Herpetic Eye Disease Study: A Controlled Trial of Topical Corticosteroids for Herpes Simplex Stromal Keratitis.

PURPOSE: To evaluate the efficacy of topical corticosteroids in treating herpes simplex stromal keratitis. METHODS: The authors performed a randomized, double-masked, placebo-con- trolled, multicenter clinical trial of 106 patients with active herpes simplex stromal keratitis who had not received any corticosteroids for at least 10 days before study enrollment. Patients were assigned to the placebo group (n = 49) or the steroid group (topical prednisolone phosphate; n = 57); both regimens were tapered over 10 weeks. Both groups received topical trifluridine. Visual acuity assessment and slit-lamp biomicroscopy were performed weekly for 10 weeks, every other week for an additional 6 weeks or until removal from the trial, and at 6 months after randomization. RESULTS: The time to treatment failure (defined by specific criteria as persistent or progressive stromal keratouveitis or an adverse event) was significantly longer in the steroid group compared with the placebo group. Compared with placebo, corticosteroid therapy reduced the risk of persistent or progressive stromal keratouveitis by 68%. The time from randomization to resolution of stromal keratitis and uveitis was significantly shorter in the steroid group compared with the placebo group even though both groups included patients who were removed from the study and treated with topical corticosteroids according to best medical judgment. Nineteen (33%) of the steroid-treated patients and 11 (22%) of the placebo-treated patients completed the 10 weeks of protocol therapy and had stable, noninflamed corneas after 16 weeks. At 6 months after randomization, no clinically or statistically significant differences in visual outcome or recurrent herpetic eye disease were identified between the steroid and placebo groups. CONCLUSIONS: The topical corticosteroid regimen used in this study was significantly better than placebo in reducing persistence or progression of stromal inflammation and in shortening the duration of herpes simplex stromal keratitis. Postponing steroids during careful observation for a few weeks delayed resolution of stromal keratitis but had no detrimental effect as assessed by visual outcome at 6 months.

Episodes of microbial keratitis with therapeutic silicone hydrogel bandage soft contact lenses.

OBJECTIVE: To report the therapeutic practice patterns of silicone hydrogel (SiH) bandage soft contact lenses (BSCL) and the resultant rate of microbial keratitis (MK). METHODS: A retrospective case series of patients treated with therapeutic BSCLs for ocular surface disease from January 2006 to January 2009 in a tertiary care cornea practice. All patients had a history of ocular surface disease. RESULTS: Seventy-four patients were treated with a therapeutic SiH BSCL (102 intervals of use) for complicated ocular surface disease. Two patients developed infectious corneal infiltrates (2/102, 2.0%) [corrected]. Two of 3 MK episodes were in patients with limbal stem-cell deficiency (2/102, 2.0%). CONCLUSIONS: Therapeutic SiH BSCLs were safely used in most patients. However, results suggest that prophylactic antibiotic use did not eliminate the risk for MK with SiH BSCL use in patients with chronic ocular surface disease.

Indications, visual outcome, and ectasia in clear corneal transplants 20 years old or more.

PURPOSE: To report the indications, visual outcome, and development of ectasia in clear corneal transplants at least 20 years or more after penetrating keratoplasty (PK). METHODS: A computer search of all post-PK patients in the electronic medical records of the Cornea Service was done. Only patients with clear primary grafts aged 20 years or more were included. Main outcome measures noted were indications for surgery, final visual outcome, and postoperative complications. A subset of patients who developed ectasia clinically was also analyzed. RESULTS: One hundred forty-nine eyes of 109 patients were identified. The most common indication was keratoconus (76.5%). After average follow-up of 27 years, the mean postoperative best-corrected visual acuity was 0.29 ± 0.38 logarithm of the minimum angle of resolution (Snellen equivalent 20/39). Postoperative complications included rejection (29.5%), cataract formation (26.2%), and steroid-induced elevated intraocular pressure (15.4%). Peripheral thinning and ectasia diagnosed by slit lamp were noted in 59 eyes (39.6%), most of which were mild (54.2%), inferiorly located (66.1%), and involved the graft-host junction (81.4%). Most of the grafts that developed ectasia had a preoperative diagnosis of keratoconus (91.5%). Mean postoperative visual acuity of ectatic grafts with rigid gas permeable contact lens and/or glasses was 0.24 ± 0.25 logarithm of the minimum angle of resolution (Snellen equivalent 20/34). CONCLUSION: PK grafts can remain clear for 20 years or more and have excellent visual outcome. Most of the 20-year-old grafts in our study were in patients with keratoconus. Rejection and graft-host ectasia are problems to be encountered in long-surviving grafts. Ectatic grafts can still attain good vision with properly fitted contact lenses and glasses.

