Purpose: We report a case of xeroderma pigmentosum in a pediatric patient with a progressive pterygium-like lesion.Case summary: A 2-year-old girl with photophobia and localized, scattered, small, round brownish macules on skin areas exposed to the sun visited hospital. During follow-up, at the age of 7, she was diagnosed with xeroderma pigmentosum in Japan, and pigmented freckles on the upper and lower eyelids of both eyes were observed. At the age of 11, a possible medial limbal pingueculum and lower lid telangiectasis of the right eye were observed via slit-lamp examination, and one year later, a pterygium-like lesion and gradual fibrovascular tissue growth were found in the same location of the right eye. At the last visit, the progressive pterygium-like lesion and, lower lid telangiectasis of the right eye, and lid pigmentations of both eyes (xeroderma pigmentosum) were observed. The size of pterygium-like lesion continues to increase, but the patient remains under observation because the lesion was too small to remove. Conclusions We report the first case of xeroderma pigmentosum in a pediatric patient with a progressive pterygium-like lesion that showed age-related degeneration. We suggest that the pterygium-like lesion may reflect the photosensitivity to ultraviolet radiation characteristic of xeroderma pigmentosum.

Purpose: We aimed to compare the accuracy of the intraocular lens (IOL) calculation formula using the standard keratometry (K) and total K (TK) during the femtosecond laser-assisted cataract surgery (FLACS) with a monofocal IOL with enhanced intermediate function using currently used formulas. Methods: A retrospective review of 125 eyes from 125 patients who had undergone FLACS with implantation of monofocal IOL with enhanced intermediate function was conducted. The predicted refractive power was calculated using an optical biometer (IOLmaster 700) according to the K and TK in the Barrett Universal II, SRK/T, Haigis, and Holladay 2 formulas. Absolute prediction error (APE) obtained from the actual postoperative refractive outcomes and the refractive error predicted in each formula was compared one month after surgery. Results: Mean APE ranged between 0.29 and 0.39 diopters (D) regardless of the calculation formula and the method of measuring corneal curvature. Significant differences were observed in the APE from the four formulas and the two keratometric measurements (p = 0.014). In a total of 125 eyes from 125 patients, the mean APE was lowest with the Barrett Universal II formula. Across all formulas, both the mean APE and the median APE tended to be lower for K than for TK, although there was no significant difference. Approximately 70% to 80% of the patients were included within 0.5 D of the refractive error across all formulas. The percentage of eyes within 0.5 D of APE outcomes was not statistically different between the K and TK data when using each formula. Conclusions Keratometric measurements considering the poster corneal curvature did not show any additional advantages when implanting the monofocal IOL with enhanced intermediate function during the FLACS.

Purpose: To investigate the oxidative damage and changes of retinas by blue light through zebrafish and to confirm the protective effect of polyphenol on retina cells using grape seed-derived polyphenols. Methods: To induce oxidative damage and changes of the retina by blue light, blue light LED (10,000 Lux, 480 nm) was added to the zebrafish grown in the dark room after pretreating polyphenols derived from grape seed at various concentrations (0, 0.1, 1, 10 μg/mL, respectively) for 4 days. Changes in retinal thickness and numbers of outer nuclear layer nuclei through hematoxylin & eosin staining were evaluated. Results: Photoreceptor layer thickness of blue light exposed group was significantly thinner than the group without blue light (108.1 ± 27.7 μm vs 41.1 ± 17.0 μm). As pretreated polyphenol concentration increased, photoreceptor layer thickness was increased (41.1 ± 17.0, 56.3 ± 18.6, 90.7 ± 23.7, 99.1 ± 23.1 μm, p < 0.05), and damage to outer nuclear layer nuclei was also decreased. Conclusions Exposure to blue light is an important factor for increasing oxidative stress in the retina. Grape seed-derived polyphenols have been shown to protect photoreceptor cells and retinal pigment epithelial cells from oxidative stress. This suggests that the antioxidant effect of polyphenol compounds may help suppress the progression of retinal diseases associated with oxidative stress such as age-related macular degeneration.

