Amyloidosis is a disorder of protein metabolism in which amyloid can be accumulated in various tissues of the body. Primary localized amyloidosis can affect the bulbar or palpebral conjuctiva, Tenon`s capsule, tarsus, limbus, lacrimal gland, or orbit. Especially secondary localized amyloidosis of the cornea can occur after trauma. scar, and corneal opacity or can be associated with a chronic ocular inflammatory disorder. The authors diagnosed the secondary localized amyloidosis that developed in a 53-years-old female patient who had undergone penetrating keratoplasty. The diagnosis was confirmed by hematoxylin-eosin and Congo red staining, and transmission electron microscopic examination.
Amyloid ; Amyloidosis* ; Ankle ; Cicatrix ; Congo Red ; Cornea ; Corneal Opacity ; Corneal Transplantation* ; Diagnosis ; Female ; Humans ; Keratoplasty, Penetrating ; Lacrimal Apparatus ; Metabolism ; Orbit

Rlsacea is chronic disease of the eye and facial skin, which is common in Caucasian. Ocular rosacea is often difficult for ophthalmologists to diagnose, especially when skin manifestations are not evident yet. Early diagnosis and early treatment is important to prevent its serious complications including corneal opacity and corneal perforation. A case of ocular rosacea in a 52-year-old Korean lady is reported. The patient was presented with hyperemic conjunctiva and vascularization, infiltration and thinning of the peripheral cornea. She did not have any specific skin lesion. But 20 years ago, she had suffered from rosacea. After administation of oral tetracycline and topicale and topical corticosteroid, the signs and symptoms of the ocular rosacea was markedly improved.
Chronic Disease ; Conjunctiva ; Cornea ; Corneal Opacity ; Corneal Perforation ; Early Diagnosis ; Humans ; Middle Aged ; Rosacea* ; Skin ; Skin Manifestations ; Tetracycline*

Rlsacea is chronic disease of the eye and facial skin, which is common in Caucasian. Ocular rosacea is often difficult for ophthalmologists to diagnose, especially when skin manifestations are not evident yet. Early diagnosis and early treatment is important to prevent its serious complications including corneal opacity and corneal perforation. A case of ocular rosacea in a 52-year-old Korean lady is reported. The patient was presented with hyperemic conjunctiva and vascularization, infiltration and thinning of the peripheral cornea. She did not have any specific skin lesion. But 20 years ago, she had suffered from rosacea. After administation of oral tetracycline and topicale and topical corticosteroid, the signs and symptoms of the ocular rosacea was markedly improved.
Chronic Disease ; Conjunctiva ; Cornea ; Corneal Opacity ; Corneal Perforation ; Early Diagnosis ; Humans ; Middle Aged ; Rosacea* ; Skin ; Skin Manifestations ; Tetracycline*

We performed keratoplasty and did not remove suture material in 189 patients (189 eyes) after operation. Among them, 48 eyes which had clear grafts with spontaneous suture breakage had been analyzed retrospectively for the age, the sex distribution, the time and the site of broken suture, the preoperative diagnosis and the relationship between the suture breakage and the presence of vasculized recipient bed or suture method. The time when suture was broken after keratoplasty ranged from 3 months to 80 months(average 30 months). Leukoma cornea, 25 eyes(52.1%) and keratoconus, 13 eyes(27.1%) were the most common cause of keratoplasty and the others were corneal dystrophy, Fuchs dystrophy and re-keratoplasty. The most common site of broken suture was at superior quadrant(30 eyes, 62.5%), The vasculized recipient bed was found in 16 eyes, 13 eyes of leukoma cornea and 3 eyes of fe-keratoplasty in preoperative state. The statistical analysis showed significant relationship between the presence of vasculized recipient bed and the suture breakage(p<0.05). Suture was broken in 28 eyes(26.7%) of 105 eyes with single continuous suture, 6 eyes(21.9%) of 20 eyes with combined interupted suture and 14 eyes(21.9%) of 64 eyes with combined interupted and continuous suture. The statistical analysis showed no significant relationship between the suture method and the suture breakage(p>0.05).
Cornea ; Corneal Opacity ; Corneal Transplantation* ; Diagnosis ; Fuchs' Endothelial Dystrophy ; Humans ; Keratoconus ; Retrospective Studies ; Sex Distribution ; Sutures* ; Transplants

