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To evaluate the clinical and pathologic characteristics of conjunctival myxoid lesions, with specific focus on PRKAR1A studies, in order to distinguish neoplastic conjunctival myxoma from other myxoid conjunctival lesions.A retrospective, interventional, multicenter study of all patients with conjunctival myxoma, conjunctival stromal tumor, or reactive fibromyxoid proliferation diagnosed during 1988-2018. Patient and family medical histories and clinical and pathologic characteristics of excised lesions were assessed.There were 28 patients with conjunctival myxoid lesions diagnosed as myxoma (16/28), conjunctival stromal tumor (10/28), or reactive fibromyxoid proliferation (2/28). The patients with abundant myxoid matrix lesions (14/28, 50%) were younger (mean 49 [range 23-68] years) than those with scant-to-moderate myxoid matrix lesions (14/28, mean 61 [range 18-82] years; P = .04). Abundant myxoid matrix lesions more likely contained predominantly stellate cells (6/14 [43%] vs 0/14 [0%]; P = .05) and fibrillar collagen (13/14 [93%] vs 2/14 [14%]; P.0001), conforming to the standard morphologic definition of myxoma. Absence of PRKAR1A protein expression was found in 2 lesions with morphologic features of myxoma (2/14, 14%), 1 of which demonstrated a pathogenic mutation in the PRKAR1A gene. There was no difference between the lesions with respect to other clinical and pathologic parameters.PRKAR1A plays a role in the development of a subset of conjunctival myxomas, particularly in tumors fulfilling stringent morphologic criteria for myxoma. With the exception of PRKAR1A studies, current immunohistochemical panels cannot reliably distinguish between neoplastic conjunctival myxomas and other myxoid lesions, underscoring the importance of morphology in establishing accurate diagnosis.

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 The purpose of this study was to report a large series of patients with peripheral hypertrophic subepithelial corneal degeneration (PHSCD) and differentiate the condition from Salzmann nodular degeneration (SND). Methods We retrospectively reviewed the charts of 49 patients diagnosed with PHSCD and reported their clinical, refractive, and topographic/tomographic findings. Results Most of the eyes were white and quiet. Minimal variable injection was present in a few eyes usually in the presence of pseudopterygium. Typical corneal involvement consists of peripheral circumferential-elevated whitish subepithelial opacities with fine superficial vessels along the limbus and linear deposits of iron in the epithelium along the central edge of the opacification. The typical topographic/tomographic findings consist of flattening directly over the corneal opacification with central flattening aligning with the axis of the opacification. In all subjects, the mean refractive astigmatism was significantly less than the mean topographic/tomographic Sim K astigmatism. Thirty-five eyes underwent surgical excision. The surgical eyes demonstrated significantly less astigmatism and better best-spectacle corrected visual acuity than pre-op. Moreover, all the eyes that underwent surgery for discomfort experienced significant improvement in their symptoms. Histopathology of the keratectomy specimens demonstrated paucicellular subepithelial fibrosis with overlying epithelium that was variable in caliber. Conclusions PHSCD is distinct from SND, primarily occurring in middle-aged women, bilateral and fairly symmetric with larger more peripheral opacities than SND, and absence of inflammatory signs and symptoms.

To evaluate endophthalmitis rates after penetrating keratoplasty (PK) and endothelial keratoplasty (EK) and to compare clinical outcomes.Retrospective cohort study.The medical and billing records of several large academic private practices in Philadelphia, PA were electronically queried for all surgical visits during which a PK or EK was performed between April 1, 2012 and August 31, 2018. Demographic information and transplant characteristics were recorded for each patient and office visit. An additional query was performed to identify all cases of endophthalmitis based on diagnosis and procedure billing codes. Charts of patients with endophthalmitis were individually reviewed, and information was collected on triggering factors, clinical evaluation at presentation, management, culture data, visual outcomes, and graft survival rates. The main outcome measure was incidence of presumed infectious endophthalmitis following PK or EK.During the study period, 1676 PKs and 2292 EKs for 3069 patients were performed. The mean age of patients in this transplant cohort was 66.4 (± 17.5) years and 54.6% of patients were women. Sixteen cases of endophthalmitis occurred during the study period; 12 cases of endophthalmitis occurred after PK and 4 cases occurred after EK. The rate of endophthalmitis after EK (4 of 2292; 0.2%) was significantly lower than that after PK (12 of 1676; 0.7%) (P = .01). In addition, the odds of developing endophthalmitis after PK or EK performed in conjunction with anterior vitrectomy were significantly higher than after either PK or EK alone (odds ratio 8.66; 95% confidence interval 2.98-25.18; P.001). Visual acuity outcomes were poorer after PK-related endophthalmitis than EK-associated cases (P = .01). The rate of graft failure at final follow-up was significantly higher in post-PK endophthalmitis (P = .02).In this large cohort of patients undergoing either PK or EK, rates of endophthalmitis were low for both procedures and significantly lower for EK compared with PK. Eyes with endophthalmitis after PK had poorer visual acuity outcomes and graft prognosis compared with those with endophthalmitis after EK.

