[Anesthesia for eye operations in mitochondrial encephalomyelopathy]

Mitochondrial encephalomyopathy involves a disturbance of the mitochondrial respiratory chain, as a result of which the blood lactate level is elevated. In stress situations a lactate acidosis can occur. The disease may be subdivided into three main syndromes: Kearns-Sayre syndrome (KKS), "myoclonus epilepsy with ragged red fibers syndrome" (MERRF), and "mitochondrial myopathy, encephalopathy, lactic acidosis and strokelike episodes syndrome" (MELAS). There are also several intermediate forms. Ophthalmological symptoms are frequent and occasionally have to be treated surgically. A 20-year-old male patient with a mixed form of these syndromes including elements of KSS and MERRF had to undergo cataract extraction. The authors decided to perform the operation under local anesthesia and sedation, with the anesthetist on standby. No problems arose. In all cases where mitochondrial encephalomyopathy is suspected the diagnosis should be confirmed by a muscle biopsy and the risk of cardiac arrest, respiratory insufficiency, and epileptic seizures ruled out prior to surgery. Local anesthesia with sedation appears to be the most favorable form of anesthesia provided the maximum dose is observed and a substance with a high convulsion threshold is chosen. Perioperative monitoring by an anesthetist and temporary provision of a cardiac pacemaker are necessary.(ABSTRACT TRUNCATED AT 250 WORDS)