06 October 2009
Perioperative care in alpers syndrome carrier: Case presentation and literature survey
Youri Vater, Gregory Dembo, Yifat Klein, Kenneth Martay, Wisam Khoury, Alex Vitin, Daniel Ogorek, Avi A. WeinbroumAm J Case Rep 2009; 10:155-160 :: ID: 878219
Abstract
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.
Keywords: mitochondrial myopathy, Alpers syndrome, Surgery, Transplantation, Liver, Perioperative Care
In Press
26 Mar 2024 : Case report
Intrabiliary Rupture of a Hepatic Hydatid Cyst: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.943893
27 Mar 2024 : Case report
Surgical Correction of Ischiopagus Tripus Conjoined Twins with Fused Pelvis: Enhancing Quality of Life thro...Am J Case Rep In Press; DOI: 10.12659/AJCR.942126
27 Mar 2024 : Case report
A 16-Year-Old Girl with Sinonasal Cutaneous Fistula Following Excision and Radiotherapy for Rhabdomyosarcom...Am J Case Rep In Press; DOI: 10.12659/AJCR.943098
27 Mar 2024 : Case report
Congenital Dislocation of the Knee in the Delivery RoomAm J Case Rep In Press; DOI: 10.12659/AJCR.943725
Most Viewed Current Articles
07 Mar 2024 : Case report
Neurocysticercosis Presenting as Migraine in the United StatesDOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
10 Jan 2022 : Case report
A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...DOI :10.12659/AJCR.935263
Am J Case Rep 2022; 23:e935263
19 Jul 2022 : Case report
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
23 Feb 2022 : Case report
Penile Necrosis Associated with Local Intravenous Injection of CocaineDOI :10.12659/AJCR.935250
Am J Case Rep 2022; 23:e935250