Trends in contact lens-related corneal ulcers at a tertiary referral center.

PURPOSE: To evaluate the changes and trends in the number and characteristics of contact lens-related ulcers (CLRUs) and to compare the results with those of previously published series at our institution. METHODS: Medical records of all patients diagnosed with presumed bacterial corneal ulcers seen at the Cornea Service, Wills Eye Institute, between January 1, 2004, and December 31, 2007, were retrospectively reviewed. RESULTS: Five hundred seven corneal ulcers were identified. Of these, 223 (43.9%) were contact lens (CL) related and 284 (56.1%) were not CL related. The proportion of CLRU showed a significant increase over time (P = 0.003), with significantly greater percentage of CLRU in 2006 and 2007 compared with 2004 (P = 0.004 and P = 0.005, respectively). One hundred thirty-one (58.7%) of the 223 CLRU patients were men. Many CLRUs were vision threatening, with 45.7% (92 of 201) more than 4 mm in size, 36.3% (81 of 223) associated with hypopyon, and 46.4% (103 of 222) central or paracentral in location. Pseudomonas aeruginosa was the most frequent agent isolated in CLRUs, found in 75 (63.0%) of 119 positive cultures. Soft daily-wear frequent replacement lenses were the most common lenses associated with corneal ulcers and were used in 68 (33.5%) of 203 cases. There was a history of overnight wear of CLs in more than half of the cases (121 of 223, 54.3%). Of these, 21 (9.4%) were not approved for overnight wear. CONCLUSIONS: There was a significant increase in the number of cases of presumed bacterial keratitis associated with soft CL wear over the study period from 2004 to 2007 at our institution. The significant increase in CLRU noted from 1996 to 1999 to 1999 to 2002 reported previously seems to have continued between 2004 and 2007.

Herpes zoster ophthalmicus: comparison of disease in patients 60 years and older versus younger than 60 years.

OBJECTIVE: To study the clinical course of herpes zoster ophthalmicus (HZO) and to compare the demographics, treatments, and outcomes in patients aged <60 years versus patients aged ≥60 years at the time of diagnosis. DESIGN: Retrospective chart review of all 112 patients presenting for management of HZO from January 1, 2008 to December 31, 2008. PARTICIPANTS: A total of 112 patients (58 aged <60 years and 54 aged >60 years) at the time of HZO onset. INTERVENTIONS: Anterior segment complications, treatments, and surgical procedures were documented at 3 months, 6 months, and 1 year, and then annually for the remainder of the follow-up period. MAIN OUTCOME MEASURES: Intraocular pressure, inflammation, steroid use, surgical procedures, anterior segment complications, post-herpetic neuralgia, and delayed herpes zoster pseudodendrites. RESULTS: Equal numbers of patients were affected with HZO in the younger and older age groups (51.8%, n = 58 vs. 48.2%, n = 54, respectively, P = 0.69). The most common decade of HZO onset was between 50 and 59 years. Younger patients were more likely to be healthy compared with older patients (P = 0.05). Delayed herpes zoster pseudodendrites were more common in the younger patients (36.7% vs. 16.7%, P = 0.03). The mean number of flares per patient-years was significantly higher in the younger patients (z test, P = 0.024). Post-herpetic neuralgia, neurotrophic keratopathy, and secondary infectious keratitis were more frequent in the older patients (P = 0.05). Prevalence of corneal perforation, corneal thinning, cataract formation, and glaucoma was similar between the 2 groups. Most patients in both groups (84.2% of younger patients and 89.5% of older patients) were taking topical steroids 3 years after referral for HZO. CONCLUSIONS: Herpes zoster ophthalmicus affects individuals aged younger than and older than 60 years in similar numbers, with the most common decade of onset between age 50 and 59 years. Younger patients had more episodes of delayed pseudodendritiform keratitis and flares of inflammation compared with older patients, who had more problems related to neurotrophic keratopathy. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Clinical outcomes and prognostic factors associated with acanthamoeba keratitis.