Purpose: To report two cases of early onset oculopharyngeal muscular dystrophy, which were suspected to be chronic progressive external ophthalmoplegia.Case summary: Case 1, a 15-year-old male and Case 2, a 13-year-old male brother, visited the clinic with persistent diplopia 6 years prior. The older brother’s best-corrected visual acuity was 0.6 in both eyes and showed an exodeviation of 25 prism diopters. Bilateral ptosis was observed with ocular muscle movement limitations in all directions, and bilateral macular edema was found on fundus examinations. The younger brother had a best-corrected visual acuity of 1.0 in both eyes and showed exodeviation of 45 prism diopters. Bilateral ptosis and ocular muscle movement limitations were also observed. Both patients were suspected to have chronic progressive external ophthalmoplegia and were referred to a neurologist for a neurological examination and muscle biopsy. The muscle biopsies showed that both patients were diagnosed with oculopharyngeal muscular dystrophy. Conclusions It is important, initially, to report a case of early onset oculopharyngeal muscular dystrophy that has eyelid and eye movement symptoms, but no other typical symptoms.

Purpose: We aimed to report on the clinical outcomes of scleral lens applications in Korean patients with various corneal disorders. Methods: This retrospective review was conducted for 62 eyes of 47 patients who had been fitted with scleral lenses for various corneal disorders. The patients were referred for inadequate spectacle-corrected visual acuity and rigid gas permeable (RGP) or soft contact lens intolerance. Uncorrected visual acuity, habitually corrected visual acuity, best lens-corrected visual acuity, topographic indices, keratometry indices, and lens parameters were evaluated. Results: Twenty-six eyes of 19 patients with keratoconus were enrolled. Other conditions included corneal scar (13 eyes of 12 patients), phlyctenules (three eyes), laceration (four eyes), chemical burn (one eye), keratitis (one eye), Peters’ anomaly (one eye), fibrous dysplasia (one eye), ocular graft-versus-host disease (two eyes of one patient), irregular astigmatism (18 eyes of 12 patients), and corneal transplant status (five eyes of four patients). The mean topographic values of the eyes include flat keratometric value (43.0 ± 6.1 diopters [D]), steep keratometric value (48.0 ± 7.4 D), and astigmatism (4.9 ± 3.6 D). Of the eyes fitted with scleral lenses, best lens-corrected visual acuity (0.10 ± 0.22 logarithm of the minimum angle of resolution [logMAR]) was significantly better than the habitually corrected visual acuity (0.59 ± 0.62 logMAR, p < 0.001). Conclusions Scleral contact lenses are a good alternative for patients with corneal abnormalities and those who are intolerable to RGP contact lenses, resulting in both successful visual outcomes and patient satisfaction, especially concerning keratoconus, corneal scar, and corneal transplant status.

Purpose: To examine the efficacy and biocompatibility of a new artificial cornea using a rabbit model. Methods: Artificial cornea were transplanted into 20 New Zealand white rabbits. The disc-shaped artificial cornea is of diameter 8.0 mm (the core, clear optical zone diameter is 5.0 mm and that of the peripheral skirt 1.5 mm); of thickness 0.5 mm; and is fabricated from PHEMA, PMMA, and PETTA. Transplantation proceeded in two stages; all rabbits were then observed weekly to 12 weeks. Anterior segment photographs were taken, and anterior segment optical coherence tomography and histological analysis performed, to confirm the biocompatibility of the skirt and the extents of cell proliferation in surrounding tissues. Results: Two rabbits were sacrificed for histological examination in weeks 4 and 8 (one each). Four eyes failed because of surgical errors (artificial corneal decentration or excessively thin flaps). Of the 12 eyes for which surgery was technically successful, six (50.0%) maintained the optical zone structure and transparency to 12 weeks. Histology revealed that cells proliferated in the skirt and bound to surrounding tissues. Six eyes (50.0%) evidenced protrusions of the artificial cornea. Conclusions Transplantation of a new artificial cornea into rabbits met with some success (as confirmed anatomically and optically). However, corneal improvement and new surgical techniques are required to increase the success rate. Also, long-term follow-up is needed.