[The IC3D classification of the corneal dystrophies]. / IC3D-Klassifikation von Hornhautdystrophien.

BACKGROUND: The recent availability of genetic analyses has demonstrated the shortcomings of the current phenotypic method of corneal dystrophy classification. Abnormalities in different genes can cause a single phenotype, whereas different defects in a single gene can cause different phenotypes. Some disorders termed corneal dystrophies do not appear to have a genetic basis. PURPOSE: The purpose of this study was to develop a new classification system for corneal dystrophies, integrating up-to-date information on phenotypic description, pathologic examination, and genetic analysis. METHODS: The International Committee for Classification of Corneal Dystrophies (IC3D) was created to devise a current and accurate nomenclature. RESULTS: This anatomic classification continues to organize dystrophies according to the level chiefly affected. Each dystrophy has a template summarizing genetic, clinical, and pathologic information. A category number from 1 through 4 is assigned, reflecting the level of evidence supporting the existence of a given dystrophy. The most defined dystrophies belong to category 1 (a well-defined corneal dystrophy in which a gene has been mapped and identified and specific mutations are known) and the least defined belong to category 4 (a suspected dystrophy where the clinical and genetic evidence is not yet convincing). The nomenclature may be updated over time as new information regarding the dystrophies becomes available. CONCLUSIONS: The IC3D Classification of Corneal Dystrophies is a new classification system that incorporates many aspects of the traditional definitions of corneal dystrophies with new genetic, clinical, and pathologic information. Standardized templates provide key information that includes a level of evidence for there being a corneal dystrophy. The system is user-friendly and upgradeable and can be retrieved on the website www.corneasociety.org/ic3d .

Coexistent Acanthamoeba keratitis and herpetic keratitis.

OBJECTIVE: To describe a series of patients with proved herpes simplex virus keratitis (herpetic keratitis) who also had documented Acanthamoeba keratitis. METHODS: Herpetic keratitis was documented with viral cultures, immunologic stains, or histopathologic examination for multinucleated giant cells in the corneal epithelium. Acanthamoeba organisms were identified using confocal microscopy and epithelial biopsy with hematoxylin-eosin staining. Biopsy of the stroma and epithelium was used to identify Acanthamoeba organisms in 1 case. RESULTS: Cultures for herpes simplex virus were positive in 6 of the 9 cases. Immunologic stains were positive in an additional 2 cases, and in 1 case multinucleated giant cells were present in the epithelium consistent with the diagnosis of herpes simplex virus keratitis. Tandem scanning confocal microscopic findings were positive for Acanthamoeba in 8 of the 9 cases, and all of them demonstrated Acanthamoeba organisms in epithelial scrape biopsy specimens. In 1 case, which was not evaluated with confocal microscopy, Acanthamoeba was detected using a stromal and epithelial biopsy. Two of the 9 patients had a history of contact lens use. CONCLUSION: Acanthamoeba keratitis may be present as a secondary or opportunistic infection in patients with herpetic keratitis.

An epidemic of presumed Acanthamoeba keratitis that followed regional flooding. Results of a case-control investigation.

OBJECTIVES: To investigate an outbreak of presumed Acanthamoeba keratitis (AK), to identify risk factors associated with its development, and to characterize the changing epidemiology of AK. METHODS: We performed a pairwise-matched case-control study involving 31 patients who were diagnosed as having AK between July 1993 and December 1994. Risk factors were identified using conditional logistic regression analysis. To investigate the impact of regional flooding, we stratified counties within Iowa by whether their water facilities were affected and then calculated population-based estimates of the incidence of AK. RESULTS: During the study, 43 presumed incident cases of AK were diagnosed; 31 were included in the case-control study. Cases were diagnosed based on the clinical presentation of keratitis, positive tandem scanning confocal microscopy examination results, and confirmatory cytopathologic findings. There were no positive culture specimens. On average, cases had symptoms for 8 weeks before diagnosis, most notably photophobia (94%), red eyes (94%), and pain (80%). Contact lens use (odds ratio [OR] = 44.16; P = .02) and fishing (OR = 22.62; P = .04) were independent predictors of the development of AK. The presence of a humidifier in the home (OR = 0.08; P = .03) and having household water that originated from a private well instead of the municipal water supply (OR = 0.12; P = .08) were protective. Twenty-nine of 30 cases resided in counties in which the water supplies were affected by flooding as determined by the Department of Natural Resources, Des Moines, Iowa. The incidence of AK in these counties was more than 10 times higher than that in the unaffected counties (relative risk = 10.83, 95% confidence interval, 1.48-79.49; P < .003). CONCLUSIONS: We describe an epidemic of keratitis that, based on clinicopathologic and epidemiological evidence, is consistent with AK. As in previous outbreaks of culture-proven AK, contact lens use was the major risk factor. Both the results of the case-control study and the population-based incidence estimates suggest that the recent outbreak may be caused, in part, by the effects of regional flooding. However, because the outbreak also coincided with a change in diagnostic techniques, we cannot eliminate recognition bias as the reason for the apparently changing epidemiology.