PURPOSE: To describe the clinical characteristics, time of presentation, risk factors, treatment, outcomes, and prognostic factors on a recent series of Acanthamoeba keratitis (AK) treated at our institution. METHODS: Retrospective case series of 59 patients diagnosed with AK from January 1, 2004 to December 31, 2008. Of these 59 patients, 51 had complete follow-up data and were analyzed using univariate and multivariate logistic regression analyses performed with "failure" defined as requiring a penetrating keratoplasty (PKP) and/or having (1) best-corrected visual acuity (BCVA) < 20/100 or (2) BCVA < 20/25 at the last follow-up. A single multivariate model incorporating age, sex, steroid use before diagnosis, time to diagnosis, initial visual acuity (VA), stromal involvement, and diagnostic method was performed. RESULTS: Symptom onset was greatest in the summer and lowest in the winter. With failure defined as requiring PKP and/or final BCVA < 20/100, univariate analysis suggests that age > 50 years, female sex, initial VA < 20/50, stromal involvement, and patients with a confirmed tissue diagnosis had a significant risk for failure; however, none of these variables were significant using multivariate analysis. Univariate analysis, with failure defined as requiring PKP and/or final BCVA < 20/25, showed stromal involvement and initial VA < 20/50 were significant for failure-only initial VA < 20/50 was significant using multivariate analysis. CONCLUSIONS: Symptom onset for AK is greatest in the summer. Patients with confirmed tissue diagnosis and female patients may have a higher risk for failure, but a larger prospective population-based study is required to confirm this. Failure is likely associated with patients who present with stromal involvement and patients presenting with an initial BCVA worse than 20/50.

Update on fungal keratitis from 1999 to 2008.

PURPOSE: To report trends in fungal keratitis from a single institution between 1999 and 2008. METHODS: Retrospective chart review of the patients presenting to the cornea service with fungal keratitis from April 1999 to December 2008. RESULTS: Seventy-eight eyes of 76 patients were identified. The most common predisposing factors included contact lens use (35.9%), trauma (21.8%), and history of penetrating keratoplasty (15.4%). There was a significant increase in the rate of contact lens-related Fusarium infections over time, which peaked in 2005 and 2006 (P = 0.021). Almost 40% of fungal keratitis cases [11 of 28 eyes (39.3%)] were soft contact lens-related Fusarium infections in 2005 and 2006, and this decreased to less than 10% [2 of 25 eyes (8%)] in 2007 and 2008. The odds of having a contact lens-related Fusarium infection in 2005-2006 compared with 2007-2008 was 4.40 (95% confidence interval of 0.60-32.50) (P = 0.178). Despite the decrease in contact lens-related Fusarium infections, the number of fungal infections remained elevated in 2007 (10 eyes) and 2008 (14 eyes), including contact lens-related infections (3 in 2007 and 6 in 2008). CONCLUSIONS: A definite increase in the number of fungal keratitis cases began in 2004 and continued through 2006 during the Fusarium outbreak associated with ReNu with MoistureLoc. Despite the decrease in contact lens-related Fusarium infections, the overall number of fungal keratitis cases remained high through 2008. Fungal keratitis was more often associated with contact lens use than with trauma in this time.

Recurrent corneal erosions and epithelial basement membrane dystrophy.

Epithelial basement membrane dystrophy, the most common hereditary anterior corneal dystrophy and considered a "category 1" dystrophy in some cases, encompasses microcystic dystrophy and other conditions affecting the epithelial basement membrane. The management of symptomatic epithelial basement membrane dystrophy includes alleviating blurred vision, treating recurrent corneal erosion, or both. Treatment of distorted vision may be as simple as prescribing lubricating drops and/or ointment, and posttrauma corneal erosion is often a limited problem that disappears over time and does not require laser or surgical treatment. This article describes treatment for more severe cases of corneal erosion, which includes mechanical debridement of the loosened epithelium.

Third or greater penetrating keratoplasties: indications, survival, and visual outcomes.

