Bietti crystalline retinal dystrophy is a rare, inherited disorder whose hallmark is the presence of retinal crystal deposits associated with later chorioretinal degeneration. This condition may rarely be complicated by the development of cystoid macular oedema leading to rapid visual decline. Currently, treatment options for this complication of Bietti dystrophy are limited and the visual prognosis is poor. Here, we present a case of cystoid macular oedema associated with Bietti dystrophy that was successfully diagnosed using multimodal imaging techniques including optical coherence tomography and fluorescein angiography. These modalities confirmed the diagnosis of macular oedema and excluded other possible causes of oedema such as choroidal neovascularisation. In this patient, cystoid macular oedema was resolved with oral acetazolamide therapy, a treatment that has not been previously reported in this context. Acetazolamide treatment resulted in oedema resolution and improvement in visual function, and can be considered a therapeutic option for other patients with Bietti dystrophy who develop cystoid macular oedema.
Acetazolamide/*administration & dosage ; Administration, Oral ; Adult ; Corneal Dystrophies, Hereditary/*drug therapy/pathology ; Diuretics/*administration & dosage ; Humans ; Macular Edema/*drug therapy/pathology ; Male ; Retinal Diseases/*drug therapy/pathology ; Tomography, Optical Coherence ; Treatment Outcome

PURPOSE: In Asian countries, laser iridotomy for the treatment of angle-closure glaucoma is a common cause of bullous keratopathy, which may be associated with a shallow anterior chamber and dark iris pigmentation in Asians. Several cases of corneal decompensation after argon laser iridotomy have been reported. In the present study, we evaluated the harmful effects of argon laser iridotomy on the corneal endothelium. METHODS: Argon laser iridotomy was performed on the right eyes of pigmented rabbits. Changes in corneal thickness and endothelial cell density after laser iridotomy were evaluated. Terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) was performed for assessment of corneal endothelial cell apoptosis. Combined staining with alizarin red and trypan blue, as well as a live/dead cell assay, were performed for evaluation of damage to the corneal endothelium induced by laser iridotomy. RESULTS: Corneal thickness did not change immediately after laser iridotomy; however, a significant increase was observed 24 hours after iridotomy (p = 0.001). The endothelial cell density of laser-treated eyes four days after laser iridotomy was significantly decreased compared with control eyes (p < 0.001). TUNEL staining showed many TUNEL-positive cells in the corneal endothelium and corneal stroma. No endothelial trypan blue-stained cell nuclei were observed after laser iridotomy; however, several large endothelial cells with damaged membrane integrity were observed. The live/dead cell assay clearly showed a large number of dead cells stained red in several areas throughout the entire corneal button 24 hours after iridotomy. CONCLUSIONS: Argon laser iridotomy induces corneal endothelial cell apoptosis in pigmented rabbit eyes, resulting in decreased endothelial cell density.
Animals ; Apoptosis ; Corneal Diseases/pathology/*surgery ; Disease Models, Animal ; Endothelium, Corneal/*pathology ; In Situ Nick-End Labeling ; Iris/*surgery ; Laser Therapy/*methods ; Lasers, Gas/*therapeutic use ; Ophthalmologic Surgical Procedures/*methods ; Rabbits

PURPOSE: In Asian countries, laser iridotomy for the treatment of angle-closure glaucoma is a common cause of bullous keratopathy, which may be associated with a shallow anterior chamber and dark iris pigmentation in Asians. Several cases of corneal decompensation after argon laser iridotomy have been reported. In the present study, we evaluated the harmful effects of argon laser iridotomy on the corneal endothelium. METHODS: Argon laser iridotomy was performed on the right eyes of pigmented rabbits. Changes in corneal thickness and endothelial cell density after laser iridotomy were evaluated. Terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) was performed for assessment of corneal endothelial cell apoptosis. Combined staining with alizarin red and trypan blue, as well as a live/dead cell assay, were performed for evaluation of damage to the corneal endothelium induced by laser iridotomy. RESULTS: Corneal thickness did not change immediately after laser iridotomy; however, a significant increase was observed 24 hours after iridotomy (p = 0.001). The endothelial cell density of laser-treated eyes four days after laser iridotomy was significantly decreased compared with control eyes (p < 0.001). TUNEL staining showed many TUNEL-positive cells in the corneal endothelium and corneal stroma. No endothelial trypan blue-stained cell nuclei were observed after laser iridotomy; however, several large endothelial cells with damaged membrane integrity were observed. The live/dead cell assay clearly showed a large number of dead cells stained red in several areas throughout the entire corneal button 24 hours after iridotomy. CONCLUSIONS: Argon laser iridotomy induces corneal endothelial cell apoptosis in pigmented rabbit eyes, resulting in decreased endothelial cell density.
Animals ; Apoptosis ; Corneal Diseases/pathology/*surgery ; Disease Models, Animal ; Endothelium, Corneal/*pathology ; In Situ Nick-End Labeling ; Iris/*surgery ; Laser Therapy/*methods ; Lasers, Gas/*therapeutic use ; Ophthalmologic Surgical Procedures/*methods ; Rabbits

