Importance of monitoring the ocular surface of patients with Kearns‐Sayre syndrome: a case report

Abstract

AbstractPurposeKearns‐Sayre Syndrome (KSS) is a congenital mitochondrial myopathy initially described by the triad: Progressive external ophthalmoplegia, pigmentary retinopathy, heart block. Surgical procedures to alleviate ptosis can cause corneal injury from exposure. The lack of blinking can lead to a difficult contraction of the muscles responsible for squeezing the meibomian glands causing their dysfunction (MGD). Therefore, the follow‐up is of special interest in those cases where we suspect KSS due to the probable appearance of complications.MethodsWe present the case of a male of 9 years old who presented a sudden‐onset ptosis and bilateral internuclear ophthalmoplegia. Initially, he was diagnosed with Myasthenia. At the age of 12, resection of the elevator upper eyelid was performed in both eyes (BE). In the successive visits: negative Bell's sign, a progressive MGD and significant corneal thinning in the lower half of the BE were observed, and the right eye (RE) was affected with an infiltrated ulcer.ResultsTreatment with high hydration in BE and RE was treated with antibiotics eye drops and antibiotic ophthalmic ointment. After reviewing the history and given the high suspicion of KSS, genetic tests were requested to confirm it. Maintenance treatment was settled in BE with high hydration, 20% autologous serum and therapeutic contact lens. After a year of exhaustive monthly follow‐up, the patient remains stable and there is no progression of the corneal thinning.ConclusionsIt is important to take care of the ocular surface in these cases of KSS due to the corneal exposure after ptosis surgery. We must achieve stability in terms of symptoms and visual acuity without incurring other complications such as infections or perforation.