PURPOSE: The purposes of this study were to report the indications, graft survival, risk factors for graft failure, and visual outcomes for third or greater penetrating keratoplasties (PKP). METHODS: Six years of Wills Eye Institute Cornea Service charts (2000-2005) were retrospectively reviewed to identify all patients who had undergone three or more PKPs. Graft survival rates by initial diagnosis and risk factors for graft failure were analyzed. RESULTS: Forty-five patients who had three or more PKPs were identified. The total number of grafts identified in these 45 patients was 152, including 45 third PKPs, 11 fourth, three fifth, two sixth, and one seventh. The most common indication for the initial PKP was pseudophakic bullous keratopathy in 18 of 45 patients (41%) followed by Fuchs dystrophy (seven of 45 patients [16%]) and stromal dystrophies (five of 45 patients [11%]). Approximately half of third grafts (24 of 45 [53%]) and one fourth of fourth grafts (three of 11 [27%]) survived at the last follow-up visit with a median follow up of 4.3 years for the third grafts and 8.4 years for the fourth grafts. One-, 2-, and 5-year graft survival rates were 89%, 78%, and 53% for the third grafts and 73%, 73%, and 64% for the fourth grafts, respectively. The median survival time for the third graft was 12.8 years in Fuchs dystrophy, 5.2 years in herpetic keratitis, 4.0 years in keratoconus, 3.0 years in pseudophakic bullous keratopathy, 2.3 years in iridocorneal endothelial syndrome, and 2.0 years in stromal dystrophies. There is no statistically significant difference between groups (P = 0.46). Risk factor analysis on the third grafts showed that previous glaucoma procedures and corneal neovascularization are statistically significant risk factors for graft failure (P = 0.04 and 0.02, respectively). CONCLUSION: Over 50% of third and fourth grafts were clear at 5 years postoperatively. Outcomes of third grafts were better in patients with Fuchs dystrophy, keratoconus, and herpetic keratitis. Absence of previous glaucoma surgery and/or corneal neovascularization is associated with better outcomes of multiple PKPs.

Quality of life in keratoconus patients after penetrating keratoplasty.

PURPOSE: To determine vision-related quality of life (QoL) measured with the National Eye Institute Visual Function Questionnaire (NEI-VFQ) in keratoconus (KCN) patients who have undergone penetrating keratoplasty (PK) in 1 or both eyes and to compare the results of our study to those of historical controls. DESIGN: Clinical-based, cross-sectional study. SETTING: Wills Eye Institute, Cornea Service, Thomas Jefferson University, Philadelphia, Pennsylvania. STUDY POPULATION: This study included 149 consecutive patients who had undergone PK for KCN. INTERVENTION: Between June 1, 2008 and December 31, 2008, the NEI-VFQ was administered to 149 patients. The relationship between demographic and clinical factors and NEI-VFQ subscale scores was evaluated. MAIN OUTCOME MEASURE: Vision-related quality of life. RESULTS: Eighty-three of 149 patients (55.7%) were male. Approximately half of the patients (76/149; 51.0%) had PK in both eyes. Visual acuity with current correction in the better eye was better than 20/40 in 80% of patients (119/149). Our sample had significantly lower (worse) NEI-VFQ scores compared to Collaborative Longitudinal Evaluation of Keratoconus (CLEK) historical control group for the subscales of role difficulties, dependency, driving, and peripheral vision. In general, scores of our sample were between scores of patients with age-related macular degeneration (AMD) category 3 and 4. Patients with visual acuity better than 20/40 (in the better eye) showed significantly higher scores in all subscales except color vision. There was a significant relationship between minimum time since the graft of 5 years or greater and NEI-VFQ overall score better than AMD category 3 (P = .004). CONCLUSION: Despite satisfactory results on visual outcome measures obtained after PK, vision-related QoL in KCN patients remains impaired.

Demographics of patients older than 50 years with keratoconus.

PURPOSE: This study was designed to determine the demographics of patients aged 50 years or more with a diagnosis of keratoconus, who were seen on the Cornea Service at Wills Eye Hospital. METHODS: A retrospective chart review of all patients with the diagnosis of keratoconus was undertaken to identify demographics characteristics between January 1, 2005 and December 31, 2005. RESULTS: A total of 697 patients were identified. Two hundred seventy-nine (40.0%) patients were age 50 years or more. The age ranged from 50 to 93 years (average 60.2 +/- 8.2 years). Keratoconus was diagnosed as a bilateral disease in 266 (95.3%) and as a unilateral disease in 13 patients (4.7%). In 186 patients (66.6% of the total), the average age at the time of diagnosis was 31.7 +/- 10.9 years (range from 13 to 70 years). Of the 279 patients, 167 had surgery (59.8%). In the group of 112 patients that did not have surgery, 25 (9.0%) were treated with glasses in both eyes, 85 (30.5%) with contact lenses in both eyes, and 2 (0.7%) with glasses in one eye and contact lenses in the other eye. The average time of follow-up was 13.6 +/- 10.5 years (range from 0.1 to 38.7 years). CONCLUSIONS: In a corneal referral practice, the number of patients older than 50 years with keratoconus is higher than previously reported in the literature. We believe that this is because our practice has a strong interest in both contact lenses and corneal surgery with the ability to follow up patients on a long-term basis.

