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PURPOSE: To determine whether preoperative endothelial cell density (ECD) and/or postoperative ECD after Descemet stripping automated endothelial keratoplasty (DSAEK) are associated with late endothelial graft failure (LEGF) in the Cornea Preservation Time Study (CPTS). DESIGN: Cohort study within multicenter randomized clinical trial. PARTICIPANTS: 1,007 individuals (1,223 study eyes), mean age 70 years, undergoing DSAEK for Fuchs’ dystrophy (94% of eyes) or pseudophakic/aphakic corneal edema (PACE) (6% of eyes) and followed for up to 5 years. METHODS: Central ECD was determined by a central image analysis reading center. Preoperative ECD was determined for 1209 eyes that did not fail and 14 eyes that experienced LEGF. ECD at 6 and 12 months after DSAEK, the change in ECD from preoperative to 6 and 12 months, surgeon-reported operative complications, and postoperative graft dislocation, were investigated for an association with LEGFs unrelated to other postoperative events. Univariable and multivariable Cox proportional hazards regression models were used to assess associations. RESULTS: The cumulative probability of LEGF was 1.3% (95% CI 0.8% to 2.4%). Median (IQR) preoperative ECDs were similar for eyes with LEGF (2523 (2367, 3161) cells/mm(2)) and eyes without failure (2727 (2508, 2973) cells/mm(2)) (p=0.34). ECD at 6 months was associated with LEGF (p

PURPOSE: To examine the association of donor, recipient, and operative factors on graft dislocation after Descemet stripping automated endothelial keratoplasty (DSAEK) in the Cornea Preservation Time Study (CPTS) as well as the effects of graft dislocation and elevated IOP on graft success and endothelial cell density (ECD) 3 years postoperatively. DESIGN: Cohort study within a multi-center, double-masked, randomized clinical trial. METHODS: 1,090 individuals (1,330 study eyes), median age 70 years, undergoing DSAEK for Fuchs endothelial corneal dystrophy (94% of eyes) or pseudophakic or aphakic corneal edema (6% of eyes). Recipient eyes receiving donor corneal tissue randomized by preservation time (PT) of 0-7 days (N=675) or 8-14 days (N=655) were monitored for early or late graft failure through 3 years. Donor, recipient, operative, and postoperative parameters were recorded including graft dislocation (GD), partial detachment, and pre- and post-operative IOP. Pre- and postoperative central donor ECD were determined by a central image analysis reading center. Proportional hazards, mixed effects, and logistic regression models estimated risk ratios and {99% confidence intervals}. RESULTS: Three independent predictive factors for GD were identified: a history of donor diabetes (odds ratio {OR}: 2.29 {1.30, 4.02}), increased pre-lamellar dissection central corneal thickness (OR: 1.13 {1.01, 1.27} per 25μ increase), and operative complications (OR: 2.97 {1.24, 7.11}). Among 104 (8%) eyes with GD, 30 (28.9%) developed primary donor or early failure and 5 (4.8%) developed late failure vs. 15 (1.2%; p

Purpose To evaluate the utility, safety, and efficacy of a parallel or cloverleaf retention suture in Descemet stripping automated endothelial keratoplasty that does not penetrate donor tissue and can be removed at the slit lamp for patients at high risk of graft detachment. Methods Data were prospectively collected over 9 years from patients who received a retention suture. Indications for use of a retention suture included difficulty with postoperative positioning, abnormal intraocular anatomy including glaucoma tube shunts, trabeculectomies, anterior chamber intraocular lens implants, previous vitrectomy, aniridia, and aphakia, or history of previous graft detachment. Results Of 128 surgeries, 12 (9.4%) required additional intervention for graft reattachment (rebubble). Overall, 120 grafts (93.8%) remained clear and attached either after surgery with a retention suture or after rebubble procedures. Mean endothelial cell density at 1 year was 1840 cells/mm, and mean endothelial cell density loss was 37.3%. Conclusions A cloverleaf or parallel retention suture in conjunction with Descemet stripping automated endothelial keratoplasty seems to reduce the risk of detachment in high-risk patients, while maintaining adequate cell density and graft clarity at 1 year.

