Introduction
To present a novel intraoperative application of photoactivated chromophore for infectious keratitis–corneal cross-linking (PACK-CXL) in the management of post-penetrating keratoplasty (PKP) multiresistant fungal keratitis in a patient with irradiation-related local immunosuppression.
Case report
A 62-year-old female underwent uneventful PKP for the management of post-irradiation actinic keratopathy. Three months postoperatively, she presented with a diffuse corneal melting abscess that was infiltrating the donor-recipient junction. Despite intensive antibiotic and antifungal therapy, corneal melting progressed to graft perforation. A repeat PKP combined with intraoperative PACK-CXL was performed. PACK-CXL was applied initially on the infected graft, involving the corneoscleral rim and then following placement of the donor button. No intra- or postoperative graft-related complications were encountered. No signs of infection were noted, and the graft remained clear during the 9-month follow-up period.
Conclusion
Intraoperative PACK-CXL combined with PKP appears to be a safe and effective technique for the management of post-PKP resistant fungal keratitis.
Background The mitochondrial DNA (mtDNA) A3243G point mutation encompasses a heterogenous group of disorders including mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), maternally inherited diabetes and deafness (MIDD), and, rarely, chronic progressive external ophthalmoplegia (CPEO). Regardless of the clinical phenotype, a specific retinopathy has been associated with the presence of this mitochondrial DNA mutation. We present six female patients exhibiting retinopathy of the A3243G point mutation at various stages.
History and Signs Six female patients (37 – 70 years old) with the A3243G point mutation (four MELAS, one MIDD, and one CPEO) exhibited a maculopathy. Visual acuity ranged from 1/60 to 10/10. Visual field abnormalities varied from minimal decreased sensitivity to absolute central scotomas. They all exhibited, at various degrees, a characteristic pattern of perimacular and peripapillary retinal pigment epithelium (RPE) alterations, with mottled dys-autofluorescence and RPE atrophy and deposits on OCT.
Therapy and Outcome The level of visual impairment depended on the foveal involvement and the extension of RPE atrophy. The severity of the maculopathy was not related to age. In the only long-term follow-up (15 years), evolution was slowly progressive.
Conclusions A single mtDNA point mutation at locus 3243 can result in a variety of clinical presentations (MELAS, MIDD, or CPEO). Ocular involvement may manifest as a perimacular/peripapillary RPE atrophy/deposit, which can variably impact central visual function (from asymptomatic to legal blindness). The discovery of such a maculopathy should prompt the ophthalmologist to complete the personal and family history, namely, asking for the presence of diabetes mellitus and/or deafness.
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