Disappearance of honeycomb opacity of Thiel-Behnke corneal dystrophy after Thygeson superficial punctate keratitis

PURPOSE: To present an unusual case in which the honeycomb opacities of Thiel-Behnke corneal dystrophy disappeared during a course of chronic Thygeson superficial punctate keratitis. CASE: A 29-year-old woman with a 5-year history of decreased visual acuity in both eyes had bilateral honeycomb-shaped corneal opacities, located mainly in the superficial central cornea. She was diagnosed as having Thiel-Behnke corneal dystrophy, which we confirmed genetically. During follow-up, her left eye was affected by chronic Thygeson superficial punctate keratitis, and the honeycomb opacity in this eye was noted to have disappeared. CONCLUSIONS: We observed the dissolution of honeycomb-like corneal opacities in a patient with confirmed Thiel-Behnke corneal dystrophy after the patient subsequently contracted chronic Thygeson superficial punctate keratitis. We surmise that epithelial inflammation associated with Thygeson epithelial keratitis may play a role in dissolving the honeycomb opacities.     

Effect of trigeminal denervation on rabbit corneal epithelium]

To investigate the effects of trigeminal denervation on the corneal epithelium, left postganglionic trigeminal neurotomy via intracranial approach was performed in 22 rabbits. Among 16 rabbits survived successfully, 13 denervated eyes (81%) showed corneal epithelial abnormalities that included 4 epithelial defects and 9 epithelial opacities. The remaining 3 eyes were normal. Histological examination showed many atrophic epithelial cells and the thinning of the corneal epithelial layer in the denervated eyes with corneal opacities. There was no abnormality in corneal stroma or endothelium. The ratio of epithelial to total corneal thickness calculated by a computer-assisted image analyzer was 6.5 +/- 2.1 (%) in control eyes and 3.8 +/- 1.9 (%) in the denervated eyes, the two values being significantly statistically different (p less than 0.001).     

Granular epithelial keratopathy as an unusual manifestation of Pseudomonas keratitis associated with extended-wear soft contact lenses

We describe four patients who, using extended-wear soft contact lenses for myopia, abruptly developed ocular irritation and injection associated with elevated granular opacities initially confined to the central corneal epithelium. Cultures of the granular epithelial lesions were positive for Pseudomonas aeruginosa in all patients. Cultures of the contact lenses and lens case solutions grew Pseudomonas species and other gram-negative organisms. All patients responded to discontinuation of lens wear and frequent topical antibiotics. All recovered baseline visual acuity, and three have successfully resumed contact lens wear. These cases document that Pseudomonas keratitis may be manifested as a granular epithelial keratopathy.     

Infectious crystalline keratopathy

In three patients (three men, 44, 67, and 83 years old) an unusual keratopathy characterized by white, branching, crystalline stromal opacities produced lesions that were insidious, that increased in size slowly, and that were clinically and histopathologically associated with little corneal inflammation. Intraocular inflammation eventually became evident in two of the patients. The keratopathy developed while the patients were using topical corticosteroids. In two cases, histopathologic studies disclosed accumulations of gram-positive cocci in the corneal stroma; a viridans streptococcus agent was isolated on culture. In one case, the viridans streptococcus was determined to be a dextran producer, which may have contributed to the pathogenesis of the keratopathy. Despite aggressive treatment with a variety of drugs, epithelial healing, and resolution of the corneal infiltrate, residual scarring persisted in two cases and neovascularization and graft edema in one case each.     

Clinical aspects of corneal trachoma.

Classification of trachoma by site rather than density of opacities is better related to visual prognosis and helps in selection for graft surgery. The cases are divided into 3 groups: peripheral corneal opacities, central corneal opacities, and diffuse corneal opacities (ground-glass cornea). A central lesion has the poorest prognosis, especially in children.     

Unusual Superficial Confluent Form of Granular Corneal Dystrophy

A 53-year-old Japanese woman had corneal opacification due to granular dystrophy (GCD) since childhood. Clinically, unusual bilateral confluent dense granular opacities with a peripheral rim of clear cornea were observed. Her mother and daughter have a milder form of GCD, with circumscribed opacities separated by clear stroma. Light and electron microscopy of the patient's corneal button showed that the opacities involved the corneal epithelium as well as the stroma.     