PURPOSE: The purpose of this study is to report the genetic diagnosis of nine cases of Fabry in one family, either as carriers or patients. METHODS: We conducted analysis of the alpha-galactosidase A gene and ophthalmologic examination of family members of a patient diagnosed with Fabry disease. RESULTS: Our patient, his brother, and two male cousins had Fabry disease; his mother, three aunts, and his female cousin were Fabry carriers. Genetic study revealed deletion mutation (1235-1236delCT) at the alpha-galactosidase A gene in all subjects. Ophthalmologic examination detected whirl-like corneal opacity in all subjects, which is a typical characteristic of Fabry disease.
alpha-Galactosidase ; Corneal Opacity ; Diagnosis ; Fabry Disease* ; Female ; Humans ; Male ; Mothers ; Sequence Deletion ; Siblings

The purpose of this study was to investigate the correlations between endothelial cell loss rate at one week, one month, three months, and six months after penetrating keratoplasty and clinical factors including recipient age, donor age, preoperative endothelial cell densities of the donors and preoperative diagnoses of the recipients. Among the 70 patients who underwent penetrating keratoplasty at Chonbuk University Hospital from December 1996 to January 1999, 30 eyes of 30 patients that showed reasonable endothelial resolution by non-contact specular microscopy during the follow up period of 6 months were chosen and evaluated.The cell density decreased continuously during the observation period. Endothelial cell loss rate averaged 11.5%at one week, 19.0% at one month, 25.0%at three months and 30.0%at six months after penetrating keratoplasty. Endothelial cell loss rate showed no significant correlation with donor age, recipient age, preoperative cell density at any examination period[r+/-0.4, p0.05]. Preoperative endothelial cell density showed significant correlation with postoperative endothelial cell density at each examination period[r0.7, p<0.05]. The rate of endothelial loss in the keratoconus group was significantly lower than those of bullous keratopathy or corneal leukoma groups at three months after penetrating keratoplasty[Wilcoxon test, p<0.05].
Cell Count ; Corneal Opacity ; Diagnosis ; Endothelial Cells* ; Follow-Up Studies ; Humans ; Jeollabuk-do ; Keratoconus ; Keratoplasty, Penetrating* ; Microscopy ; Tissue Donors

PURPOSE: To report the first case of steroid sulfatase (STS) gene deletion, confirmed by multiplex ligation-dependent probe amplification (MLPA) analysis in identical twins with pre-Descemet corneal dystrophy associated with X-linked ichthyosis. CASE SUMMARY: 19-year old identical twin brothers with itching senses and hereditary thick scaly skin of the extremity and trunk visited our dermatologic clinic. Upon visiting, an ophthalmologic consultation with anterior segment examination showed diffuse punctate corneal opacities in the pre-Descemet layer. On MLPA analysis of the identical twin brothers, a definitive diagnosis of X-linked ichthyosis was made by identifying STS gene deletion. CONCLUSIONS: Identification of the deletion and mutation of the involved gene using gene analysis can provide insight to diagnosis and clinical characteristics of X-linked ichthyosis.
Cornea ; Corneal Opacity ; Diagnosis ; Extremities ; Gene Deletion ; Humans ; Ichthyosis* ; Multiplex Polymerase Chain Reaction ; Pruritus ; Siblings ; Skin ; Steryl-Sulfatase ; Twins, Monozygotic