PURPOSE: To identify donor and recipient factors, including eye bank tissue observations, predictive of operative complications in the Cornea Preservation Time Study. METHODS: One thousand three hundred thirty study eyes undergoing Descemet stripping automated endothelial keratoplasty for Fuchs dystrophy or pseudophakic/aphakic corneal edema were randomized to receive a donor cornea with preservation time (PT) of 0 to 7 days (N = 675) or 8 to 14 days (N = 655). Donor factors included demographics, prelamellar corneal and postlamellar lenticule dissection thickness, central endothelial cell density, and tissue processing time. Recipient factors included demographics, intraocular pressure, and glaucoma medications or surgery (trabeculectomy, laser trabeculoplasty). Eye bank observations included donor tissue folds, pleomorphism/polymegethism, and endothelial cell abnormalities. Possible tissue-related operative complications were recorded including difficult donor lenticule unfolding and positioning. Multivariable logistic regression with backward selection was used to identify statistically significant (P < 0.01) associations between factors and operative complications. RESULTS: The only factor predictive of operative complications [58 (4.4%) of 1330 surgeries] was prelamellar dissection donor corneal thickness (P = 0.002). For every 50 μm of donor corneal thickness prior to lamellar dissection, operative complication odds increased by 40% (odds ratio [99% confidence interval (CI)]: 1.40 [1.06–1.83]) adjusting for PT and whether the epithelium was on or off. The estimated mean prelamellar dissection donor corneal thickness for PT 0 to 7 days was 537 μm (99% CI: 516 μm–558 μm) compared with 567 μm (99% CI: 546 μm–588 μm) for PT 8 to 14 days (P < 0.001). CONCLUSIONS: Thicker donor tissue (prelamellar dissection) is associated with operative complications and should be considered in tissue selection for Descemet stripping automated endothelial keratoplasty lenticule preparation.

The structure of the cornea is vital to its transparency, and dystrophies that disrupt corneal organization are highly heritable. To understand the genetic aetiology of Fuchs endothelial corneal dystrophy (FECD), the most prevalent corneal disorder requiring transplantation, we conducted a genome-wide association study (GWAS) on 1,404 FECD cases and 2,564 controls of European ancestry, followed by replication and meta-analysis, for a total of 2,075 cases and 3,342 controls. We identify three novel loci meeting genome-wide significance (P, Fuchs endothelial corneal dystrophy (FECD) is one of the most common reasons for corneal transplantation, and is known to cluster in families. Here, the authors discover new genetic loci associated with FECD with sex-specific effects and implications for disease mechanism.

Purpose To associate donor, recipient, and operative factors with graft success 3 years after Descemet stripping automated endothelial keratoplasty (DSAEK) in the Cornea Preservation Time Study (CPTS). Design Cohort study within a multicenter, double-masked, randomized clinical trial. Participants One thousand ninety individuals (1330 study eyes) with a median age of 70 years undergoing DSAEK for Fuchs endothelial corneal dystrophy (94% of eyes) or pseudophakic or aphakic corneal edema (PACE; 6% of eyes). Methods Eyes undergoing DSAEK were randomized to receive a donor cornea with preservation time (PT) of 0 to 7 days (n = 675) or 8 to 14 days (n = 655). Donor, recipient, and operative parameters were recorded prospectively. Graft failure was defined as regraft for any reason, a graft that failed to clear by 8 weeks after surgery, or an initially clear graft that became and remained cloudy for 90 days. Failure in the first 8 weeks was classified further as primary donor failure or early failure, in the absence or presence of operative complications, respectively. Proportional hazards and logistic regression models were used to estimate risk ratios (RR) and 99% confidence intervals (CIs) for graft failure. Main Outcome Measures Graft success at 3 years. Results One thousand two hundred fifty-one of 1330 grafts (94%) remained clear at 3 years and were considered successful. After adjusting for PT, tissue from donors with diabetes (RR, 2.35; 99% CI, 1.03–5.33) and operative complications (RR, 4.21; 99% CI, 1.42–12.47) were associated with increased risk for primary or early failure. Preoperative diagnosis of PACE (RR, 3.59; 99% CI, 1.05–12.24) was associated with increased risk for late failure by 3 years after surgery compared with Fuchs dystrophy. Graft success showed little variation among other factors evaluated, including donor age (RR, 1.19 per decade; 99% CI, 0.91–1.56 per decade), preoperative donor endothelial cell density (RR, 1.10 per 500 cells; 99% CI, 0.74–1.63 per 500 cells), graft diameter (RR, 1.22 per 1 mm; 99% CI, 0.39–3.76 per 1 mm), and injector use for graft insertion (RR, 0.92; 99% CI, 0.40–2.10). Conclusions Descemet stripping automated endothelial keratoplasty success in the early and entire postoperative period is more likely when the donor did not have diabetes and was without operative complications and in the long-term postoperative period in recipients with Fuchs dystrophy compared with those with PACE. Mechanisms whereby diabetic donors and PACE recipients reduce the rate of graft success after DSAEK warrant further study.