Amiodarone ; adverse effects ; Anti-Arrhythmia Agents ; adverse effects ; Arrhythmias, Cardiac ; drug therapy ; Corneal Diseases ; chemically induced ; Humans ; Male ; Middle Aged

PURPOSE: This retrospective observational case series of fifty-one consecutive patients referred to the eye clinic with acute-stage Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) from 1995 to 2011 examines the effect of early treatment with a systemic corticosteroid or intravenous immunoglobulin (IVIG) on the ocular outcomes in patients with SJS or TEN. METHODS: All patients were classified by age (< or =18 years vs. >18 years) and analyzed by treatment modality and early intervention with systemic corticosteroids (< or =5 days), IVIG (< or =6 days), or amniotic membrane graft transplantation (AMT) (< or =15 days). The main outcomes were best-corrected visual acuity (BCVA) in logarithm of the minimum angle of resolution (logMAR) and ocular involvement scores (OIS, 0-12), which were calculated based on the presence of superficial punctate keratitis, epithelial defect, conjunctivalization, neovascularization, corneal opacity, keratinization, hyperemia, symblepharon, trichiasis, mucocutaneous junction involvement, meibomian gland involvement, and punctal damage. RESULTS: The mean logMAR and OIS scores at the initial visit were not significantly different in the pediatric group (logMAR = 0.44, OIS = 2.76, n = 17) or the adult group (logMAR = 0.60, OIS = 2.21, n = 34). At the final follow-up, the logMAR and OIS had improved significantly in the adult group (p = 0.0002, p = 0.023, respectively), but not in the pediatric group. Early intervention with IVIG or corticosteroids significantly improved the mean BCVA and OIS in the adult group (p = 0.043 and p = 0.024, respectively for IVIG; p = 0.002 and p = 0.034, respectively for corticosteroid). AMT was found to be associated with a significantly improved BCVA or OIS in the late treatment group or the group with a better initial OIS (p = 0.043 and p = 0.043, respectively for BCVA; p = 0.042 and p = 0.041, respectively for OIS). CONCLUSIONS: Our findings suggest that patients with SJS or TEN who are aged 18 years or less have poorer ocular outcomes than older patients and that early treatment with steroid or immunoglobulin therapy improves ocular outcomes.
Acute Disease ; Adolescent ; Age Factors ; Amnion/*transplantation ; Biopsy ; Child ; Child, Preschool ; Corneal Diseases/etiology/pathology/*therapy ; Female ; Follow-Up Studies ; Glucocorticoids/*administration & dosage ; Humans ; Immunoglobulins, Intravenous/*administration & dosage ; Infant ; Male ; Retrospective Studies ; Stevens-Johnson Syndrome/complications/pathology/*therapy ; Time Factors ; Treatment Outcome ; *Visual Acuity

This study was performed to evaluate the effects of conditioned media (CM) from human amniotic epithelial cells (HAECs) on the corneal wound healing process. Eighteen rabbits (36 eyes) were used and randomly assigned to three groups according treatment: CM from HAECs (group 1), vehicle alone (group 2), and saline (group 3). Corneal alkali injuries were induced with 1 N sodium hydroxide. Each reagent used for treatment evaluation was injected into the dorsal bulbar subconjunctiva and the area of the corneal epithelial defect was measured every other day. Two animals from each group were euthanized at a time on days 3, 7, and 15, and the cornea was removed for histological examination. The sum of the epithelial defect areas measured on day 0 to day 6 as well as day 0 to day 14 in group 1 was significantly smaller than those of other groups. Histological examination revealed that the group 1 corneas had less inflammatory cell infiltration and showed more intact epithelial features compared to the other groups. These results suggest that CM from HAECs promote corneal wound healing in rabbits.
Alkalies/*toxicity ; Amnion/*cytology ; Animals ; Cornea/*injuries ; Corneal Diseases/chemically induced/therapy/*veterinary ; Culture Media, Conditioned/*pharmacology ; Epithelial Cells/*physiology ; Humans ; Male ; *Rabbits

We report a case of vortex keratopathy in a patient treated with vandetanib for non-small cell lung cancer (NSCLC). A 44-year-old female who underwent two cycles of chemotherapy for NSCLC complained of visual blurring in both eyes after the initiation of vandetanib, an anti-epidermal growth factor receptor (EGFR) and anti-vascular endothelial growth factor receptor 2 protein tyrosine kinase inhibitor. On ophthalmic examination, visual acuities were 20 / 20 OU and, with the exception of diffuse vortex keratopathy in both eyes, other findings were unremarkable. Vandetanib is believed to have caused vortex keratopathy in this patient. Anti-EGFR properties affecting normal corneal epithelial cell migration and wound healing or drug associated metabolite deposition, which is the case in numerous drug-associated vortex keratopathies, may be possible underlying mechanisms in the formation of this corneal complication.
Adult ; Carcinoma, Non-Small-Cell Lung/*drug therapy/pathology ; Cornea/drug effects/*pathology ; Corneal Diseases/*chemically induced/diagnosis ; Diagnosis, Differential ; Dose-Response Relationship, Drug ; Female ; Follow-Up Studies ; Humans ; Lung Neoplasms/*drug therapy/pathology ; Microscopy, Acoustic ; Piperidines/administration & dosage/*adverse effects ; Quinazolines/administration & dosage/*adverse effects ; Visual Acuity