The cornea and disorders of lipid metabolism

Disorders of lipid metabolism, either hyperlipidemia or hypolipidemia, are associated with the formation of corneal opacities. Corneal arcus, the most commonly encountered peripheral corneal opacity, is frequently associated with abnormal serum lipid levels, but may occur without any predisposing factors. Reports also have linked corneal arcus with alcoholism, diabetes mellitus and atherosclerotic heart disease. Unilateral arcus is a rare entity that is associated with carotid artery disease or ocular hypotony. Diffuse corneal opacities associated with hypolipidemic disorders such as LCAT deficiency, fish eye disease and Tangier disease, may be the initial manifestation of these disorders and puts the ophthalmologist in a position to make an early diagnosis. Corneal arcus, along with a central corneal opacity, is seen in Schnyder's crystalline stromal distrophy. The association of the disorder with a dyslipidemia remains controversial. A review of lipid metabolism, corneal arcus and several disorders of lipid metabolism that affect the cornea are presented.     

Meesmann corneal dystrophy associated with epithelial basement membrane and posterior polymorphous corneal dystrophies

PURPOSE: To report a rare case of bilateral and symmetric Meesmann corneal dystrophy concurrent with bilateral epithelial basement membrane dystrophy and bilateral but asymmetric posterior polymorphous corneal dystrophy in a patient of Armenian origin. METHODS: Complete ophthalmologic examination was performed on a 6-year-old boy from Armenia who was diagnosed with bilateral symmetric Meesmann corneal dystrophy combined with bilateral epithelial basement membrane dystrophy and bilateral but asymmetric posterior polymorphous corneal dystrophy. This case was observed and treated for 24 years. RESULTS: On slit-lamp biomicroscopy, the patient showed bilateral multiple intraepithelial cystic lesions, bilateral irregularly shaped grayish-white opacities in the superficial corneal epithelium, and bilateral but asymmetric transparent vesicles surrounded by gray halos at the level of the Descemet membrane and the endothelium. CONCLUSIONS: This case is reported because of the unusual occurrence of Meesmann corneal dystrophy with other corneal dystrophies.     

Anterior amorphous corneal opacity and corneal thinning

PurposeTo present the clinical features of four cases with bilateral anterior amorphous corneal opacity.MethodsA retrospective study in four patients with bilateral anterior amorphous corneal opacity was conducted. Examinations included visual acuity, keratometry, slit-lamp biomicroscopy, confocal microscopy, anterior segment optical coherence topography, and histology.ResultsThree female and one male patients (mean age, 52.3 ± 8.9 years) showed bilaterally oval, amorphous sheetlike corneal opacities with central depression and thinning. Superior limbal opacities were observed in two of these patients. The best-corrected visual acuity ranged from 20/50 to 20/400, and the mean of the keratometry was 39.81 ± 3.97 D (diopters). They had mild dry eyes. The anterior segment optical coherence topography demonstrated hyporeflective abnormalities in the anterior depressed stroma in these four patients. Confocal microscopy revealed large round cells at the epithelial layer in one patient, and amorphous opacities with some strand-shaped opacities in the anterior stroma in all four patients. The mean of the corneal endothelial cells density in the eight eyes was 1521 ± 402 cells/mm². Central corneal stromalysis occurred in three patients, and descemetocele developed in two eyes. One patient received penetrating keratoplasty and two underwent lamellar keratoplasty. The histology of the corneal specimen revealed edematous basal epithelial cells, focal collagen disorganization in the thin stroma, and wartlike excrescences in a thickened Descemet's membrane.ConclusionAnterior amorphous corneal opacity is a rare keratopathy and may be one kind of rare corneal degeneration or dystrophy. Corneal stromalysis may occur in hyporefrective amorphous opacities and progress to descemetocele.     

