize the preoperative period and minimize complications in these patients.
Olgu: A 4-years-9-months-old girl underwent orthotopic liver transplantation (OLT) from her mother. The American Society of Anesthesiologists physical classification of the patient was class IV. Standard monitoring was placed, including pulse oximetry, electrocardiogram, capnography, and invasive blood pressure. After preoxygenation by 80 % O2 for 3 min, general anesthesia was induced with propofol 2 mg/kg, fentanyl 1 μg/kg, and rocuronium 0.6 mg/kg followed by oral endotracheal intubation after the loss of train of four. General anesthesia was maintained with 2 % sevoflurane and 50 % oxygen in the air, remifentanil infusion (0.01-0.5 μg/kg per hour), and rocuronium infusion (0.3 mg/kg). Hemodynamic changes were monitored with Pulse index Contour Continuous Cardiac Output measured parameters, including stroke volume, stroke volume variation, pulse pressure variation, and cardiac output. Hemodynamic stability was maintained with 0.01-0.5 µg/kg/min norepinephrine infusion. . After the operation, sugammadex (2 mg/kg) was administered intravenously (i.v.). She was extubated in the operating room without delays
Tartışma ve Sonuç: In summary, meticulous preoperative planning should be done to minimize physical and psychological distress, retain optimum hydration status, avoid protein catabolism and prevent hypoglycemia attacks. Pediatric patients with GSD III should have their procedures done by trained multidisciplinary teams, including anesthesiologists, surgeons, pediatrists, and metabolic professionals at specialist centers. A perioperative comprehensive, detailed assessment and treatment for these patients are critical.
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