Purpose Longstanding corneal edema can lead to anterior stromal scarring that may limit visual acuity following Descemet stripping endothelial keratoplasty (DSEK). The ideal time to perform DSEK to prevent permanent changes is unclear. Our aim is to determine whether earlier DSEK is associated with improved visual outcomes. Design Cohort study. Methods Setting: Institutional. Study Population: Total of 120 eyes of patients who underwent DSEK for corneal edema following cataract surgery (CE); 87% of eyes had a diagnosis of Fuchs dystrophy. Intervention: Post-DSEK visual acuity was compared in patients who underwent DSEK ≤6 months vs >6 months after CE. Linear and logistic regression were performed to assess the relationships between DSEK timing and best spectacle-corrected visual acuity (BSCVA) while accounting for baseline preoperative patient characteristics. Main Outcome Measures: Postoperative best-corrected visual acuity 6 months after DSEK (POM6 BSCVA). Results Median CE-to-DSEK time was 8.62 (interquartile range [IQR] 12.28) months (n = 120). Overall median preoperative VA and POM6 BSCVA were 0.54 (IQR 0.68) and 0.24 (IQR 0.16), respectively. Median POM6 BSCVA was better in patients with CE-to-DSEK time ≤6 months (median 0.18, IQR 0.19) vs >6 months (median 0.30, IQR 0.21) ( P = .014). A significant relationship was found between CE-to-DSEK time and POM6 BSCVA (coefficient = 0.002, P = .033), accounting for preoperative vision and pachymetry. Patients who underwent DSEK ≤6 months after CE were more likely to achieve POM6 BSCVA better than 20/40 (odds ratio = 3.73 P = .035). Conclusion Performing earlier DSEK for pseudophakic corneal edema appears to be associated with improved vision. Further prospective study is warranted to determine the optimal time to perform DSEK in patients with pseudophakic corneal edema.

PurposeTo compare sequential versus concomitant surgery of glaucoma drainage implant (GDI) and Boston keratoprosthesis type 1 (KPro).MethodsPatients who received GDI and KPro in the same eye were divided into 2 groups: GDI placement prior to KPro surgery (sequential group) or GDI placement concomitant with KPro surgery (concomitant group). Main outcome measures were GDI failure, defined as intraocular pressure (IOP) >21 mm Hg, less than a 20% IOP reduction from baseline, or IOP ResultsThirty-five eyes were included in the study: 17 in the sequential group and 18 in the concomitant group. The cumulative incidence of failure after 5 years of follow-up was 23.5% in the sequential group and 27.8% in the concomitant group (p = 0.250). Mean BCVA was significantly better in the concomitant group after 6 months, 1 year, 2 years, and at the last follow-up (pConclusionsGlaucoma drainage implant placement at the time of KPro surgery had similar cumulative incidence of failure, but significantly favorable visual outcomes, compared to sequential surgeries. The most frequent complication was GDI erosion, occurring in approximately one fourth of the patients.

Objective To determine whether the 10-year success rate of penetrating keratoplasty for corneal endothelial disorders is associated with donor age. Design Multicenter, prospective, double-masked clinical trial. Participants A total of 1090 participants undergoing penetrating keratoplasty at 80 sites for Fuchs' dystrophy (62%), pseudophakic/aphakic corneal edema (34%), or another corneal endothelial disorder (4%) and followed for up to 12 years. Methods Forty-three eye banks provided corneas from donors aged 12 to 75 years, using a randomized approach to assign donor corneas to study participants without respect to recipient factors. Surgery and postoperative care were performed according to the surgeons' usual routines. Main Outcome Measures Graft failure defined as a regraft or, in the absence of a regraft, a cloudy cornea that was sufficiently opaque to compromise vision for 3 consecutive months. Results In the primary analysis, the 10-year success rate was 77% for 707 corneas from donors aged 12 to 65 years compared with 71% for 383 donors aged 66 to 75 years (difference, +6%; 95% confidence interval, −1 to +12; P = 0.11). When analyzed as a continuous variable, higher donor age was associated with lower graft success beyond the first 5 years ( P Conclusions Although the primary analysis did not show a significant difference in 10-year success rates comparing donor ages 12 to 65 years and 66 to 75 years, there was evidence of a donor age effect at the extremes of the age range. Because we observed a fairly constant 10-year success rate for donors aged 34 to 71 years, which account for approximately 75% of corneas in the United States available for transplant, the Cornea Donor Study results indicate that donor age is not an important factor in most penetrating keratoplasties for endothelial disease. Financial Disclosure(s) Proprietary or commercial disclosure may be found after the references.

To evaluate the clinical outcomes of mycophenolate mofetil (MMF) treatment of mucous membrane pemphigoid (MMP).This is a retrospective analysis of consecutive patients with clinical MMP seen in the Ocular Surface Disease Clinic at the Wills Eye Institute, between January 1, 2004, and December 31, 2010, treated with MMF. The main outcomes measured were control of inflammation and discontinuation of MMF.A total of 23 MMP patients taking MMF were identified. The median age of the MMF-treated patients was 77.0 years. Eleven of the 23 patients (47.8%) had biopsy-proven MMP. All patients were at least Foster grading system stage 2, with most stage 3 or 4. Eight patients (34.8%) failed previous treatments with dapsone, methotrexate, prednisone, azathioprine, cyclophosphamide, or 6-mercaptopurine. The average duration of MMF treatment was 23.32 ± 33.17 months (range 1-124.83 months, median 7.4 months). Of the 23 patients with MMP, control of inflammation was achieved with MMF within 3 months for 56.5% [95% confidence interval (CI) 54.5-59.6], within 6 months for 69.6% (95% CI 65.2-76.6), and within 12 months for 82.6% (95% CI 75.3-92.4) of the patients. Nineteen patients (82.4%) achieved control of inflammation, with 16 of the 19 (84.2%) achieving control of inflammation with MMF as monotherapy. Fifteen patients were treated with MMF as initial therapy. Twenty-one percent of patients (5 of 23) were taken off MMF for failure of inflammatory control (4) or an allergic reaction (1).Treatment of MMP with MMF in this uncontrolled case series resulted in control of inflammation in the majority of patients with minimal side effects. Our data support consideration of MMF as an initial treatment option for active ocular MMP.