Boston keratoprosthesis outcomes and complications.

PURPOSE: To evaluate the outcomes of the Boston Keratoprosthesis (KPro) at our institution. METHODS: A computerized database retrospectively identified all patients who received a Boston KPro from August 2005 to October 2007. RESULTS: There were 37 patients, 24 (65%) were male. Mean age was 66.3 years (median 69 years, range: 24-93 years; SD = 18.1 years). Mean follow-up was 16 months (range: 6-28 months; SD = 6.0 months). There were 36 type 1 (97%) and 1 type 2 (3%) KPros. The primary indication was failed penetrating keratoplasty in 29 patients (78%)-22 (59%) from multiple failures. Preoperative comorbidities included glaucoma [27 patients (73%)] and limbal stem cell deficiency [13 patients (35%)]. No intraoperative complications occurred. Postoperative complications included retroprosthetic membrane [24 patients (65%)], increased intraocular pressure [14 patients (38%)], glaucoma progression [5 patients (13.5%)], and endophthalmitis [4 patients (11%)-3 who discontinued prophylactic antibiotics secondary to compliance]. Thirty-six KPros (97%) were retained-1 type 2 KPro (3%) in a patient with ocular cicatricial pemphigoid extruded and was replaced. Mean best-corrected visual acuities were counting fingers preoperatively (range: light perception to 20/100) and 20/90 at last follow-up (range: light perception to 20/25). At last follow-up, 31 patients (84%) improved 2 lines or greater-3 patients (8%) had worse vision. CONCLUSIONS: Retention rates were excellent, and best-corrected visual acuities improved in the vast majority of patients. Complications can occur and require frequent follow-up to monitor and treat glaucoma progression, endophthalmitis, and inflammation. Patient compliance is of paramount importance. Despite the potential complications, the Boston KPro provides visual improvement in patients with an otherwise poor prognosis.

Comparative study of graft rejection in keratoconus patients with and without self-reported atopy.

PURPOSE: To compare the incidence and characteristics of graft rejection in keratoconus patients with and without self-reported atopy. METHODS: From November 2007 to February 2008, 75 consecutive keratoconus patients who had undergone penetrating keratoplasty completed a questionnaire regarding personal history of atopy. The questionnaires were reviewed without knowledge of history of graft rejection. The patients were divided into three groups: nonatopic (group 1), mild atopic (group 2), and severe atopic (group 3). Charts were retrospectively reviewed with regard to the incidence and characteristics of graft rejection episodes for each patient. RESULTS: There were 13 patients in group 1, 36 patients in group 2, and 26 patients in group 3. The incidence of graft rejection (rejection episodes per 100 person-years) in groups 1, 2, and 3 was 7.3, 15.8, and 13.4, respectively. Although the mild and severe atopic groups had roughly twice the rate of rejection of the nonatopic group, there was no statistically significant difference among the groups (P = 0.25). The mean age of 37.1 +/- 8.9 years at the time of surgery was significantly lower (P = 0.004) and the rate of bilateral penetrating keratoplasty was significantly higher (40/55 eyes 73%) (P = 0.01) in group 2 than in the two other groups. The majority of first graft rejection episodes were mild or moderate in atopic patients (69% in group 2 and 94% in group 3) and severe (50%) in nonatopic patients; however, no significant difference was found among the groups (P = 0.13). CONCLUSIONS: Atopic conditions may increase the incidence of graft rejection in keratoconus patients, and the characteristics of graft rejection in atopic patients may differ from those in nonatopics because of the immune alteration, but differences in this study were not statistically significant.

Steroid-induced intraocular pressure elevation or glaucoma after penetrating keratoplasty in patients with keratoconus or Fuchs dystrophy.

