[Oedema with acute pseudokeratoconus corneal aspect in a case of Kearns-Sayre syndrome].

INTRODUCTION

Mitochondrial encephalomyopathy is a set of rare diseases in which corneal involvement is exceptional.

CASE REPORT: A 34-year-old patient, a known carrier of Kearns-Sayre syndrome, was hospitalized for acute corneal decompensation, with a keratoconus-like hydrops stemming from an endothelial-Descemet membrane disruption. Under local hypotonic treatment, the favorable progression was fast with a decline in the edema and coaptation of the endothelial-Descemet membrane disruption.

CONCLUSION

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.