Supplemental Digital Content is Available in the Text., Purpose: The aim of this study was to describe the aims, methods, donor and recipient cohort characteristics, and potential impact of the Cornea Preservation Time Study (CPTS). Methods: The CPTS is a randomized clinical trial conducted at 40 clinical sites (70 surgeons) designed to assess the effect of donor cornea preservation time (PT) on graft survival 3 years after Descemet stripping automated endothelial keratoplasty (DSAEK). Eyes undergoing surgery for Fuchs endothelial corneal dystrophy or pseudophakic/aphakic corneal edema were randomized to receive donor corneas stored ≤7 days or 8 to 14 days. Donor and patient characteristics, tissue preparation and surgical parameters, recipient and donor corneal stroma clarity, central corneal thickness, intraocular pressure, complications, and a reading center-determined central endothelial cell density were collected. Surveys were conducted to evaluate pre-CPTS PT practices. Results: The 1330 CPTS donors were: 49% >60 years old, 27% diabetic, had a median eye bank–determined screening endothelial cell density of 2688 cells/mm2, and 74% eye bank prepared for DSAEK. A total of 1090 recipients (1330 eyes including 240 bilateral cases) had: median age of 70 years, were 60% female, 90% white, 18% diabetic, 52% phakic, and 94% had Fuchs endothelial corneal dystrophy. Before the CPTS, 19 eye banks provided PT data on 20,852 corneas domestically placed for DSAEK in 2010 to 2011; 96% were preserved ≤7 days. Of 305 American Academy of Ophthalmology members responding to a pre-CPTS survey, 233 (76%) set their maximum PT preference at 8 days or less. Conclusions: The CPTS will increase understanding of factors related to DSAEK success and, if noninferiority of longer PT is shown, will have great potential to extend the available pool of endothelial keratoplasty donors. Clinical Trial Registration—URL: http://www.clinicaltrials.gov. Unique identifier: NCT01537393.

The aim of this study was to compare endothelial cell loss and graft success 6 months after endothelial keratoplasty (EK) with paired donor corneas stored in Optisol GS and Life4°C solutions and their associated storage chambers.Donor pairs were stored, one in Optisol GS and the other in Life4°C, and prepared for Descemet stripping automated EK or Descemet membrane EK. Matched pairs of recipients with Fuchs dystrophy were randomized to 1 member of each donor pair. Clarity of recipient stroma, intraocular pressure, and complications were followed for 6 months. Central endothelial images of the donor cornea at screening and 3 and 6 months after EK were analyzed by a masked central reading center. Dual grading of endothelial cell density was performed using the center method. Differences in endothelial cell density and cell loss were examined by paired analysis.Thirty-two pairs were enrolled, and 27 were analyzed (5 had improper matching or loss to follow-up). Donor age was 59 ± 14 years, median death-to-surgery time was 4 days (range, 2-9 days), 6 recipient pairs had Descemet membrane EK, and 21 had Descemet stripping automated EK. Recipient age was comparable in the Optisol GS and Life4°C groups (70 vs. 68 years, respectively, P = 0.46). Six-month central endothelial cell loss did not differ significantly between the Life4°C and Optisol GS groups (18 ± 18% vs. 20 ± 20%, respectively, P = 0.55). All recipient corneas were clear at 6 months in both groups.Endothelial cell loss and graft success were comparable at 6 months for paired donor corneas stored in Optisol GS and Life4°C.Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT01657500.