The different opacity patterns of Lisch corneal dystrophy

BACKGROUND: Lisch corneal dystrophy is an epithelial corneal dystrophy where diffuse gray corneal opacifications are seen in direct illumination that appear in different patterns. Examination in retroillumination shows that these gray changes do consist of multiple densely crowded clear microcysts according to the opacity units. We wanted to investigate if one single case of Lisch corneal dystrophy can be differentiated easily from the other epithelial corneal dystrophies by exact analysis of the opacity units. PATIENTS AND METHODS: We examined three non-related patients with Lisch corneal dystrophy at the slit-lamp. The epithelial corneal opacities were documented photographically with direct slit-lamp illumination and retroillumination. RESULTS: All three patients showed the characteristic intraepithelial densely crowded clear microcysts in retroillumination. Diffuse gray radial opacities were noted in both corneas of a 15-year-old female patient. A club-shaped opacity was noted in one cornea of a 35-year-old female patient and a feathery gray opacity pattern was found in one cornea of a 49-year-old male patient. All three patients showed the characteristic densely crowded clear microcysts of Lisch corneal dystrophy in retroillumination. CONCLUSION: Taking the previous literature into account, five different opacity patterns can be summarised: radial, band, flame/feathery, whorled and club-shaped gray epithelial corneal opacities. A single case of Lisch corneal dystrophy can be differentiated from other epithelial corneal dystrophies by means of an exact analysis of the opacity pattern and units by slit-lamp examination.     

Histopathological findings of epithelial ingrowth after laser in situ keratomileusis

PURPOSE: To investigate the histopathological features of epithelial ingrowth after laser in situ keratomileusis (LASIK). METHODS: Five epithelial ingrowth specimens of 4 patients (2 to 17 months after initial LASIK) were examined. The lesions were surgically removed and examined using light and transmission electron microscopy. RESULTS: Early epithelial ingrowth was clinically observed as faint opacities with demarcation lines and epithelial pearls. Late epithelial ingrowth was observed as homogeneous whitish masses. Histopathologically, early epithelial ingrowth consisted of multilayered squamous epithelium resembling normal corneal epithelium, whereas late ingrowth was made up of clumps containing amorphous materials with scarce cellular elements. CONCLUSIONS: Our findings suggest that clinical appearance and histopathology of epithelial ingrowth from LASIK flap margins differ in early and late stages, which might reflect proliferative activity of the lesions.     

A new, band-shaped and whorled microcystic dystrophy of the corneal epithelium.

Five family members and three unrelated patients (four women, four men, 23 to 71 years old) had a dystrophy of the corneal epithelium. Direct slit-lamp examination showed bilateral or unilateral, gray, band-shaped, and feathery opacities that sometimes appeared in whorled patterns. Retroillumination showed intraepithelial, densely crowded, clear microcysts. Light and electron microscopy disclosed diffuse vacuolization of the cytoplasm of epithelial cells in the affected area. Visual acuity was so reduced in three patients that abrasion of the corneal epithelium was performed. The corneal abnormalities recurred within months, with the same reduction in visual acuity as before. The corneal opacities were progressive in two patients but diminished noticeably in another after he began using a hard contact lens. We found no other ophthalmic irregularities or associated systemic abnormalities and no indication of drug-induced keratopathy.     

Corneal opacities in the Hallermann-Streiff syndrome

We present six patients with typical Hallermann-Streiff syndrome. All have microphthalmia and were operated for congenital cataract. Three of the patients developed a severe glaucoma and one patient presented repeated uveal effusions. Five of our patients have the same pattern of corneal stromal opacities. The opacities are ill defined and bilateral; the stroma between the opacities is clear. The opacities are observed in two children around the age of 5. Follow up of 10 years did not reveal a manifest increase of the lesions. The authors believe that corneal stromal opacities are a feature of the Hallermann-Streiff syndrome and they would urge ophthalmologists to look for this.     

Exacerbation of granular corneal dystrophy type II (Avellino corneal dystrophy) after LASEK

PURPOSE: To report cases of granular corneal dystrophy type II (Avellino corneal dystrophy) that were exacerbated by uncomplicated laser epithelial keratomileusis (LASEK) for myopia. METHODS: Eight patients (15 eyes) with granular corneal dystrophy type II who underwent LASEK were examined by DNA sequencing, slit-lamp microscopy, and confocal microscopy. RESULTS: The number and density of the opacities increased after LASEK. Best spectacle-corrected visual acuity decreased after LASEK as the density of the opacities increased. In three patients, mitomycin C was used intraoperatively, but corneal deposits also worsened in these patients. CONCLUSIONS: LASEK is contraindicated in patients with granular corneal dystrophy type II because vision may be reduced from an increase in the density of corneal opacities postoperatively.     