PURPOSE To present a large case series of epithelial ingrowth or implantation following endothelial keratoplasty (EK) with the purpose of identifying the common causes as well as the various clinical presentations. We aim to determine the typical clinical course and the most effective treatment for this rare but serious complication. METHODS This is a retrospective study of 13 patients who developed epithelial ingrowth or implantation post-EK. Slit lamp photographs were independently examined along with other diagnostic imaging and histopathology to confirm the diagnosis. Patient medical records including operative reports were reviewed to determine the number of surgeries that occurred before EK and details of surgical technique, for example, whether venting incisions were performed. Records from follow-up visits were reviewed to determine the natural progression and management of these cases. The literature was reviewed and a meta-analysis was performed. RESULTS The patients were divided into 5 groups according to the type of epithelial presentation. Eight patients had involvement within the interface away from the visual axis. One patient had ingrowth in the interface within the visual axis, 2 had retrocorneal involvement, and 1 had anterior chamber involvement. One had both retrocorneal and anterior chamber involvement. Venting incisions were performed in 8 patients, but only 1 had ingrowth related to the venting incision. Nine patients were observed without evidence of significant progression. Four patients had surgical treatment to remove the epithelium. CONCLUSIONS Epithelial ingrowth or implantation occurs most commonly within the interface away from the visual axis and typically does not progress. The presentation of a homogeneous gray-white interface opacity is characteristic. Ingrowth can result from venting incisions, but rarely does. Other causes are eccentric trephination or loose donor or host epithelium being dragged into the eye at the time of surgery.

To report a case series of eyes with posterior polymorphous corneal dystrophy and steep nonkeratoconic corneas.Retrospective, descriptive, nonrandomized case series.Thirty-five eyes of 18 patients (14 cases from 6 families and 4 isolated cases) with diffuse posterior polymorphous corneal dystrophy had mean topographic simulated keratometry readings of 52.21 diopters (D), with a range of 46.47 D to 59.86 D and an SD of 3.69 D, with no slit-lamp or topographic findings suggestive of keratoconus.The patients in this series demonstrate diffuse posterior polymorphous corneal dystrophy and nonkeratoconic steep corneas.

PURPOSE To report the first case of fungal keratitis caused by presumed Carpoligna species. METHODS A 37-year-old gardener sustained a full-thickness, stellate corneal laceration while cutting wood outdoors with a circular saw. Two months after surgical repair, he developed a severe infectious keratitis with descemetocoele at the apex of the original stellate laceration. RESULTS Culture results confirmed fungal elements without evidence of bacteria. Oral and topical voriconazole were initiated. Due to compliance and cost issues, voriconazole was replaced with natamycin 5% prior to discharge from hospital. The patient improved and healed without perforation. The patient was left with a central stromal scar. DNA extraction from the fungal colony allowed PCR amplification of the 28s ribosomal RNA region of the fungus that led to the diagnosis of Carpoligna pleurothecii. Corticosteroids were never used during the patient's treatment. CONCLUSION This is the first reported case of infectious keratitis caused by presumed Carpoligna species. The treatment for Carpoligna pleurothecii keratitis includes voriconazole, natamycin, and possibly amphotericin B.

PURPOSE: To describe the histopathologic findings and relevant clinical details of 3 patients undergoing penetrating keratoplasty (PK) after failure of posterior lamellar endothelial keratoplasty (EK). METHODS: Retrospective clinicopathologic case series. Patients 1 and 2 underwent EK for pseudophakic bullous keratopathy. Patient 3 underwent EK for persistent corneal edema secondary to a Descemet membrane (DM) detachment after cataract extraction. Patient 1 had persistent diffuse corneal edema and broad, long-standing iridocorneal adhesions that precluded repeat EK. Patient 2 had high intraocular pressure and severe anterior chamber inflammation 1 day postoperatively with subsequent noncorneal clearing and elected PK over repeat EK. Progressive corneal edema with resultant poor visual acuity after EK was the reason for PK in patient 3. RESULTS: Histopathologic examination disclosed thickened, edematous corneas with attenuated endothelium consistent with graft failure caused by endothelial decompensation in all 3 cases. Although various degrees of posterior lamellar graft detachment were observed in each instance, significant parts of each graft remained adherent to the host stroma or to segments of residual host DM. The wounds in the adherent areas, although discernible, were relatively inconspicuous, resembling those seen at the flap-stromal interface after laser in situ keratomileusis. The donor graft endothelium was atrophic in all cases, and a delicate retrocorneal fibrous membrane was present in 2 cases. Most of the graft in cases 1 and 2 remained adherent, with small areas of peripheral detachment. In contrast, the graft in case 3 adhered peripherally but had separated from the stroma centrally, forming a thin cleft. CONCLUSIONS: Histopathology suggests endothelial decompensation, incomplete graft adherence, and the formation of retrocorneal fibrous membranes as possible etiologies for EK failure. The adherence of endothelial grafts to residual host DM suggests that it may not be necessary to remove optically clear DM before endothelial graft placement. The inconspicuous nature of the EK interface suggests that it may not play a large role in image degradation, although more study is needed.

To identify current indications and trends in indications for penetrating keratoplasty (PKP) and associated procedures. Retrospective chart review of all patients who underwent PKP at Wills Eye Institute from January 1, 2001, to December 31, 2005. A total of 1,162 cases were performed in this 5-year period. Leading indications for PKP were pseudophakic corneal edema (PCE) in 330 (28.4%) cases, followed by regraft in 250 (22.0%), keratoconus in 186 (16%), and Fuchs’ endothelial dystrophy in 126 (10.8%) cases. Of the 330 cases of PCE, 232 (70.3%) were associated with posterior chamber intraocular lenses (PCIOLs) and 96 (29.1%) with anterior chamber lenses. In 330 eyes with PCE, the lens was not exchanged in 246 (74.5%) cases and was exchanged in 76 (23%) cases. Seventy of the exchanged lenses were anterior chamber intraocular lenses (ACIOLs) and six lenses were PCIOLs. In cases of ACIOL exchanges, 10 were for scleral sutured IOLs, 18 for PCIOLS, and 42 for another ACIOL. Pseudophakic corneal edema remains the leading indication for PKP at our institution followed by regraft, continuing a trend noted in our previous studies. Although the percentage of cases of PCE associated with PCIOLS increased, fewer lenses were exchanged, perhaps reflecting increased confidence in biocompatibility of newer IOLs. The decrease in overall number of corneal transplants in these 5 years continues a trend noted in our previous study and mirrors the national decline in PKP.

