Cloudy Cornea with Arcus Juvenilis in a Case of Dense Deposit Disease.

A 25-year-old male patient presented with complaints of blurred vision in both eyes since 2 years. The patient was a known case of nephrotic syndrome with dyslipidaemia for which he was on diuretics and lipid-lowering agents for 3 years. On examination, his visual acuity was 6/9 in both eyes with cloudy cornea and arcus juvenilis. Fundus examination was within normal limits. On systemic work-up, his lipid profile was deranged with increased serum total cholesterol, very low density lipoprotein, low density lipoprotein and triglyceride. The serum high density lipoprotein was decreased. Renal function test revealed elevated serum creatinine with significant proteinuria. Renal biopsy was suggestive of dense deposit disease on immunofluorescence and transmission electron microscopy. Ocular manifestation of dense deposit disease is characterised by retinal drusen, pigmentary atrophy, choroidal neovascular membrane and atypical serous retinopathy. To the best of our knowledge, anterior segment changes in dense deposit disease has not been reported. This is the first case reporting cloudy cornea with arcus juvenilis in a case of dense deposit disease.

[Correlation between the corneal arcus and dyslipoproteinemia]. / Korelacija kornealnog arkusa sa dislipoproteinemijama.

Arcus corneae is the degeneration of the cornea, in which are depositing in its stroma, which is clinically manifested like cloudiness which is coincidental with edge of cornea, by the corneoscleral limb, looking like white ring. During 2002 in ophthalmology cabinet in Dom zdravlja Fojnica, 32 patients, aged 54-56, with diagnosis arcus corneae were treated, which means that findings of lipoprotein fractions in serum has been made. Those findings showed us positive correlation between degeneration of cornea and raising of serums lipoproteins level, especially LDL, cholesterol, thriglicerids, and that there isn't reverse relation, negative correlation between serum's HDL and certain ophthalmology's manifestation.

[Ocular manifestation in LCAT deficiency--a clinicopathological correlation]. / Okuläre Manifestation bei LCAT-Mangel - eine klinisch-histopathologische Korrelation.

BACKGROUND: Bilateral stromal corneal opacifications are important to detect potentially associated systemic diseases. Differential diagnosis includes, besides inflammatory diseases mucopolysaccharidoses, HDL deficiencies (LCAT deficiency, Tangier disease and fish eye disease), Schnyder's crystalline corneal dystrophy, lipid keratopathy, cystinosis, gout and mucolipidoses. CASE REPORT: The cornea of a 60-year old male patient, 180 cm height, showed milky hazy diffuse stromal inclusions with a prominent lipoid arc (VA O. D. 20/30 and O. S. 20/40, contre-jour VA less than 20/150). Ten years earlier, lattice corneal dystrophy was diagnosed. The cornea was thickened (0.61 mm). Except for the cornea, the anterior and posterior segments of the eye were unremarkable. Serum HDL concentration was decreased pathologically (7 mg/dl), although the LCAT activity was combined with apo-AI deficiency. Due to membrane instability, the erythrocytes showed target cell configuration. Penetrating excimer-laser keratoplasty was performed O. S. The epithelial wound closed only on the 32nd postoperative day. Histologically, the corneal stroma showed multiple vacuoles and amyloid deposits. Genetic analysis revealed two mutations in chromosome 16. CONCLUSION: To our knowledge, this is the second description in the literature of a patient with LCAT deficiency and secondary amyloidosis of the cornea. Additionally, LCAT deficiency is associated with anaemia and proteinuria. After a penetrating keratoplasty, prolonged wound healing is possible. Because of the bilateral corneal clouding, the ophthalmologist may help to identify patients with LCAT deficiency. Thus, it is possible to start antiarteriosclerotic therapy.

[Corneal Ascher ring. A ring-shaped stromal corneal opacity]. / Kornealer Ascher-Ring. Eine ringförmige stromale Hornhauttrübung.

An unusual bilateral ring-shaped corneal opacity has been observed in two patients. Biomicroscopical characteristics are minute greyish dots which are distributed within the outer stromal layers forming an arcus lipoides-like circular band. Besides of a typical arcus senilis no other corneal anomalies can be found. Up to date only 4 patients with similar corneal opacities have been described. Etiology remains unclear and according to the author who has first described this symptom we propose to name this corneal anomaly "Ascher-Ringe".

Corneal arcus: some morphology and applied pathophysiology.

The frequency and density of corneal arcus in 101 patients (101 eyes), 54 males and 47 females, were greatest in the 12 o'clock sector of the peripheral cornea. Sectors below 12, nasally and temporally, were approximately symmetrically affected. Both properties decreased progressively and significantly at the 11 and 1 o'clock sectors, then 10/2, then 9/3; 8/4 and 7/5 differed insignificantly from each other but were significantly less affected than 9/3, and more affected than 6 o'clock. Breadth and density were significantly positively correlated. The greater vascularity and higher temperature at the upper limbus might explain this pattern. The morphology of arcus is concluded to indicate the site of a peripheral corneal filter which prevents ingress of large light-scattering particles with the centripetal flow of tissue fluid. Filtration of immune complexes at the same site may initiate tissue destruction, hence some peripheral corneal ulcerations.

Granular corneal dystrophy with late manifestation.

Ninety-two cases of granular corneal dystrophy, most of them belonging to 5 pedigrees are described. The age of manifestation in this Finnish type of granular dystrophy is first in the end of the second decade, and visual acuity is in mean normal through the whole life. An autopsy study showed no changes outside cornea elsewhere in the eyeball. In one family with granular dystrophy, another type of dystrophy, hereditary fleck dystrophy of the cornea, was accidentally found.

Arcus senilis: an important forensic physical finding.

Arcus senilis is a deposition of lipid at the corneal periphery. It is dependent on vascularity for formation and is not a degenerative change. The prevalence of arcus increases with age, and it appears more in blacks and males. It appears to be related to cardiovascular disease but has not been shown to be a reliable predictor of the disease. We studied the incidence of arcus width with age and found no definite correlation.

Corneal degenerations.

Corneal degenerations are among the most commonly observed clinical disorders in ophthalmology. They may be classified as (1) aging changes, (2) depositions, and (3) marginal degenerations. Aging changes are often incidental findings and do not usually interfere with vision. Depositions can occur secondary to previous ocular disease or trauma. Marginal degenerations are generally of unknown etiology, however, they may sometimes accompany a systemic disorder. The diseases in this latter group are usually the most difficult to treat and the most visually disabling.