Outbreak of keratitis presumed to be caused by Acanthamoeba.

PURPOSE: A sharp increase of Acanthamoeba keratitis from two cases per year to 30 cases per year at our institution prompted this study to determine whether there was a change in the clinical characteristics, basic epidemiology, and outcome of this disease. METHODS: We reviewed all cases of Acanthamoeba keratitis diagnosed at the University of Iowa Hospitals and Clinics from mid-1993 through 1994. RESULTS: We screened 217 patients with keratitis by tandem scanning confocal microscopy and suspected Acanthamoeba in 51 patients. Diagnosis was confirmed by cytology in 43 patients (48 eyes). There were no positive cultures. Patients examined within four weeks of onset of symptoms were younger (mean age, 32.6 +/- 15.4 years) and wore contact lenses (11 of 18 patients), and infrequently herpes simplex keratitis (four of 18 patients) was diagnosed. Patients examined after four weeks were older (mean age, 54.0 +/- 19.5 years), infrequently wore contact lenses (six of 25 patients), and often had herpes simplex keratitis (12 of 25 patients). CONCLUSIONS: Corneal examination with tandem scanning confocal microscopy was associated with a marked increase in the detection of Acanthamoeba, strongly suggesting that the disease is more prevalent than suspected. Acanthamoeba may account for many cases of clinically presumed herpes simplex keratitis, the leading cause of corneal blindness in the United States. Acanthamoeba should be considered in the differential diagnosis of any unexplained keratitis, even those of short duration.

Correlation between surface water contamination with amoeba and the onset of symptoms and diagnosis of amoeba-like keratitis.

AIM: A seasonal variation was noted in the incidence of amoeba-like keratitis in Iowa. The level of contamination of amoeba in surface water has been found previously to vary on a monthly basis for Tulsa, Oklahoma, a region with a similar climate. It was hypothesised that this seasonal variation in amoeba-like keratitis correlated with the concentration of amoeba in surface water. METHODS: The monthly variation in the onset of symptoms was determined and date of diagnosis in 137 cases of amoeba-like keratitis, in Iowa, from January 1993 to the end of December 1996. RESULTS: Over a 4 year period a significant variation was found, on a monthly basis, in the onset of symptoms as reported by the patient. There were two peak periods per year when the onset of symptoms was most frequent: June and November. This corresponded closely with the concentration of amoeba in the ground water reported for a similar region in the mid-west (Tulsa, Oklahoma). A yearly increase was found in the number of cases diagnosed from January 1993 to the end of December 1996. CONCLUSIONS: This study supports the hypothesis that the concentration of amoeba in surface water may influence the rate of an amoeba-like keratitis infection.

Quantification of the reduction of glare disability after standard extracapsular cataract surgery.

Glare disability is often cited as an indication for cataract extraction, but very little objective data exist showing improvement of glare disability following standard extracapsular cataract extraction with posterior chamber intraocular lens implantation. In a series of 25 patients we determined glare disability by the reduction in visual acuity with dim room lighting (baseline) and with full room lights; with the brightness acuity tester (BAT) on low, medium, and high; with the true vision analyzer (TVA) glare light. Glare disability was quantified by the difference between the log VA postoperatively and the log VA preoperatively. Glare disability with BAT medium was no different than that with bright room lights (P greater than .05). Glare disability with BAT high was greater than that with BAT medium (P less than .01) but did not differ from that with TVA (P greater than .05). Glare disability was significantly reduced (P less than .01) six weeks postoperatively as measured by all tests except BAT low. Cataract surgery can be expected to reduce glare disability as measured by these tests.

Diagnosis of Aspergillus keratitis in vivo with confocal microscopy.

Confocal microscopy provides a new, noninvasive way of imaging the human cornea in vivo. One of its most important clinical uses is the diagnosis and management of infectious keratitis. The authors used tandem scanning confocal microscopy to image the corneas in two culture-proven cases of Aspergillus keratitis. Fungal hyphae were imaged as high-contrast filaments 6 microns in diameter from 60 to 400 microns in length. Confocal microscopy may be a fast and safe diagnostic tool in determining the presence of fungal hyphae in vivo within the human cornea.

