Leber hereditary optic neuropathy (LHON) is a mitochondrial disease leading to optic atrophy due to degeneration of the retinal ganglion cell. A curative treatment is not available at the moment, but a new antioxidant drug, Idebenone, is expected to reduce the progression of the disorder. Two male patients, genetically confirmed with LHON, were clinically, morphologically, and electrophysiologically evaluated, before and three, six, nine and 12 months after starting the treatment. The patient with 3460G>A mutation in mitochondrially-encoded nicotinamide adenine dinucleotide, reduced form (NADH):ubiquinone oxidoreductase core subunit (mtND)1 gene showed an improvement in visual acuity, visual field, and visual evoked potentials with no effect on morphological examinations, while the patient with 11778G>A mutation in mtND4 gene showed no functional, nor morphological recovery after one year of treatment. This study demonstrates that Idebenone, depending on the genetic profile of the disease, may be effective in functional improvement in patients with LHON.
Purpose. To report a case of malignant glaucoma in a pseudophakic female patient, with no history of glaucoma, resolved through pars plana anterior vitrectomy.Case presentation. An 80-year-old female patient presented in our Emergency Department after a five-day history of pain in her left eye (LE). In the last day, the patient noticed marked visual loss and ocular pain. Visual acuity was light perception and Goldman tonometry was 80 mmHg in her LE. The biomicroscopy revealed absent peripheral and central anterior chamber (AC) and posterior chamber (PC) pseudophakia. Posterior segment ecography showed no vitreous or choroidal abnormalities. A peripheral laser YAG iridotomy was made and the patient was treated with intravenous 20% mannitol, topical timolol, topical brimonidine, and topical cycloplegics. 12 hours later, despite a patent iridotomy in the LE eye, intraocular pressure (IOP) was 55 mmHg, absent AC with severe corneal edema. The diagnosis of pseudophakic malignant glaucoma was made and laser YAG capsulotomy was performed with no resolution of symptoms and signs. 24 hours later, we performed pars plana anterior vitrectomy. Postoperatively, the AC depth increased and the IOP decreased to 20mmHg. After a week, the patient was discharged with hand movement perception visual acuity in her LE, 20 mmHg IOP, reduced corneal edema, normal depth AC. After a month, the corneal edema resolved, the visual acuity was 2/50, IOP was 20mmHg, and the AC had a normal depth.Conclusion. Malignant glaucoma is a sight threatening condition, reported in pseudophakic eyes. Although, literature describes cases solved by cycloplegics and laser YAG capsulotomy, our patient needed pars plana anterior vitrectomy for the resolution of symptoms and signs.
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