PURPOSE To report the results of unilateral implantation of a Boston keratoprosthesis (BKPro) in patients with good visual acuity in the fellow eye. METHODS A retrospective analysis of 37 eyes of 37 patients who underwent an optical BKPro and had a preoperative best-corrected visual acuity (BCVA) of 20/40 or better in the fellow eye at Wills Eye Hospital from September 2005 to November 2013 was performed. Patients were excluded if they had less than 1 year of postoperative follow-up. RESULTS The mean follow-up after BKPro implantation was 31.7 months (range, 12-78 months; SD, 21). Twenty-eight patients underwent a BKPro for failed penetrating keratoplasty, and 9 patients underwent a primary BKPro. The most common complications were elevated intraocular pressure and retroprosthetic membrane formation in 16 eyes (43%) each. The mean final BCVA (1.0 ± 0.8 logMAR; median, 1.0 logMAR) was statistically better than the mean preoperative BCVA (1.8 ± 0.2 logMAR; median, 1.8 logMAR) (P < 0.001). The probability of postoperative BCVA of 20/200 or better was 54.1% with an average 45.1 ± 4.7 (confidence interval: 35.9-54.3) months of follow-up. The probability of postoperative BCVA of 20/40 or better was 37.8% with an average 51.8 ± 4.9 (confidence interval: 42.0-61.5) months of follow-up. CONCLUSIONS Half of the patients in our study achieved the minimum visual acuity required for binocular functioning, whereas one-third of the patients achieved a BCVA somewhat similar to that of the contralateral eye. The complications were similar to those reported previously with good retention of the device. BKPro implantation is a reasonable option for patients with good vision in the fellow eye.

To report the short-term safety profile and clinical outcomes of scleral fixation of intraocular lenses (IOLs) using Gore-Tex suture.Retrospective, interventional case series. 85 eyes of 84 patients undergoing ab externo scleral fixation of a Bausch and Lomb Akreos AO60 or Alcon CZ70BD IOL using Gore-Tex suture. Primary outcome measures were change in visual acuity and occurrence of intraoperative and postoperative complications with minimum follow-up of 90 days.85 eyes of 84 patients were identified. Mean logarithm of the minimum angle of resolution visual acuity improved from 1.43±0.72 (20/538 Snellen equivalent) preoperatively to 0.64±0.61 (20/87 Snellen equivalent) postoperatively (p0.001). Mean follow-up was 325 days (median 264 days, range 90-996 days). There were no intraoperative complications noted. Postoperative complications included hypotony in eight patients (9.4%), ocular hypertension in six eyes (7%), vitreous haemorrhage in six eyes (7%), hyphema in two eyes (2%), serous choroidal detachment in two eyes (2%), cystoid macular oedema in two eyes (2%) and corneal oedema in one eye (1%). There were no cases of postoperative endophthalmitis, suture erosion/breakage, retinal detachment, suprachoroidal haemorrhage, uveitis-glaucoma-hyphema syndrome or persistent postoperative inflammation in the follow-up period.Ab externo scleral fixation of IOLs with Gore-Tex suture was well tolerated in all cases. No suture-related complications were encountered. This procedure led to improvement in visual acuity and was not associated with significant intraoperative or postoperative complications.

Purpose To describe the clinical features, clinical course, and response to therapy of an atypical peripheral corneal opacification. Methods Retrospective case series review. Results Six patients are described with similar findings of bilateral, fairly symmetric, peripheral, hypertrophic, subepithelial corneal opacification. All patients were female and had no concurrent systemic conditions. All six patients complained of ocular irritation. Three patients complained of blurred vision, and one patient had monocular diplopia. In three patients followed up for more than 30 months, the corneal opacification remained stable in one patient and enlarged in the other two patients. The other three patients were examined once or twice, so the evaluation of disease progression was precluded. Two patients were treated surgically with superficial keratectomy followed by 2-minute intraoperative application of 0.02% mitomycin-C. Pathologic examination of bilateral keratectomy specimens from one patient showed subepithelial fibrosis without inflammation. Conclusions The clinical findings are reminiscent but atypical of Salzmann nodular degeneration.

To identify current indications and trends in indications for penetrating keratoplasty and associated procedures.Retrospective chart review of all patients who underwent penetrating keratoplasty at Wills Eye Hospital from January 1, 1996 to December 31, 2000.A total of 1,529 corneal transplants were performed during this 5-year period. The leading indications for penetrating keratoplasty were pseudophakic bullous keratopathy (PBK) in 416 (27.2%) cases, followed by regraft in 276 (18.1%), keratoconus in 236 (15.4%), and Fuchs endothelial dystrophy in 233 (15.2%) cases. Of the 416 PBK cases, 271 (65.1%) were associated with posterior chamber intraocular lenses (PC IOLs), 141 (33.9%) with anterior chamber intraocular lenses (AC IOLs), and four (1%) with iris-fixated lenses. Of these 141 AC IOLs, 107 (75.9%) were closed-loop and 34 (24.1%) were open-loop. Of the 416 PBK cases, in 281 (67.5%) cases, the IOL was not exchanged; in 127 (30.5%) cases, the IOL was exchanged; in six (1.5%) cases, the IOL was repositioned; and in two (0.5%) cases, the IOL was removed at the time of surgery. Of the 271 PC IOLs, 248 (91.5%) were not exchanged and 19 (7.1%) were exchanged. Of the 141 AC IOLs, 33 (23.4%) were not exchanged and 104 (73.7%) were exchanged (p = 0.001).Pseudophakic bullous keratopathy remains the leading indication for corneal transplantation at our institution followed by regraft. The percentage of PBK cases associated with PC IOLs has increased significantly, whereas the percentage associated with AC IOLs has decreased. The frequency of regraft has also increased significantly.

