Ocular Manifestations, Treatment Outcome and Clinical Course of Infective Keratitis of Keratitis-Ichthyosis-Deafness Syndrome

PURPOSE@#To report the ocular manifestations and treatment outcomes of infective keratitis in a patient with keratitis-ichthyosis-deafness (KID) syndrome and to report a newly discovered mutation in the GJB2 gene in Korea.CASE SUMMARY: A 32-month-old boy was referred to the Ophthalmology Clinic for bilateral corneal opacity and glare. The patient showed alopecia, hyperkeratotic skin in both limbs, and hearing loss in both ears. Ocular examination showed loss of eyebrows and eyelashes, hyperkeratotic lesions of the eyelids, mucopurulent discharge in the eyelids, and opacity and scarring with superficial vascularization and conjunctivalization in both corneas. Molecular analysis showed a pathogenic variant in the GJB2 gene and confirmed the diagnosis of KID syndrome. Superficial keratectomy and amniotic membrane transplantation were performed in both eyes. Corneal opacity recurred in the left eye; treatment with bevacizumab eyedrops was instituted and penetrating keratoplasty was performed in the left eye. Corneal stromal opacity relapsed in the left eye; 5% vancomycin and 5% ceftazidime eyedrops were started and methicillin-resistant Staphylococcus aureus was cultured from a corneal scraping specimen.@*CONCLUSIONS@#A careful observation of ocular manifestations in KID syndrome is needed to prevent infective keratitis and limbal cell deficiency; intensive antibiotic eyedrop treatment is recommended to prevent permanent visual impairment.

A Case of Visual Loss and Ophthalmoplegia Following Injection of Hyaluronic Acid into the Glabella

PURPOSE: To report a case of sudden unilateral visual loss and total external ophthalmoplegia combined with multifocal brain infarction following injection of hyaluronic acid (Restylane; Q-Med AB, Uppsala, Sweden) into the glabella area. CASE SUMMARY: A 25-year-old woman was referred for sudden unilateral visual loss and blepharoptosis. Visual acuity was no light perception in the right eye and 0.15 in the left eye. The best corrected visual acuity was no light perception in the right eye and 1.0 in the left eye. The right pupil was dilated and a relative afferent pupillary defect was detected. The patient showed blepharoptosis and total external ophthalmoplegia on the right side. Fundus examination revealed central retinal artery occlusion and pale disc in the right eye. The patient had developed skin necrosis and a surrounding reddish reticular pattern on her face around the glabella. She was injected with hyaluronic acid for 5 times into the glabella area. Fluorescein angiography of the right eye revealed markedly prolonged choroidal filling around the optic disc with no retinal arterial filling. Brain MRI showed multifocal punctuate acute infarction in both frontal lobes. After 2 months, ophthalmoplegia improved partially although her right eye vision did not, and 15 PD right exotropia was observed. CONCLUSIONS: Although most complications of dermal fillers are mild and transient, severe and persistent adverse effects can occur including tissue necrosis, retinal artery occlusion, and brain infarction. Therefore cosmetic procedures should be carefully performed when administering periocular dermal filler injection. A prompt consultation with an ophthalmologist is recommended when there is evidence of visual problems after injection.

Age-Related Changes in the Thickness of the Lamina Cribrosa Measured by Spectral Domain OCT

PURPOSE: To measure the lamina cribrosa thickness (LCT) in vivo of healthy people and to determine any association between the LCT and age or sex. METHODS: We evaluated 100 eyes of 100 healthy volunteers. Forty eyes were selected and divided into 2 specific age groups (38-49 and 78-89 years) and the differences were assessed with respect to age and sex. Each participant underwent a complete eye examination and the LCT was evaluated with spectral domain optical coherence tomography (OCT) using enhanced depth imaging (EDI) mode. RESULTS: Eighty-seven eyes were considered for the measurement and analysis of the LCT and 13 eyes were excluded because of an unclear image for identifying the LCT. The mean age was 55.0 +/- 19.5 years. The mean LCT in healthy volunteers was 231.3 +/- 41.6 microm (range, 152.5 - 327.5 microm) and a negative relationship was found between LCT and age (LCT = -1.614 x age + 320.191 microm, 95% CI for slope -1.91 to -1.32, r2 = 0.572, p < 0.001). Regarding differences related to sex, males had relatively thicker LCT than females, irrespective of age, although not statistically significant (p = 0.623). CONCLUSIONS: The present study results showed a decrease in human LCT with increasing age. Therefore, a decreasing LCT with an increasing age could be considered a strong risk factor for the development of glaucoma. In addition, measuring the LCT may help to understand the disease prognosis.

Central Corneal Thickness and Corneal Endothelial Cells in Pseudoexfoliation Syndrome

PURPOSE: To evaluate the central corneal thickness (CCT) and corneal endothelial cell density (ENDO) in pseudoexfoliation syndrome. METHODS: Medical records of patients who underwent a preoperative examination for cataract surgery and an examination for glaucoma were retrospectively reviewed. The present study included 44 patients (59 eyes) and age- and sex-matched controls. The total pseudoexfoliation patients divided into 2 groups, such as pseudoexfoliation syndrome (28 eyes) and pseudoexfoliative glaucoma (21 eyes) .The intraocular pressure (IOP), CCT, ENDO, corneal curvature (CC) and axial length (AL) were measured. RESULTS: The total pseudoexfoliation group showed significantly higher IOP (p = 0.001), thinner CCT (p = 0.003) and lower ENDO (p = 0.037). The pseudoexfoliation syndrome group showed significantly thinner CCT (p = 0.010) and lower EDNO (p = 0.041), but no significant difference in IOP (p = 0.941). There was no significant difference in CCT (p = 0.060) and ENDO (p = 0.171) between the pseudoexfoliative glaucoma group and controls, but the pseudoexfolliative glaucoma group showed significantly higher IOP (p < 0.001). CONCLUSIONS: The pseudoexfoliation group showed thinner CCT which should be taken into consideration when evaluating IOP with the applanation tonometer and diagnosing glaucoma, especially in pseudoexfoliation syndrome patients.