Inherited corneal opacifications with an unusual distribution

PURPOSE: To describe corneal opacities of a new type and distribution in a small family. METHODS: Family members were interviewed and examined to establish a pedigree and to detect any corneal abnormalities. RESULTS: Two family members presented with corneal opacities. Both had, in the very peripheral cornea, flat, greyish, rounded opacities, 20-200 microm in diameter, on the Descemet's membrane. In addition, the mother had the same type of opacities over the central cornea just inside the Bowman's layer. The remaining parts of the corneas were clear. Vision was unaffected and the opacities caused no discomfort. There was no other corneal pathology. The subjects' general health was good. CONCLUSIONS: To our knowledge, these types and distribution of corneal opacities have not been described previously. Although the mode of inheritance at this point is uncertain, we believe the changes are of a dystrophic nature.     

Effect of diethylcarbamazine citrate and anti-inflammatory drugs on experimental onchocercal punctate keratitis

Subconjunctivally injected Onchocerca lienalis microfilariae (Mf) migrate into the guinea pig cornea, resulting, when the microfilariae die, in punctate stromal opacities resembling those of human onchocerciasis. Administration of diethylcarbamazine citrate (DEC-C) following subconjunctival injection of Mf increased the proportion of dead Mf in the cornea, the number of punctate opacities and the extent of peripheral corneal neovascularization. Betamethasone (a synthetic steroid) and lodoxamide tromethamine (an inhibitor of mediator release from mast cells) inhibited the formation of punctate opacities. Chlorpheniramine maleate and cimetidine (H1 and H2 histamine receptor antagonists), given together, did not alter the formation of punctate opacities but inhibited the peripheral corneal neovascularization. These observations suggest that mast cell mediators other than histamine may be of importance in the formation of the corneal punctate opacities.     

Unusual corneal deposit after the topical use of cyclosporine as eyedrops

PURPOSE: To report a patient who developed bilateral corneal opacities 5 days after the beginning topical cyclosporine. METHODS: Case report. A 45-year-old woman with graft-versus-host disease presented with bilateral corneal deposits. She had been treated with topical physiological saline solution, sodium hyaluronate, ofloxacin, fluorometholone, and oxybuprocaine. Cyclosporine eyedrops were added for persistent corneal epithelial defect bilaterally. Five days after cyclosporine, she complained of visual loss and dense corneal opacities were detected that covered the pupil bilaterally. RESULTS: Deposits were also observed on the punctal plugs, and infrared spectroscopy and X-ray analysis showed that these deposits had properties of cyclosporine. CONCLUSION: Topical cyclosporine, alone or in combination with other eyedrops, may cause severe corneal deposits in patients with disturbance of the corneal epithelial barrier and decreased tear clearance.     

Bacillus keratitis associated with contaminated contact lens care systems

We examined two soft contact lens wearers who developed keratitis associated with Bacillus contamination of their contact lens care systems. Patient 1 developed a corneal ulcer caused by B. subtilis, and Patient 2 demonstrated multiple, diffuse, punctate corneal epithelial opacities associated with B. cereus contamination in the contact lens and lens case compartment. The contact lens cases of both patients contained Bacillus spores that survived multiple heat disinfection treatments. Three different contact lens chemical disinfection systems used for the minimum recommended time failed to kill the Bacillus organisms.     

Thygeson's superficial punctate keratitis

Thygeson's superficial keratitis is a chronic disease of the cornea characterized by focal epithelial lesions. We studied 45 cases of Thygeson's superficial punctate keratitis. Twenty-eight were male and 17 were female patients. The disease had been active in our patients from one month to 24 years. The clinical findings, course, outcome, and response to therapy were studied. Viral cultures and microscopic studies were performed on the corneal epithelial scrapings from ten cases. The average course of the disease was prolonged in patients who received topical steroid therapy. Subepithelial opacities were seen more frequently in patients who had received treatment with idoxuridine (IDU).     

Capsaicin-induced corneal changes associated with sensory denervation in neonatal rat

A single subcutaneous injection of capsaicin (50 mg/kg) to neonatal Wistar rats induced prominent corneal changes consisting of initial edematous lesions and late degenerative opacities in the epithelium and stroma, with disintegrated epithelial cells on histologic sections. Qualitative and quantitative analyses of the corneal nerves by means of gold chloride impregnation revealed a marked degeneration of the intraepithelial terminal fibers, resulting in a significant decrease in neural density by over 70% of the normal value at 4 and 6 weeks after neonatal capsaicin treatment. Thereafter, regeneration of nerve fibers occurred, but the neural density did not return to the normal level at 6 months after treatment.