[Oedema with acute pseudokeratoconus corneal aspect in a case of Kearns-Sayre syndrome].
BACKGROUND
Mitochondrial encephalomyopathy is a set of rare diseases in which corneal involvement is exceptional.


METHODS
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.


CONCLUSIONS
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.
Limpas
Y
Y
Centre d'expertise médicale du personnel naviguant, Hôpital d'Instruction des Armées Sainte Anne, 83800 Toulon. yvon.limpas@noos.fr
Schauer
P
P
Vignal
R
R
Wary
P
P
D000208:Acute Disease; D000328:Adult; D015715:Corneal Edema / Q000150:complications*; D006801:Humans; D007625:Kearns-Sayre Syndrome / Q000150:complications*; D007640:Keratoconus / Q000150:complications*; D008297:Male