Importance Demonstrating that endothelial cell loss following Descemet stripping automated endothelial keratoplasty (DSAEK) is independent of donor cornea preservation time (PT) could increase the pool of corneal tissue available for keratoplasty. Objective To determine whether endothelial cell loss 3 years after successful DSAEK is related to PT. Design, Setting, and Participants A multicenter, double-masked, randomized clinical trial included 40 clinical sites (70 surgeons) in the United States, with donor corneas provided by 23 US eye banks. A total of 945 eyes of 769 participants were included in the Cornea Preservation Time Study that had not experienced graft failure 3 years after DSAEK, performed primarily for Fuchs endothelial corneal dystrophy (96% of the cohort). The study was conducted from April 16, 2012, to June 5, 2017. Interventions DSAEK with random assignment of a donor cornea with PT of 0 to 7 days (0-7d PT) or 8 to 14 days (8-14d PT). Main Outcomes and Measures Endothelial cell density (ECD) at 3 years determined by a central image analysis reading center from clinical specular or confocal central endothelial images. Results Nine hundred forty-five eyes of 769 participants (median age, 70 years [range, 42-90 years], 60.8% women, 93.0% white) in the Cornea Preservation Time Study that had not experienced graft failure 3 years after DSAEK were included. At the initial eye bank tissue screening, mean (SD) central ECD was 2746 (297) cells/mm 2 in the 0-7d PT group (n = 485) and 2723 (284) cells/mm 2 in the 8-14d PT group (n = 460). At 3 years, the mean (SD) ECD decreased from baseline by 37% (21%) in the 0-7d PT group and 40% (22%) in the 8-14d PT group to 1722 (626) cells/mm 2 and 1642 (631) cells/mm 2 , respectively (mean difference, 73 cells/mm 2 ; 95% CI, 8-138 cells/mm 2 ; P = .03). When analyzed as a continuous variable (days), longer PT was associated with lower ECD (mean difference by days, 15 cells/mm 2 ; 95% CI, 4-26 cells/mm 2 ; P = .006). Endothelial cell loss (ECL) was comparable from 4 to 13 days’ PT (n = 878; 36%-43% when tabulated by day). Available extension study ECD results at 4 years mirrored those at 3 years in the 203 eyes in the 0-7d PT group (mean [SD] ECD, 1620 [673] cells/mm 2 and mean [SD] ECL, 41% [23%]) and 209 eyes in the 8-14d PT group (mean [SD] ECD, 1537 [683] cells/mm 2 and mean [SD] ECL, 44% [23%]) (mean difference, 112 cells/mm 2 ; 95% CI, 5-219 cells/mm 2 ; P = .04). Conclusions and Relevance Although ECL 3 years after Descemet stripping automated endothelial keratoplasty is greater with longer PT, the effect of PT on ECL is comparable from 4 to 13 days’ PT. Trial Registration clinicaltrials.gov Identifier:NCT01537393

The aim of this study was to describe the clinicopathologic correlation of textural interface opacities (TIOs) in a Descemet stripping automated endothelial keratoplasty (DSAEK) donor button after its removal.A 75-year-old woman underwent combined phacoemulsification with intraocular lens placement and DSAEK in her right eye. She had TIOs 1 week postoperatively and continued to have poor visual acuity 8 months postoperatively. The original DSAEK graft was removed, and a repeat DSAEK procedure with a new donor disc was performed. A control corneal button was obtained from a 79-year-old woman who suffered chronic rejection and had a failed DSAEK. Both corneal specimens were sent for light and electron microscopy.Light microscopy of the donor tissue from the patient with TIOs showed stromal irregularities projecting from the cut anterior surface and the expected decrease in the endothelial cell density associated with the procedure and with artifacts. Electron microscopy examination showed irregular collagen fibrils of varying lengths at the stromal surface. Light and electron microscopy examination of the donor tissue from the control patient showed a smooth anterior stromal surface without projecting collagen fibrils.The histopathology of the endothelial disc from the patient with TIOs demonstrated variably irregular lamellae that extended from the anterior donor corneal stromal surface. These extending lamellae were absent on the anterior stromal surface of the control corneal disc, suggesting that they are one possible cause of TIOs and the subsequent suboptimal best-corrected visual acuity and quality of vision experienced by a subset of DSAEK-operated patients.