PURPOSE: To describe the treatment of Schnyder's crystalline dystrophy (SCD) with excimer laser phototherapeutic keratectomy (PTK). METHODS: Chart review of three patients (four eyes) with SCD treated with PTK between March 1992 and December 1998. Pre- and posttreatment visual acuity, subjective glare, photophobia, manifest refraction, ultrasound pachymetry, hyperopic shift, and biomicroscopic findings were studied. Brightness acuity testing (BAT) was evaluated in two eyes. RESULTS: Four eyes were treated during the study period. The average best corrected visual acuity (BCVA) improved from 20/175 to 20/40 under bright conditions. All patients reported subjective improvement in glare and photophobia. No visually significant recurrence was observed during the follow-up period which ranged from seven months to three years. The mean spherical equivalent refractive shift was +3.28 diopter (D). One patient developed irregular astigmatism from an eccentric ablation. No vision threatening complications were observed. CONCLUSION: PTK can be effective at improving visual symptoms in patients with SCD. It can be a useful therapeutic alternative to lamellar or penetrating keratoplasty in these patients.

Purpose We report two patients with primary graft failure (PGF) from a mutual donor. The surgery in each case was done by a different surgeon experienced in penetrating keratoplasty (PK). The cases were managed differently in the postoperative period with good visual results in both cases. We discuss the various management options available when confronted with apparent PGF in the early postoperative period. Methods Both patients had excessive graft edema and haze in the immediate postoperative period. Case 1 received a repeat graft after 4 weeks because there was no improvement in graft clarity despite intensive topical steroids. In case 2, repeat surgery was deferred with ultimate gradual improvement in the clarity of the graft over a period of 6 months. Results Both patients obtained clear grafts with a visual acuity of 20/30 at 15 months after surgery. Conclusion Primary graft failure is a rare complication of PK. Repeat PK is the most definitive therapeutic alternative, although some grafts may clear without any additional surgery. We suggest all cases of PGF be observed for > or = 3-4 weeks for signs of graft clearing before proceeding with a repeated PK.

Purpose: To present an aniridic patient with failed penetrating keratoplasties OU (PK) who underwent successful repeat PK OD, which has remained clear for 4 years with continuous bandage lens (BCL) wear. Methods: A 73-year-old aniridic woman who had a failed PK complicated by ocular surface disease refused limbal stem cell transplantation and underwent repeat PK in her right eye. The immediate postoperative course was uneventful except for mild irregularity of the graft epithelium. Two months postoperatively, she presented with an epithelial defect along the inferotemporal graft margin adjacent to an exposed, loose interrupted suture. The suture was removed, and although the initial epithelial defect healed, she suffered recurrent episodes of epithelial breakdown and generalized whorl epitheliopathy. A BCL was inserted and rendered prompt healing of the graft epithelium, which has remained smooth and intact for 4 years. Results: The graft remains thin and clear with a smooth and intact epithelial surface beneath the BCL and no signs of limbal stem-cell deficiency. Conclusion: Long-term BCL wear provided good corneal transplant surface protection in an aniridic patient with recurrent epithelial breakdown, thereby sparing the need for limbal stem cell transplantation and the use of potentially toxic systemic immunosuppressive therapy. Meticulous follow-up is required to monitor contact lens loss and epithelial breakdown as well as signs of secondary infectious keratitis.

Importance The identification of steep corneal curvatures in a significant percentage of patients with posterior polymorphous corneal dystrophy (PPCD) confirms this previously reported association and suggests a role for the ZEB1 protein in keratocyte function. Objective To determine whether PPCD is characterized by significant corneal steepening. Design, Setting, and Participants Cross-sectional study at university-based and private ophthalmology practices of 38 individuals (27 affected and 11 unaffected) from 23 families with PPCD. Exposure Slitlamp examination and corneal topographic imaging were performed for individuals with PPCD and unaffected family members. Saliva or blood samples were obtained from each individual for DNA isolation and ZEB1 sequencing. Corneal ZEB1 expression was measured using immunohistochemistry. Main Outcomes and Measures Percentage of individuals affected with PPCD and controls with an average keratometric value greater than 48.0 diopters (D) in each eye; the mean keratometric value averaged for both eyes of individuals with PPCD and controls; and the correlation of ZEB1 mutation with keratometric value. Results ZEB1 coding region mutations were identified in 7 of the 27 affected individuals. Ten of the 38 individuals (26.3%) had average keratometric values greater than 48.0 D OU: 10 of 27 individuals with PPCD (37.0%; 6 of 7 individuals with ZEB1 mutations [85.7%] and 4 of 20 individuals without ZEB1 mutations [20.0%]) and 0 of 11 unaffected individuals ( P = .04 for unaffected vs affected individuals; P = .004 for individuals with PPCD with vs without ZEB1 mutation). The mean keratometric value of each eye of affected individuals (48.2 D) was significantly greater than that of each eye of unaffected family members (44.1 D) ( P = .03). Affected individuals with ZEB1 mutations demonstrated a mean keratometric value of 53.3 D, which was significantly greater than that of affected individuals without ZEB1 mutations (46.5 D; P = .004). Fluorescence immunohistochemistry demonstrated ZEB1 expression in keratocyte nuclei. Conclusions and Relevance Abnormally steep corneal curvatures are identified in 37% of all individuals with PPCD and 86% of affected individuals with PPCD secondary to ZEB1 mutations. ZEB1 is present in keratocyte nuclei, suggesting a role for ZEB1 in keratocyte function. Therefore, ZEB1 may play a role in both corneal stromal and endothelial development and function, and PPCD should be considered both an endothelial dystrophy and an ectatic disorder.