PURPOSE: To compare the survival of corneal grafts and the changes in endothelial cell density in penetrating keratoplasty using domestic or imported corneas. METHODS: Medical records of 236 eyes of 211 patients who underwent penetrating keratoplasty from November 2004 to August 2011 in Seoul National University Hospital and were followedup at least 1 year were retrospectively reviewed. After excluding the patients who received the combined surgeries with other surgeries except cataract surgery, the eyes were divided into 2 groups depending on the origin of donor tissue resulting in a domestic cornea group (108 eyes) and an imported cornea group (128 eyes). Recipient demographics, preoperative diagnosis, donor age, death-to-preservation time, death-to-operation time and pre-and postoperative visual acuities were compared between the 2 groups. Kaplan-Meier survival and changes in endothelial cell density were analyzed at 3, 6 and 12 months and then every year. RESULTS: The most common preoperative diagnoses were regraft and corneal opacity in the domestic and imported cornea groups, respectively, without statistical difference. Death-to-preservation time was 8.9 hours and 8.0 hours in the domestic and imported cornea groups, respectively, without statistical difference. However, death-to-operation time was longer in the imported cornea group (4.98 days) than in the domestic cornea group (2.18 days). There were no differences in pre- and postoperative visual acuities, decrease in annual changes in endothelial densities and survival up to 3 years between the 2 groups. In addition, the survival and decreased annual changes in endothelial densities were not different from each other in penetrating keratoplasty combined with cataract surgery or in penetrating keratoplasty for a corneal edema. CONCLUSIONS: Our study results suggest that clinical outcomes of the penetrating keratoplasty using imported corneas are comparable in efficacy when compared with the clinical outcomes using domestic corneas.
Cataract ; Cornea* ; Corneal Edema ; Corneal Opacity ; Demography ; Diagnosis ; Endothelial Cells ; Humans ; Keratoplasty, Penetrating* ; Medical Records ; Retrospective Studies ; Seoul ; Tissue Donors ; Transplants ; Visual Acuity

Opacification of the cornea due to the deposition of lipids may be primary without evidence of previous corneal vascularization, or secondary to either preexisting corneal disease or systemic disturbances of lipid metabolism. If the deterioration of vision continues, penetrating keratoplasty may be needed. We evaluated a 19 year-old female patient referred to our department due to progressive corneal opacity in the left eye. She did not have significant past or family histories of corneal diseases. Ocular examinations revealed the whitish corneal deposits with deep stromal vascularization in her left eye. For confirmation of the diagnosis, lamellar keratectomy with amniotic membrane transplantation was performed and deep stromal vessels were photocoagulated using an argon laser. Histologic findings were compatible with lipid degeneration. The corneal opacities reduced markedly and did not show any evidence of recurrence during the follow up period of six months. Therefore, we report this case with the review of the literature.
Amnion* ; Argon ; Cornea ; Corneal Diseases ; Corneal Opacity ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Keratoplasty, Penetrating ; Light Coagulation* ; Lipid Metabolism ; Recurrence ; Young Adult

PURPOSE: To report an experience of improving symptoms through treatment with electrocauterization and subconjunctival bevacizumab injection in two patients with keratitis accompanied by corneal neovascularization and opacity. CASE SUMMARY: (Case 1) A 20-year-old woman visited our institution complaining of binocular blurred vision and congestion for the previous 3 years. Her best corrected visual acuity (BCVA) was 0.07 in the right eye and 0.4 in the left eye (Han Chun-suk visual acuity chart, decimal). The diagnosis was bilateral rosacea keratitis. Antibiotics, steroid eye drops and oral antibiotics were administered, but no improvement was observed. Electrocauterization was performed at the origin of the neovascularization of the cornea and bevacizumab was injected at the peripheral subconjunctiva in the right eye. Thereafter the corneal neovascularization did not recur, corneal opacity was reduced, and BCVA improved to 0.5. (Case 2) A 19-year-old woman visited complaining of left eye congestion for the previous 3 years. Her BCVA was 1.0 in the right eye and 0.08 in the left eye.The diagnosis was phlyctenular keratitis of the left eye. Antibiotics, steroid eye drops and oral antibiotics were administered, but no improvement was observed. Therefore, electrocauterization and subconjunctival bevacizumab injection were performed in the cornea of the left eye, after which corneal neovascularization and opacity decreased. CONCLUSIONS: In these cases, we report improvement of symptoms after treatment with electrocauterization and subconjunctival bevacizumab injection after failed treatment of palpebral sanitation, antibiotic and steroid.
Anti-Bacterial Agents ; Bevacizumab* ; Cornea ; Corneal Neovascularization* ; Corneal Opacity ; Diagnosis ; Electrocoagulation ; Estrogens, Conjugated (USP) ; Female ; Humans ; Keratitis ; Ophthalmic Solutions ; Rosacea ; Sanitation ; Telescopes ; Visual Acuity ; Young Adult