Importance Demonstrating that success of Descemet stripping automated endothelial keratoplasty is similar across donor cornea preservation times (PTs) could increase the donor pool. Objective To determine whether the 3-year rate of graft success using corneal donor tissue preserved 8 to 14 days is noninferior to that of donor tissue preserved 7 days or less. Design, Setting, and Participants A multicenter, double-masked, randomized noninferiority clinical trial was conducted from April 16, 2012, to June 5, 2017, at 40 clinical sites (70 surgeons) in the United States, with donor corneas provided by 23 US eye banks. A total of 1090 individuals (1330 study eyes) underwent Descemet stripping automated endothelial keratoplasty (1255 eyes [94.4%] for Fuchs endothelial corneal dystrophy). Interventions Descemet stripping automated endothelial keratoplasty with random assignment of a donor cornea with a PT of 7 days or less (0-7d PT) or 8 to 14 days (8-14d PT). Main Outcomes and Measures Graft success at 3 years. Results Of the 1090 participants (1330 study eyes; 60.2% women and 39.8% men; median age at enrollment, 70 years [range, 42-90 years]), the 3-year cumulative probability of graft success was 95.3% (95% CI, 93.6%-96.9%) in the 0-7d PT group and 92.1% (95% CI, 89.9%-94.2%) in the 8-14d PT group (difference, 3.2%). The upper limit of the 1-sided 95% CI on the difference was 5.4%, exceeding the prespecified noninferiority limit of 4%. The difference was mostly owing to more primary donor failures in the 8-14d PT group, with the conditional probability of failure after the first month being 2.4% in the 0-7d PT group and 3.1% in the 8-14d PT group. In preplanned secondary analyses, longer PT was associated with a lower rate of graft success (unadjusted hazard ratio for graft failure per additional day of PT, 1.10; 95% CI, 1.03-1.18; P = .008 [PT analyzed as days]), with success rates of 96.5% (95% CI, 92.3%-98.4%) for PT of 4 days or less, 94.9% (95% CI, 92.5%-96.6%) for PT of 5 to 7 days, 93.8% (95% CI, 91.0%-95.8%) for PT of 8 to 11 days, and 89.3% (95% CI, 84.4%-92.7%) for PT of 12 to 14 days ( P = .01 [PT analyzed as categorical variable]). Conclusions and Relevance The 3-year success rate in eyes undergoing Descemet stripping automated endothelial keratoplasty was high irrespective of PT. However, the study was unable to conclude that the success rate with donor corneas preserved 8 to 14 days was similar to that of corneas preserved 7 days or less with respect to the prespecified noninferiority limit. Although longer PT was associated with a lower success rate, the difference in rates was small when PT was less than 12 days. Trial Registration clinicaltrials.gov Identifier:NCT01537393

Purpose: The purpose of the study is to compare the clinical efficacy and safety of ciprofloxacin ophthalmic solution 0.3% (Ciloxan) with a standard therapy regimen (fortified tobramycin, 1.3%-cefazolin, 5.0%) for treating bacterial corneal ulcers. Methods: This randomized, parallel group, double-masked, multicenter study was conducted in 324 patients at 28 centers in the United States, Europe, and India. Patients were randomized into 2 treatment groups: 160 to ciprofloxacin and 164 to fortified tobramycin-cefazolin. Positive microbiologic cultures were obtained in 188 (58%) of 324 patients. Of these, 176 patients met protocol criteria and were evaluated for treatment efficacy: 82 in the Ciprofloxacin group and 94 in the standard therapy group. The dosing schedule for both treatment groups was 1 to 2 drops of the first study medication (ciprofloxacin or fortified tobramycin) every 30 minutes for 6 hours, then hourly for the remainder of day 1; 1 to 2 drops every hour on days 2 and 3; 1 to 2 drops every 2 hours on days 4 and 5, followed by 1 to 2 drops every 4 hours on days 6 to 14. The second medication (ciprofloxacin or cefazolin) was instilled 5 to 15 minutes after the first drug, following the same dosing frequency. Physician's judgment of clinical success, cure rate, changes in ocular signs, and symptoms and the rate of treatment failures were the primary efficacy criteria. Results: Topical ciprofloxacin monotherapy is equivalent clinically and statistically to the standard therapy regimen of fortified antibiotics. No statistically significant treatment differences were found between ciprofloxacin (91.5%) and standard therapy (86.2%) in terms of overall clinical efficacy ( P = 0.34). Similarly, no differences were noted in resolution of the clinical signs and symptoms ( P > 0.08) or the time to cure ( P = 0.55). The incidence of treatment failures was less in the ciprofloxacin group (8.5%) compared with the standard therapy group (13.8%). Significantly fewer patients treated with Ciprofloxacin reported discomfort than did patients treated with the standard therapy regimen ( P = 0.01). Conclusion: Ciprofloxacin ophthalmic solution 0.3% monotherapy is equivalent clinically and statistically to standard therapy (fortified tobramycin-cefazolin) for the treatment of bacterial corneal ulcers and produces significantly less discomfort.