To evaluate the indications and outcomes of sutureless amniotic membrane transplant (AMT; ProKera) in the management of ocular surface disorders.Chart review of patients who had ProKera (Bio-Tissue, Inc.) implantation for ocular surface disorders between June 2008 and May 2012 at 3 ophthalmology practices in Philadelphia, PA. The parameters evaluated included age, sex, indication for AMT, any other combined procedure, duration of retention of amniotic membrane, the effect of amniotic membrane on ocular surface healing, follow-up time, and complications.There were 35 eyes of 33 patients with a mean age of 68.2±19.5 years (range: 17-99 years). There were 25 male patients, and median follow-up was 110 days (mean: 164.6 days; range: 30-960 days). The indication for insertion of ProKera was nonhealing corneal ulcers of infective origin (group 1) in 9 eyes (25.7%), neurotrophic keratopathy (group 2) in 11 eyes (31.4%), chemical injury (group 3) in 5 eyes (14.3%), and other indications (group 4) in 10 eyes (28.6%). Complete or partial success was seen in a total of 44%, 64%, 80%, and 70% eyes in groups 1 to 4, respectively. Discomfort with the device was seen in 6 (17.1%) of 35 eyes, and recurrence of primary pathologic condition occurred in 5 (14.3%) of 35 eyes.ProKera treatment is easy to use and reasonably well tolerated, with moderate success in corneal ulcers and encouraging results in acute moderate chemical injury. Recurrence of primary pathologic condition is seen particularly associated with neurotrophic keratitis and dry eye syndrome.

Purpose. To describe a patient with bilateral, recurrent marginal corneal infiltrates consistent with Fuchs' superficial marginal keratitis who developed bilateral nasal pseudopterygia encroaching on the visual axis, reducing visual acuity in both eyes. Methods. Bilateral superficial keratectomy combined with conjunctival autografting was performed in each eye. Results. Best corrected visual acuity improved from 20/60 in the left eye and 20/40 in the right eye to 20/25 in both eyes. Our patient has had no disease relapses in her right eye, as of 29 months after the surgery, but experienced a mild flare-up of marginal keratitis in her left eye 8 months after the surgery. Despite this flare-up, disease symptoms in the left eye have markedly diminished. Conclusion. Superficial keratectomy combined with conjunctival autograft is a therapeutic alternative in the management of severe, recurrent Fuchs' superficial marginal keratitis. In the present case, it has resulted in an improvement in visual acuity as well as a decrease in the frequency of disease flare-ups. The clinical course of this patient suggests that the conjunctiva may play a role in the etiology of Fuchs' superficial marginal keratitis.

PURPOSE To determine whether central discoid corneal dystrophy (CDCD), previously reported as a novel corneal dystrophy, is actually Schnyder corneal dystrophy (SCD) through screening of the UBIAD1 gene in the members of the family in which CDCD was reported. METHODS Genetic analysis was performed in 3 affected members and 1 unaffected member of a pedigree with CDCD including the affected 31-year-old proband. RESULTS All 4 affected members of the described pedigree demonstrated discoid central corneal clouding, with subtle, superficial, crystalline deposits noted in one of the affected individuals. Screening of UBIAD1 in the affected individuals demonstrated a previously unreported missense mutation, p.Asp240Asn, which was not identified in an unaffected family member or in 100 control individuals. CONCLUSIONS The clinical findings of the family reported to have CDCD were indistinguishable from those found in SCD. However, CDCD was originally thought to be distinct from SCD because of the absence of positive lipid staining and the presence of alcian blue staining consistent with glycosaminoglycans in the proband's cornea. Our recent investigation has revealed that corneal specimens from other patients with SCD have also demonstrated staining for glycosaminoglycans. Discovery that mutations in UBIAD1 caused SCD allowed genetic testing of this CDCD family. Our newly reported UBIAD1 mutation suggests that CDCD is actually a variant of SCD. This report underscores the utility of genetic testing in determining whether newly described corneal dystrophies are in fact unique entities or just variants of well-known diseases.

To determine whether preoperative and/or postoperative central endothelial cell density (ECD) and its rate of decline postoperatively are predictive of graft failure caused by endothelial decompensation following penetrating keratoplasty to treat a moderate-risk condition, principally, Fuchs dystrophy or pseudophakic corneal edema.In a subset of Cornea Donor Study participants, a central reading center determined preoperative and postoperative ECD from available specular images for 17 grafts that failed because of endothelial decompensation and 483 grafts that did not fail.Preoperative ECD was not predictive of graft failure caused by endothelial decompensation (P = .91). However, the 6-month ECD was predictive of subsequent failure (P.001). Among those that had not failed within the first 6 months, the 5-year cumulative incidence (+/-95% confidence interval) of failure was 13% (+/-12%) for the 33 participants with a 6-month ECD of less than 1700 cells/mm(2) vs 2% (+/-3%) for the 137 participants with a 6-month ECD of 2500 cells/mm(2) or higher. After 5 years' follow-up, 40 of 277 participants (14%) with a clear graft had an ECD below 500 cells/mm(2).Preoperative ECD is unrelated to graft failure from endothelial decompensation, whereas there is a strong correlation of ECD at 6 months with graft failure from endothelial decompensation. A graft can remain clear after 5 years even when the ECD is below 500 cells/mm(2).

We report two histopathologically proven cases of epithelial downgrowth in which the secondary glaucoma was controlled by a double-plate Molteno tube shunt and useful vision restored with a penetrating keratoplasty. The combination of a tube shunt and penetrating keratoplasty may be indicated to maintain useful vision in advanced cases of epithelial downgrowth associated with secondary glaucoma.

We present three patients in whom epithelial downgrowth occurred following the excision of iris inclusion cysts. The sheet-like downgrowth was aggressively treated and successfully eradicated in two of the three patients. Early identification and prompt therapy are essential.

