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Severe Chronic Progressive External Ophthalmoplegia-Associated Ptosis Successfully Treated With Scleral Lenses: Response.

develop cerebellar ataxia, making handling of scleral lenses difficult. Particularly for the later reason we rather propose KSS patients to undergo ptosis surgery than wearing scleral lenses for ptosis correction. A further argument against the scleral lenses in KSS patients is that many of them develop retinitis pigmentosa resulting in visual impairment, further impeding the handling of the lenses. Particularly missing in the study is the genetic diagnosis of KSS or CPEO-plus. We should know the genetic cause of KSS or CPEO-plus in the index patient. Although single mtDNA deletions occur sporadically in 94% of the patients (4), we should know if the family history of the index patient was taken and if it was positive for a mitochondrial disorder. Because the severity of cardiac involvement is the lifelimiting factor in KSS (5), we should know if the patient had cardiac involvement and if she had undergone implantation of a pacemaker or an implantable cardioverter defibrillator already. Overall, although ptosis correction with scleral lenses could be a therapeutic option in some of the KSS respectively CPEO-plus patients, we recommend surgery in case of a patient with KSS. This is because of the progressive nature of the disease, ataxia, visual impairment, and the reduced life expectancy. Only in KSS patients who are not limited by these restrictions, the temporary use of scleral lenses could be tried. Author contribution: J. Finsterer: concept, writing literature search, and discussion; F.S.: literature search, critical remarks, and discussion.