Liver transplantation

Orthotopic liver transplantation (OLT) has become a relatively safe procedure over the last decades, with reported 5-year survival rate of over 80%.    Increasing numbers of patients will be transplated at a younger age and they will seek obstetric anaesthesia services, as their fertility is restored after succesful transplantation. In 1978, the first succesful pregnancy was reported. Continue reading
Glycogen storage disease type Ia (GSD-Ia; Von Gierke disease) is an inherited disease caused by glucose-6-phosphatase deficiency. Perioperative management of patients with GSD has important implications for anaesthesiologists due to different system involvements. Continue reading
Glycogen storage disease type 1a is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Continue reading

Neurologic complications after liver transplantation are quite common, with central pontine myelinolysis (CPM) being a rare but fatal complication. In this report, we describe the case of female liver transplant recipient who developed CPM after orthotopic liver transplantation (OLT).A 62-year old woman was admitted to the ICU for postoperative recovery after OLT. The procedure was described as uneventful. The patient had a history of decompensated cirrhosis, tension ascites, class I hepatic encephalopathy and chronic hyponatremia which necessitated repeated hospitalizations in the year prior to transplantation.The patient was unable to be weaned from ventilator support and did not show an improvement in mental status (GCS=5) despite the cessation of sedation. After neurologic consultation, a brain MRI was performed which showed evidence of CPM in the pons. Despite supportive therapy, the patient did not show an improvement of mental status and after a 55-day hospitalization in the ICU she died from septic complications.The etiology of CPM is multifactorial, with liver transplant recipients being at an increased risk. Supportive treatment is the standard of care, and there are not enough evidence supporting other types of treatment.

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