Two schizophrenic patients who had been taking medication for a long period presented with visual disturbance of 6-month duration. Slit-lamp examination revealed fine, discrete, and brownish deposits on the posterior cornea. In addition, bilateral star-shaped anterior subcapsular lens opacities, which were dense, dust-like granular deposits, were noted. Although we strongly suspected that the patient might have taken one of the drugs of the phenothiazine family, we were unable to obtain a history of medications other than haloperidol and risperidone, which were taken for 3 yr. We performed a drug profiling test using urine samples and detected methotrimeprazine. The patient underwent surgery for anterior subcapsular lens opacities. Visual acuity improved in both eyes, but the corneal deposits remained. We report an unusual case of methotrimeprazine-induced corneal deposits and cataract in a patient with psychosis, identified by using the urine drug profiling test.
Adult ; Antipsychotic Agents/*adverse effects/therapeutic use/urine ; Cataract/*chemically induced ; Corneal Diseases/*chemically induced ; Female ; Humans ; Male ; Mental Retardation/diagnosis/drug therapy ; Methotrimeprazine/*adverse effects/therapeutic use/urine ; Middle Aged ; Schizophrenia/diagnosis/drug therapy ; Visual Acuity

PURPOSE: The objective of this study is to evaluate the therapeutic efficacy of ethylenediaminetetraacetic acid (EDTA) chelation and excimer laser phototherapeutic keratectomy (PTK) combined with amniotic membrane transplantation (AMT) for the treatment of band keratopathy (BK). METHODS: Eleven eyes in ten patients with BK received combined PTK (ablation zone of central 7.0-7.5 mm, depth of 50 microm), EDTA chelation (0.05 M, 3 minutes), and amniotic membrane transplantation using fibrin glue. Preand postoperative best corrected visual acuities, symptom changes, reepithelialization time, cosmesis, recurrence, and complications were analyzed. RESULTS: Visual acuity improved in three eyes (27.3%) and did not change in eight eyes (72.7%). Symptoms improved in all patients, and the mean reepithelialization time was 10.6+/-5.3 days. The cosmetic results were good in eight eyes (72.7%) and were fair in three eyes (27.3%). During the mean follow-up period of 11.4+/-6.1 months (range, 6 to 23 months), no postoperative complications or recurrences were observed. CONCLUSIONS: The combination of EDTA chelation, PTK, and AMT is safe and effective for the treatment of band keratopathy.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Amnion/*transplantation ; Chelating Agents/*therapeutic use ; Combined Modality Therapy ; Corneal Diseases/*therapy ; Edetic Acid/*therapeutic use ; Female ; Humans ; Male ; Middle Aged ; Photorefractive Keratectomy/*methods ; Retrospective Studies ; Treatment Outcome ; Visual Acuity

The purpose of this study is to characterize and compare the ultrastructural changes occurring during the in vivo cultivation of corneal epithelium on amniotic membrane (AM) at several different time points. Corneal burn patients (n=7) with a corneal epithelial defect and severe limbal damage were selected. Initially, AM transplantation with limbal autograft was performed at the acute stage of corneal burn to reconstruct the damaged ocular surface. One to six (mean interval; 3.3+/-1.2) months later, the central part of AM containing an in vivo expanded corneal epithelium was excised and retransplanted in adjacent lesions. The excised epithelium with AM was examined by electron microscopy and immunohistochemical study. By electron microscopy, one and two months after expansion, cultivated epithelium on AM showed an undifferentiated epithelium and an incomplete basement membrane (BM). But, after three months, the cultivated epithelium began to differentiate into a multilayered epithelium with a continuous BM with increased hemidesmosomes. These findings were further confirmed by immunohistochemical study, that cytokeratin K3 was expressed in the cultivated corneal epithelium and newly formed BM was partially positive of collagen IV at three months. At least 3 months may be needed for the proliferation and differentiation of in vivo cultivated corneal epithelium on AM.
Stem Cells/cytology ; Stem Cell Transplantation/*methods ; Middle Aged ; Microscopy, Electron ; Male ; Keratin-3/biosynthesis ; Immunohistochemistry ; Humans ; Epithelium, Corneal/cytology/*metabolism/*pathology/*transplantation ; Corneal Diseases/*therapy ; Burns/*surgery/therapy ; Biological Dressings ; Amnion/*ultrastructure ; Adult