Photorefractive Keratectomy With Mitomycin C in Meesmann's Epithelial Corneal Dystrophy.

PURPOSE: To describe a case of Meesmann's epithelial corneal dystrophy that underwent photorefractive keratectomy (PRK) with mitomycin C. METHODS: Case report. RESULTS: A 36-year-old woman was evaluated for refractive surgery. She had a history of recurrent epithelial erosions and moderate visual loss over the past 10 years. Biomicroscopy revealed bilateral micro-cystic epithelial lesions and a diagnosis of Meesmann's epithelial corneal dystrophy was proposed. Corneal optical coherence tomography showed epithelial thickening with apparent intraepithelial cysts in the superficial layers. The patient's daughter's examination showed the same biomicroscopy pattern. PRK was performed. Epithelial healing was uneventful and only tiny microcysts could be observed after 3 months. However, complete recurrence of the intraepithelial cysts were observed after 1 year with visual acuity dropping due to residual refractive error. CONCLUSIONS: This case suggests that residual refractive error and recurrence of the cystic lesions and punctate erosions should be anticipated after PRK in patients with Meesmann's epithelial corneal dystrophy. [J Refract Surg. 2017;33(1):53-55.].

Trasplante autólogo de limbo como nuevo tratamiento de la distrofia corneal epitelial de Lisch / Keratolimbal autograft transplantation as a possible new treatment of Lisch epithelial corneal dystrophy

CASO CLÍNICO: Presentamos el caso de una mujer de 64 años con agudeza visual de 20/40 en su ojo derecho, debida a una opacificación grisácea y plumosa con microquistes en el epitelio corneal, compatible con una distrofia epitelial de Lisch. Se trató secuencialmente con varios desbridamientos epiteliales, lente de contacto y mitomicina C, a pesar de los cuales, recidivaba. La extirpación de un sector limbar y el trasplante de limbo autólogo obtuvieron buenos resultados y sin recurrencia. CONCLUSIÓN: El trasplante autólogo de limbo tras extirpar la zona de limbo afectado puede ser considerado como una nueva opción terapéutica en el tratamiento de la distrofia epitelial de Lisch asimétrica, cuando otros tratamientos han fallado

CASE REPORT: The case concerns 64-year-old woman with visual acuity of 20/40 in the right eye. Slit-lamp examination revealed a grey, feathery corneal opacification with intraepithelial microcysts compatible with Lisch epithelial corneal dystrophy (LECD). It was treated with epithelial debridements, contact lenses and mitomycin C, but the opacification recurred within months. The removal of limbus sector and autologous limbal transplantation (KLAT) were used successfully without recurrence. Conclusions: After removal of damaged limbus, KLAT should be considered as a treatment option for asymmetric LECD when other treatments have failed

Development of allele-specific therapeutic siRNA in Meesmann epithelial corneal dystrophy.

BACKGROUND: Meesmann epithelial corneal dystrophy (MECD) is an inherited eye disorder caused by dominant-negative mutations in either keratins K3 or K12, leading to mechanical fragility of the anterior corneal epithelium, the outermost covering of the eye. Typically, patients suffer from lifelong irritation of the eye and/or photophobia but rarely lose visual acuity; however, some individuals are severely affected, with corneal scarring requiring transplant surgery. At present no treatment exists which addresses the underlying pathology of corneal dystrophy. The aim of this study was to design and assess the efficacy and potency of an allele-specific siRNA approach as a future treatment for MECD. METHODS AND FINDINGS: We studied a family with a consistently severe phenotype where all affected persons were shown to carry heterozygous missense mutation Leu132Pro in the KRT12 gene. Using a cell-culture assay of keratin filament formation, mutation Leu132Pro was shown to be significantly more disruptive than the most common mutation, Arg135Thr, which is associated with typical, mild MECD. A siRNA sequence walk identified a number of potent inhibitors for the mutant allele, which had no appreciable effect on wild-type K12. The most specific and potent inhibitors were shown to completely block mutant K12 protein expression with negligible effect on wild-type K12 or other closely related keratins. Cells transfected with wild-type K12-EGFP construct show a predominantly normal keratin filament formation with only 5% aggregate formation, while transfection with mutant K12-EGFP construct resulted in a significantly higher percentage of keratin aggregates (41.75%; p<0.001 with 95% confidence limits). The lead siRNA inhibitor significantly rescued the ability to form keratin filaments (74.75% of the cells contained normal keratin filaments; p<0.001 with 95% confidence limits). CONCLUSIONS: This study demonstrates that it is feasible to design highly potent siRNA against mutant alleles with single-nucleotide specificity for future treatment of MECD.

The use of high modulus silicone hydrogel (SiHy) lens in the management of epithelial defects.

Corneal dystrophies are relatively rare bilateral anomalies. Meesman dystrophy is typically asymptomatic with little effect on visual performance. This case highlights the use of a high modulus silicone hydrogel (SiHy) lens in the management of an atypical presentation of Meesman's dystrophy with associated visual impairment due to epithelial defects. The selection of a SiHy material provided increased oxygenation to re-establish corneal integrity. However, selecting the appropriate modulus was an additional factor to consider in this case since it had a direct effect on the visual outcome. The higher modulus SiHy lenses provided a better visual success for this patient than one with a lower modulus. Modulus consideration may prove to be an additional factor in the lens choice of SiHy in the management of epithelial defects.