Descemet stripping automated endothelial keratoplasty (DSAEK) has its own set of complications including interface abnormalities. This case series presents the largest number of patients who developed textural interface opacity (TIO) at the graft-host interface after DSAEK.This is a retrospective multicenter case series of 30 patients from 7 institutions with the finding of TIO. Clinical information collected included donor preparation details, recipient information, and surgical technique. Clinical outcomes included best-corrected visual acuity and status of TIO appearance at the last follow-up visit. Slit-lamp photographs were analyzed and compared.The majority of the patients (73%) had a best-corrected visual acuity of 20/40 or better. Four of the donor tissues were prepared with a microkeratome blade with the same lot number. Six patients had a central interface space between host and donor stromal surfaces--presumed interface fluid but potentially viscoelastic. A slight majority (57%) of patients had improvement in the severity of TIO, with 20% noted to have a complete resolution of TIO (mean follow-up of 11.9 months). Two clinical types of TIO were seen: an elongated type and a punctate type.Most patients with TIO after DSAEK obtain good visual outcomes. TIO spontaneously improves or even resolves during follow-up without intervention. The etiology of this condition is unknown, but we propose 2 different mechanisms. The elongated type could be secondary to an irregular cut of the donor with the microkeratome blade. The punctate type may be secondary to retained viscoelastic.

The two most common autosomal dominant dystrophies of the corneal stroma are lattice corneal dystrophy type I and granular dystrophy. A third autosomal dominant stromal dystrophy (Avellino) has also been recognized. Chromosome linkage analysis of four families with Avellino dystrophy mapped the disease-causing gene to chromosome 5q. Subsequent linkage analysis of two families with typical lattice dystrophy and two with typical granular dystrophy also revealed significant linkage with the same markers. Thus, each of three clinically and histopathologically distinct phenotypes is independently linked to 5q. The maximum combined lod score using all 114 affected patients was 28.6 with marker D5S393. None of the 14 known human amyloid-associated genes map to chromosome 5.

BACKGROUND: Sclera is commonly preserved in glycerin or ethanol before being used for ophthalmic surgery. The purpose of this study was to determine the ability of bacteria to survive in sclera preserved in glycerin or ethanol. METHODS: Fresh sclera was inoculated with Staphylococcus aureus, Streptococcus pneumoniae, or Pseudomonas aeruginosa and transferred to preservative vials containing glycerin, 95% ethanol, or trypticase soy broth (control) and stored at room temperature. Pieces of sclera were removed from preservative at designated intervals over a 14-day period. The sclera was then homogenized, plated on blood agar, and incubated at 37° C. Colonies were counted at 24, 48, and 72 hours. RESULTS: S. pneumoniae, P. aeruginosa, and S. aureus were recovered from glycerin preserved sclera for up to 12 hours, 1.5 days, and 8 days, respectively. No bacteria was recovered from the ethanol preserved sclera. CONCLUSIONS: Bacteria cannot be recovered from ethanol preserved sclera but can survive in glycerin preserved sclera for at least 8 days. Ethanol may offer advantages over glycerin as a scleral preservative due to its greater antibacterial activity. [Refract Corneal Surg. 1 994; 1 0:38-40.)