PURPOSE. To identify the genetic basis of posterior polymorphous corneal dystrophy (PPCD) through screening of four positional candidate genes and the COL8A2 gene, in which a presumed pathogenic mutation has previously been identified in affected patients. METHODS. DNA extraction, PCR amplification, and direct sequencing of the COL8A2, BFSP1, CST3, MMP9, and SLPI genes were performed in 14 unrelated, affected patients and in unaffected family members. RESULTS. In the COL8A2 gene, the previously identified, presumed pathogenic mutation (Gln455Lys) was not discovered in any of the affected patients. A missense mutation, Thr502Met, was identified in 2 of the 14 affected probands, although it was not considered to be pathogenic, as it has been identified in unaffected individuals. Although several novel and previously identified single nucleotide polymorphisms producing synonymous and missense amino acid substitutions were identified in the COL8A2, BFSP1, CST3, MMP9, and SLPI genes, no presumed pathogenic sequence variants were found. CONCLUSIONS. No pathogenic mutations were identified in the COL8A2 gene or in several positional candidate genes in a series of patients with PPCD, indicating that other genetic factors are involved in the development of this autosomal dominant corneal dystrophy.

We report a case of Ferrara intracorneal ring (Mediphacos) implantation and cataract surgery for the correction of pellucid marginal corneal degeneration. Preoperatively, the patient's uncorrected visual acuity (UCVA) was 0.05 in the right eye and 0.10 in the left eye. The best spectacle-corrected visual acuity (BSCVA) was 0.50 with -2.00 -11.25 x 80 in the right eye and 0.60 with -2.00 -5.50 x 95 in the left eye. One month postoperatively, the UCVA was 0.50 in the right eye and 0.30 in the left eye. The BSCVA was 0.80 with -1.00 in the right eye and 0.60 with -0.75 -1.25 x 160 in the left eye. Ferrara intrastromal rings and cataract surgery resulted in a more regular corneal shape with a reduction in astigmatism for good visual recovery.

Purpose To report a series of patients with no previous history of herpes simplex virus (HSV) infection who had new onset of herpetic keratitis after penetrating keratoplasty (PK). Design Noncontrolled, retrospective case series. Methods We included in the study the patients who had new onset of herpetic keratitis after penetrating keratoplasty for corneal diseases unrelated to HSV infection who were seen at the Cornea Service at Wills Eye Hospital (Philadelphia, Pennsylvania) from January 1996 to December 2002. The diagnosis of HSV epithelial keratitis was based on clinical characteristics of either a classic herpetic dendrite, a geographic ulcer, or a nonhealing epithelial defect that responded only to antiviral therapy. Results Fourteen patients were included in the study. Eight of these (57%) had presented with a geographic ulcer whereas six patients (43%) had a classic dendrite. The most common primary corneal disease that led to PK was pseudophakic bullous keratopathy (36%), followed by keratoconus (29%), Fuchs dystrophy (21%), and corneal scar unrelated to HSV (14%). Conclusions The ophthalmologist should be aware of the possibility of herpetic keratitis in eyes after PK, even in patients with no previous history of HSV infection.

Purpose To present the microbial spectrum and susceptibilities of isolates in endophthalmitis following penetrating keratoplasty. Design Interventional case series. Methods The 1,074 consecutive cases of endophthalmitis presenting to Wills Eye Hospital between 1989 and 2000 were reviewed. Fourteen patients with endophthalmitis after penetrating keratoplasty were identified, and vitreous biopsy isolates from these patients were examined. Results Eleven (78.6%) of 14 vitreous samples were culture-positive, and two others (14.3%) had organisms viewed on pathology specimen, for a total of 13 (92.9%) organism-proven cases of endophthalmitis. Isolates included 10 (76.9%) gram-positive cocci (six Streptococcus sp., three Staphylococcus sp., one identified on pathology specimen only) and three (23.1%) gram-negative organisms ( Proteus mirabilis , Serratia marcescens , one identified on pathology specimen only). Susceptibilities to organism-appropriate antibiotic testing are reported, including cefazolin (six of eight, 75.0%), ciprofloxacin (four of seven, 57.1%), nafcillin (four of six, 66.7%), and vancomycin (seven of seven, 100.0%). Conclusion This is the largest series on microbial susceptibilities in postpenetrating keratoplasty endophthalmitis. We report a high percentage of culture-positivity, and a high incidence of gram-positive species, and in particular Streptococcus species, with all tested gram-positive organisms susceptible to vancomycin.

Purpose. To present a small kindred with a unique dominantly inherited corneal stromal dystrophy. Methods. A 31-year-old man was noted to have bilateral, symmetric, central discoid corneal stromal opacification. We performed bilateral penetrating keratoplasties for decreased visual acuity, glare, and photophobia. Results. Light microscopy revealed multiple extracellular vacuoles, concentrated in the anterior one-half of the central corneal stroma. Material within the vacuoles demonstrated intense reactivity with alcian blue and colloidal iron stains, consistent with glycosaminoglycan deposition. Transmission electron microscopy demonstrated nonmembrane-bound vacuoles in the stroma that contained a faintly osmiophilic matrix and black circular profiles. Immunohistochemical analysis of the vacuolar deposits revealed that chondroitin sulfate was the primary glycosaminoglycan present. A clinical and serologic evaluation revealed no evidence of a systemic storage disorder. Genetic analysis did not reveal a mutation in the coding region of the CHST6 gene. Conclusions. Given these unique clinical and histopathologic findings as well as nearly identical clinical findings in the patient's father and one of four brothers, the authors believe that this represents a previously unreported, dominantly inherited corneal stromal dystrophy.

PURPOSE: To report a case of porphyria cutanea tarda presenting as cicatricial conjunctivitis. DESIGN: Observational study. METHODS: A 31-year-old man presented with bilateral inferior symblepharon, superior tarsal conjunctival scarring and concretions, and recurrent conjunctival and episcleral injection. RESULTS: Four years after initial presentation, the patient developed hepatitis C, and 2 years later blisters on his scalp and hands. Direct immunofluorescence studies of biopsies taken from the palpebral conjunctiva of the right lower lid were negative for cicatricial pemphigoid. A twenty-four hour urine specimen analysis revealed elevated levels of uroporphyrins and polycarboxylated porphyrins, confirming the diagnosis of porphyria cutanea tarda. The patient was treated with repeated phlebotomies and oral hydroxychloroquine, which resulted in a significant decrease in the skin lesions, conjunctival injection, and concretions under the upper lids. CONCLUSIONS: Cicatricial conjunctivitis may be a manifestation of porphyria cutanea tarda.

