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Antovan K. Seyedi Honarvar, Sr., Niklas Darin, Mar Tulinius, Marita Andersson Grönlund; Increased Central Corneal Thickness In Children And Adolescents With Kearns-Sayre Syndrome. Invest. Ophthalmol. Vis. Sci. 2011;52(14):1111.
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Corneal decompensation and corneal endothelial abnormality have been associated with Kearns-Sayre syndrome (KSS) in occasional case reports. The aim of this study was to evaluate central corneal thickness (CCT) in individuals with KSS and to compare the results with healthy controls.
A retrospective study was performed on 10 patients with KSS diagnosed and followed-up between 1983 and 2007 at the Queen Silvia Children’s Hospital in Gothenburg, Sweden. They all had a large scale deletion of the mtDNA. The CCT was measured as part of an ophthalmological examination by ultrasound pachymetry in 6 eyes (4 right eyes; 2 left eyes) from four patients (4 females) with a mean age of 12.8 years (range 3.3-18.3 yrs). The mean value of 10 consecutive measurements of the center of the cornea of each eye was recorded, if possible. A control group consisted of 48 eyes from 24 persons (14 females, 10 males) with a mean age of 6.8 years (range 0.4-16.4 yrs) and they were investigated under identical conditions.
CCT was significantly higher in the patients with KSS (688.3 +/- 91.3) than in the control group (580.9 +/- 34.2 microm); p=0.009. 5/6 eyes from five patients with KSS had CCT >=615 microm compared with 5/48 eyes from four individuals in the control group (p=0.0004). There was no statistically significant difference between the right (581.6 +/- 33.9) and left eyes (580.2 +/- 35.2) of the controls.
These results indicate that patients with KSS have an increased CCT compared with controls, which can be of diagnostic value when investigating children who are suspected to have a mitochondrial disorder. We hypothesize that this increased CCT may reflect an impaired function of the endothelial cell pumps.
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