Œdème avec déformation cornéenne d'aspect pseudo-kératoconique aigu au cours d'un syndrome de Kearns-Sayre

The possible physiopathologic mechanisms are debated; the preferred hypothesis was a dysfunction of endothelial cell pumps. We emphasize the extremely complex medical management of these fragile patients presenting mitochondrial encephalopathy. General corticotherapy is contraindicated and beta-blockers including in eye drops must be used extremely carefully because of cardiac abnormalities such as conduction disturbance and/or arrhythmia and dilated cardiomyopathies.