We report a technique in which penetrating keratoplasty is performed in conjunction with open-sky pupilloplasty in a phakic patient. The technique was used in a 27-year-old man with poor vision and severe light sensitivity in the left eye dating back to an episode of presumed herpes simplex keratouveitis 13 years previously. Examination showed a best corrected visual acuity of 20/40, a paracentral midstromal corneal scar, a fixed dilated pupil, and a clear lens. Postoperatively, the pupil was relatively round with a diameter of approximately 4.0 mm, the cosmetic result was favorable, the photophobia had resolved, and the lens and corneal transplant were clear.

Purpose To describe a patient with conjunctival metastasis as the initial manifestation of metastasis from a cutaneous melanoma. Design Single interventional case report. Methods A 48-year-old woman with a history of cutaneous axillary melanoma developed a rapidly growing conjunctival mass. Subsequent systemic evaluation disclosed asymptomatic liver metastasis. The conjunctival lesion was resected. Results Histopathologic evaluation of the conjunctival tumor disclosed an epithelioid cell melanoma located in the conjunctival stroma, without appreciable junctional activity, compatible with metastatic melanoma. Conclusions Conjunctival metastasis from cutaneous melanoma can rarely be the initial manifestation of disseminated melanoma.

Linear IgA bullous dermatosis is an uncommon mucocutaneous autoimmune disorder that is distinct from dermatitis herpetiformis and bullous pemphigoid. Two patients who had significant conjunctival involvement but minimal skin disease are described. Irreversible conjunctival scarring indistinguishable from ocular pemphigoid developed in both patients.

To identify baseline (donor, recipient, and operative) factors that affect endothelial cell loss following penetrating keratoplasty for a moderate-risk condition (principally Fuchs dystrophy or pseudophakic or aphakic corneal edema).In a subset (n = 567) of Cornea Donor Study participants, preoperative and postoperative endothelial cell densities (ECDs) were determined by a central reading center. Multivariate regression analyses were performed to examine which baseline factors correlated with ECD over time.Larger grafts (P.001), younger donor age (P.001), and female donor (P = .004) were significantly associated with higher ECD during follow-up. Median endothelial cell loss at 5 years was 68% for grafts larger than 8.0 to 9.0 mm in diameter, 75% for grafts 7.0 mm to smaller than 8.0 mm in diameter, and 74% for grafts 8.0 mm in diameter. Grafts from female donors experienced a 67% cell loss compared with a 72% cell loss among grafts from male donors. Method of tissue retrieval, donor cause of death, history of diabetes, and time from death to preservation or to surgery were not significantly associated with changes in ECD over time.Following penetrating keratoplasty for endothelial dysfunction conditions, larger donor graft size, younger donor age, and female donor were associated with higher ECD over 5 years. These data warrant exploring the possibility that similar associations may exist following endothelial keratoplasty.clinicaltrials.gov Identifier: NCT00006411.

The authors report a case of Propionibacterium acnes endophthalmitis after intracapsular cataract extraction with implantation of an anterior chamber intraocular lens. The patient's chronic inflammation persisted for 5 years after cataract surgery despite treatment with pars plana vitrectomy, intraocular lens removal, topical and oral steroids, and topical fortified antibiotics. Fluctuations in the inflammation were paralleled by changes in the size and appearance of a white plaque on the posterior corneal surface. Anterior chamber tap cultures were positive for P. acnes after 8 days of incubation under anaerobic conditions. The inflammation was not controlled until the posterior corneal plaque, which was the presumed nidus of the chronic infection, was removed and the patient was treated with intravitreal and oral antibiotics.

Congenital entropion is a rare eyelid anomaly that can cause chronic corneal erosions or ulceration. The diagnosis may be easily overlooked by both the pediatrician and the ophthalmologist, particularly when the lids are tightly closed in the crying child. We present three cases of congenital entropion associated with corneal ulceration. Each patient underwent a complete ophthalmologic examination. Examination under anesthesia, including corneal scrapings for culture and photography, was performed before surgical repair of the entropion. There were two cases of lower lid entropion and one case of upper lid entropion. In all three cases symptoms were present since birth, and the diagnosis was overlooked by the treating pediatrician. Corneal ulceration ultimately developed in all three cases. Cultures revealed Staphylococcus aureus in one case, and coagulase negative Staphylococcus in another case. Cultures were negative in one case. In all three patients the ulcers healed rapidly after surgical entropion repair. Congenital upper or lower lid entropion is an uncommon condition that does not spontaneously improve and is an important cause of corneal ulceration in infants. Recognition of this condition is often difficult, and early surgical intervention to repair the lid deformity may help to avoid permanent corneal scarring and visual loss.

Detachment of Descemet's membrane is a rare complication of cataract surgery that can cause permanent corneal decompensation. We report five patients with Descemet's membrane detachments who were successfully treated with intracameral injection of 20% sulfur hexafluoride gas with resultant resolution of their corneal edema.

The association of glaucoma and penetrating keratoplasty presents a difficult management problem, particularly when medical therapy and conventional glaucoma surgery have failed. We have found alloplastic tube shunt surgery to be an effective method for intraocular pressure control in glaucomatous eyes subjected to penetrating keratoplasty. We report 46 patients who underwent alloplastic tube shunt implantation before (13 patients, group A), in combination with (17 patients, group B), or after (16 patients, group C) penetrating keratoplasty. All groups had significantly lowered intraocular pressure postoperatively. The most common complication was graft failure, in 31% of patients in group A, 29% in group B, and 44% in group C. Other postoperative complications and results including visual outcome are reviewed. Because the management of advanced glaucoma in patients undergoing keratoplasty is difficult, alloplastic tube shunt implantation should be considered.