Posterior amorphous corneal dystrophy (PACD) is a rare, autosomal dominant disorder affecting the cornea and iris. Next-generation sequencing of the previously identified PACD linkage interval on chromosome 12q21.33 failed to yield a pathogenic mutation. However, array-based copy number analysis and qPCR were used to detect a hemizygous deletion in the PACD linkage interval containing 4 genes encoding small leucine-rich proteoglycans (SLRPs): KERA, LUM, DCN, and EPYC. Two other unrelated families with PACD also demonstrated deletion of these SLRPs, which play important roles in collagen fibrillogenesis and matrix assembly. Given that these genes are essential to the maintenance of corneal clarity and the observation that knockout murine models display corneal phenotypic similarities to PACD, we provide convincing evidence that PACD is associated with haploinsufficiency of these SLRPs.

Echography has become a valuable diagnostic tool in ophthalmology. Ultrasonic biomicroscopy (UBM) in particular may be applied to the evaluation of small lesions of the anterior segment of the eye. Disease processes such as conjunctival and iris melanoma, other forms of neoplasia, intraocular cysts, narrow angle glaucoma, and intraocular foreign bodies can be diagnostically evaluated and followed longitudinally by UBM. Combining UBM with spectroscopy may become useful in determining cell type origins of a variety of tumors. Eye banking also has an increased need for UBM in corneal tissue banking. The recent development of the Laser In Situ Keratomileusis procedure has allowed corneal surgeries to create a partial thickness flap of tissue in the cornea, remove tissue from the base of the cornea with excimer laser ablation, and replace the hinged flap. This causes a substantial change in refractive error while thinning the cornea and leaving an interface within the corneal stroma. The ability to detect this type of surgery is essential in eye banking. Ultrasonic pachymetry to determine central thickness and biomicroscopy to detect the presence of an interface are essential in avoiding the use of these corneas for transplantation purposes. Determining the topography of the preserved corneas is another potential application for ultrasonography. Using this information to reduce optical aberration after transplant is crucial in improving visual performance post transplantation. A review of the anatomy of the eye, pathology of ocular diseases relevant to UBM, and principles of eye banking will be presented.

Purpose To report a series of dislocations of the donor graft into the posterior segment associated with Descemet stripping endothelial keratoplasty (DSAEK) and to identify possible risk factors for dislocation and clinical outcomes. Design Retrospective case series. Methods Cases of donor graft dislocation into the posterior segment associated with endothelial keratoplasty were identified from the clinical experience of 7 surgeons. Observations included the preoperative surgical history of each eye, preoperative and postoperative visual acuity, management of the complication, and the postoperative clinical course. No identified cases were excluded from this series. Results Eight posterior graft dislocations were associated with DSAEK surgery. Each eye had a history of vitrectomy. Five eyes had sutured posterior chamber intraocular lenses, 1 eye had a sulcus intraocular lens, and 2 eyes were aphakic. Each eye required repeat grafting, and in 6 of 8 eyes, pars plana vitrectomy was used to remove the dislocated graft. Final visual acuities ranged from 20/30 to no light perception. Conclusions Graft dislocation into the posterior segment is a rare complication of DSAEK surgery that can lead to permanent vision loss. It has occurred in eyes that have undergone previous vitrectomy and complicated intraocular lens placement or were aphakic. As is the case with a dropped lens nucleus during cataract extraction, visual acuities after a dropped DSAEK graft range from very good to no light perception. Better postoperative results seem to be associated with prompt removal of the posteriorly dislocated graft.

Posterior amorphous corneal dystrophy (PACD) is a rare autosomal dominant corneal dystrophy and has been reported in only 11 families to date.1–9 Characteristic clinical features include partial or complete posterior lamellar corneal opacification, decreased corneal thickness (typically

BACKGROUND: Cyanoacrylate adhesive has been used to treat corneal perforations and stromal melting disorders. We examined the efficacy of aerosolization as a means of applying cyanoacrylate to the cornea. METHODS: Central corneal perforations were created with a 1-millimeter trephine in cadaver eyes. A small amount of N-butyl cyanoacrylate was delivered to the perforation site via aerosol. Experiments were also conducted with cadaver eyes which had received lamellar keratectomies to simulate corneal thinning disorders. RESULTS: Adequate seal of the 1-millimeter perforation was achieved following aerosol application of cyanoacrylate adhesive. In experiments conducted with eyes which had been perforated and those which received lamellar keratectomies, smooth anterior surface contours were achieved using the aerosol technique. CONCLUSIONS: Aerosolization may be an effective method of applying cyanoacrylate adhesive. [J Refract Corneal Surg. 1994;10:149-150.]