Diagnosis of Acanthamoeba keratitis in vivo with confocal microscopy.

We present eight cases of Acanthamoeba keratitis. In each case; the Acanthamoeba organisms were visualized in the epithelium and anterior stroma using tandem scanning confocal microscopy. The organisms were highly reflective, ovoid, and were 10-25 microns in diameter. The Acanthamoeba organisms in the human corneas were identical in size and shape to Acanthamoeba organisms on an agar plate visualized with the same confocal microscope. Confocal microscopy is a useful method for identifying Acanthamoeba organisms in vivo within the corneal epithelium and anterior stroma.

Evaluation of infectious crystalline keratitis with confocal microscopy in a case series.

We sought to determine whether there are unique findings in infections crystalline keratitis (ICK) examined by confocal microscopy and if confocal microscopy is predictive for bacteriology in ICK. A retrospective review of consecutive patients with a presumed diagnosis of ICK by slit-lamp examination was performed. These patients were then examined with confocal microscope and cultured. Sixteen patients were identified by biomicroscopy. Average age was 71 years; 12 of 16 patients were women; 10 of 16 had prior penetrating keratoplasty; and 12 of 16 were taking topical steroids. Confocal microscopy revealed a variable appearance to the crystals in the corneal stroma. Eight of 16 patients had distinct needle-like deposits at varying depths in the stroma, and eight had amorphous deposits grouped at different levels of the stroma. The results of confocal microscopic examination resembled the reported histopathology with clusters of deposits, but its current resolution does not allow identification of bacterial morphology. There was no correlation of morphology with culture results. Organisms were recovered in 12 of 16 patients by culture. In 10 of 16 patients, the infection was successfully treated with topical antibiotics, usually cefazolin. Crystal morphology of ICK can be observed by confocal microscopy. No pathognomonic, single pattern for this disease is seen with the confocal microscope. The latter may be an aid in determining the clinical response to treatment.

Model for ocular tear film function.

Blepharitis patients have a number of disturbances in their tear film associated with meibomian gland dysfunction that affect evaporation and tear osmolarity. We tested a series of 156 consecutive patients, with a presumed diagnosis of blepharitis, dry eye, or allergic disease, and a series of 72 normals. We compared their tear film characteristics using tear osmolarity, tear volume, tear production (fluorophotometric and Schirmer test), tear turnover (decay constant), tear evaporation, and meibomian gland function evaluated by gland drop-out, expressed lipid viscosity, and volume. Of the 156 patients tested, we found 37 had dry eye, 10 had only allergic disease, 73 had meibomian gland dysfunction and dry eye, and 36 had only meibomian gland dysfunction. We created a model of the relative influence some of these factors had on each other using their correlation coefficients. The highest correlations for osmolarity were Schirmer test (-0.44), lipid volume low (-0.44), lipid viscosity high (0.39), gland drop-out (0.39), and tear evaporation (0.36). With regression analysis we accounted for 47% of the total variation in osmolarity, but only 17% of the variation in tear evaporation. We also present our classification system for blepharitis and dry eye patients based on our measurable physiologic parameters.

Observations on the natural history of herpes zoster ophthalmicus.

We have studied certain aspects of the natural history of acute herpes zoster ophthalmicus in placebo-treated patients followed prospectively over one year as part of a therapeutic drug trial. Observations on the incidence of ocular complications relating to the efficacy of oral acyclovir in this disease have been previously published. This report provides supplemental observations on the natural history of frequently observed ocular complications of zoster ophthalmicus: corneal hypesthesia, episcleritis, dendritiform keratopathy, stromal keratitis, anterior uveitis, and post herpetic neuralgia. These ocular complications of zoster typically present within the first two weeks of the diagnosis. This report characterizes the onset of corneal hypesthesia, episcleritis, dendritiform keratopathy, stromal keratitis, and anterior uveitis as well as interrelationships amongst these sequellae of herpes zoster ophthalmicus. Post-herpetic neuralgia occurs in 52% of patients and persists beyond a year in 22% of those affected.

Improved detection of oculomycoses using induced fluorescence with cellufluor.

Cellufluor, a fluorochrome with affinity for chitin and cellulose, was retrospectively compared to gram and giemsa stains for detection of human oculomycoses. From culture-proven specimens, this chemofluorescent stain detected hyphae or yeasts on 50% of smears previously considered negative. Cellufluor is a useful adjunct in the detection and confirmation of fungal elements from ocular specimens.