Perioperative care in alpers syndrome carrier: Case presentation and literature survey

Background: Mitochondrial disorders encompass progressive multi-systemic disabling neurological disorders, involving muscles, brain, kidney and the liver. Patients with mitochondrial disorders are at risk of metabolic decompensation when under stress or when requiring general anesthesia. Due to multiorgan dysfunction, orthotopic liver transplantation (OLT) may be optional when anticonvulsive drug-induced fulminant hepatic failure (FHF) ensues. Case Report: A 23-years old Caucasian woman has been suffering from a mitochondrial disease involving Complex III electron transport chain deficiency comparable with the Alpers syndrome. Seizures and sensory neuropathy were ultimately treated by valproic acid (VPA, 250 plus 125 mg/d), which eventually caused subacute then FHF; VPA was stopped. Twenty-four hours prior to surgery the patient deteriorated and required cardiopulmonary resuscitation (CPR). She was placed on amiodarone (0.5 mg/min), which regained sinus rhythm, intracranial pressure (ICP) bolt monitor was inserted, and airways were protected. Soon afterwards OLT was performed under propofol (2-4 mg/kg/h) for brain protection and anesthesia, remifentanil, and oxygen. In the ICU, propofol was stopped and fentanyl was started, which enabled the patient to regain consciousness and follow commands. However, myoclonic jerks progressively increased, requiring the reestablishment of VPA; despite this and the fentanyl therapy seizures continued and her weaning off the ventilator became impossible. The patient developed meningitis and pneumonia and expired at day 32 postoperatively. Conclusions: Stress and surgery in mitochondrial myopathy sufferes, especially if urgent, pose challenges to the anesthesiologist. Total IV anesthesia seems remarkably safe, including prolonged use of propofol. Geneticians, neurologists and surgeons may aid the anesthesiologists to individually-tailor perioperative care to patients with metabolic syndromes.