PURPOSE A 90-year-old woman was referred to us with a 1-month history of progressively worsening blepharoconjunctivitis. She had a complete acquired ankyloblepharon of the right lids, which resulted in the appearance of a right upper lid abscess on computed tomography. METHODS Blepharotomy with separation and debridement of the lid margins was performed. RESULTS Cultures were positive only for herpes simplex virus, type II. CONCLUSIONS Herpes simplex blepharoconjunctivitis may take many clinical forms. Our patient's severe manifestations led to the clinical and radiologic appearance of preseptal cellulitis with a right upper lid abscess.

Information has come to light regarding the granular-lattice corneal dystrophy reported in the November 1993 issue of theArchives. 1 Granular-lattice corneal dystrophy has been shown to map to chromosome 5q along with granular and lattice type I corneal stromal dystrophies. 2 This information is very exciting with regard to the genetic origin of the actual dystrophies themselves. In addition, family 3, 1 which was used for the linkage studies 2 and claimed to have no Italian heritage, did not share alleles with the Italian families in this study. This family is the only one that cannot be traced to Italy in terms of its national origin. The fact that it did not share alleles with the Italian families means that it may represent a second mutation that gave rise to the dystrophy. This appears to have occurred in a different and distinct geographic location. See also page 1080 I

Objective: To describe the phenotypic variation exhibited by members of families with combined granularlattice (Avellino) corneal dystrophy. Setting: We examined 40 patients (age range, 12 to 85 years) from six unrelated families with Avellino corneal dystrophy. This included the first individuals to ever be examined near the presumed site of origin in Italy. In addition, one family was the first to trace its origins to Germany rather than to Italy. We studied the phenotypic expression of the disease in the cornea, visual acuities, subjective complaints, complications, treatment, and histologic condition of these individuals. Results: The granular stromal lesions reach their mature quantity and size early in life, and appear as either gray and crumb-shaped deposits or superficial with an annular and planar distribution. The lattice component appears gradually, beginning and maturing later in life. The phenotypic variation within families was found to be substantial. Widely variable proportions of lattice and granular changes were found within single sibships. Visual acuities ranged from 20/20 to 20/400. Recurrent corneal erosions were present but infrequent. Subjective complaints included glare and decreased night vision. Penetrating keratoplasty was required in one individual to restore vision. Histopathologic examination revealed typical amyloid and granular deposits. Granular deposits were found replacing Bowman's membrane and extending to the corneal surface. These deposits probably represent the cause of recurrent erosions. Conclusions: Combined granular and lattice corneal dystrophy may present with substantial phenotypic variation. The disease can be found in individuals who trace their ancestry to both Italy and Germany, a wider geographic distribution than previously proposed.

Merkel cell carcinoma (Trabecular carcinoma) is a rare malignant tumor of the skin. Because these tumors tend to spread locally via the lymphatic system, very few papers have dealt with the treatment of distant metastasis. Systemic disease is uncommon and usually fatal. In this paper, we report a case of Merkel cell carcinoma with proven brain metastasis and a solid choroidal tumor. The patient responded well to radiation and chemotherapy and is alive and neurologically intact three years after diagnosis. All previous patients with metastatic Merkel cell carcinoma to the brain died within two months of the diagnosis. We use this case to discuss possible routes of metastatic dissemination and to review the treatment options in patients with distant metastatic disease. To our knowledge, this is the first reported case of presumed choroidal metastasis of Merkel cell carcinoma and the longest documented survival in a patient with brain involvement.

We use computer software graphics (MacPaint) to create individualized drawings that can be reproduced as high-resolution printouts, kept in disk storage, or transmitted via telephone lines.