PURPOSE: The aim of the present study was to evaluate the frequency of steroid-induced intraocular pressure (IOP) elevation and/or glaucoma in patients with keratoconus (KCN) compared with patients with Fuchs endothelial dystrophy after penetrating keratoplasty (PK). METHODS: A retrospective review of the medical records of patients with KCN or Fuchs dystrophy, who underwent PK and were examined on the Cornea Service, Wills Eye Institute, was performed. IOP measurements were recorded preoperatively; postoperative first month and maximal IOP measurements between 1 and 3 months, 4 and 6 months, 7 and 12 months, 1 and 2 years, 2 and 3 years, and 3 and 4 years were noted. Steroid-induced IOP elevation and/or glaucoma were grouped into 5 different categories; an increase in IOP of at least 5 or 10 mm Hg over the preoperative baseline and also IOP > or =22, 30, and 40 mm Hg. Addition of glaucoma medications and/or characteristic glaucomatous optic disc and visual field changes were also assessed. RESULTS: A total of 100 patients with KCN and 58 patients with Fuchs dystrophy were included in this study. The overall frequency of steroid-induced IOP elevation after PK was 73% in the KCN group and 60.3% in the Fuchs dystrophy group. The frequency of IOP elevation of at least 5 or 10 mm Hg over the preoperative baseline were 72% and 24% in KCN group and 56.9% and 20.7% in the Fuchs dystrophy group, respectively. The frequency of IOP elevation > or =22 or > or =30 mm Hg was 22% and 6% in the KCN group and 29.3% and 1.7% in the Fuchs dystrophy group, respectively. There was one patient in the KCN group who had IOP >40 mm Hg. There was no difference between the groups in terms of frequency of IOP elevation (P > 0.05 for all). Glaucomatous visual field defect was detected in 4 patients in the KCN group and only one patient in the Fuchs dystrophy group. Despite the maximum medical therapy, 2 patients in the KCN group underwent glaucoma surgery and none in the Fuchs dystrophy group. CONCLUSION: Steroid-induced IOP elevation or glaucoma after PK is not unusual in eyes with KCN or Fuchs dystrophy. Careful and ongoing observation of IOP throughout the prolonged follow-up period is recommended for these individuals with prompt attention to IOP treatment as indicated.

Slitlamp biomicroscopy and photographic image analysis of herpes simplex virus stromal keratitis.

OBJECTIVE: To validate photographic bioimaging for evaluating the severity of herpes simplex virus keratitis. METHODS: Stromal keratitis of patients in the Herpetic Eye Disease Study was clinically measured with a slitbeam micrometer and then photographed at trial entry. Calibrated images of 169 eyes were analyzed for the size, location, and density of stromal keratitis and endotheliitis, with shape factor as a function of area and perimeter. Validity was assessed by comparing clinical and computerized measurements and by correlating the keratitis area with visual acuity. Logistic regression explored characteristics associated with larger or denser corneal inflammation. RESULTS: Stromal keratitis had a median area of 22.4 mm(2) (interquartile range, 12.8-31.6 mm(2)) with a median shape factor of 0.69 (interquartile range, 0.56-0.79); 126 eyes (75%) had their midpoint within 2 mm of the cornea's geometric center. Photoanalytical area estimates of herpetic stromal keratitis correlated closely with clinical measurements (correlation coefficient, 0.83). Eyes with larger stromal keratitis had worse vision (correlation coefficient, 0.32) and were more likely to have iritis (P = .01). Necrotizing stromal keratitis was significantly whiter (P = .02). CONCLUSIONS: Image analysis validly assesses the disciform geometry of herpetic stromal keratitis and confirms that increased severity is associated with uveitis and reduced vision.

Keratoconus associated with other corneal dystrophies.

OBJECTIVE: To report the concomitant presentation of keratoconus and corneal dystrophies at Wills Eye Hospital for the 10-year period from January 1, 1997, to December 31, 2006. METHODS: Patients with concomitant keratoconus and corneal dystrophies were identified using a computer database. Complete ophthalmologic examination, keratometry, pachymetry, and computerized videokeratography were performed in all patients. When present, cornea guttata were confirmed by clinical examination and specular microscopy. Histopathologic examination with special stains of excised corneal buttons was performed. RESULTS: Fifty-one patients manifested typical signs and topographic evidence of keratoconus associated with another corneal dystrophy. Fuchs dystrophy was the most common association accounting for 27 cases (52.9%), followed by anterior basement membrane dystrophy with 13 cases (25.5%) and posterior polymorphous dystrophy with 7 cases (13.8%). A bilateral combination of Fuchs dystrophy and anterior basement membrane dystrophy with keratoconus was seen in 3 cases (5.8%). Finally, there was 1 bilateral case (2%) of granular dystrophy. Histopathologic studies in cases that underwent penetrating keratoplasty confirmed the clinical diagnoses. CONCLUSION: To our knowledge, this is the largest report of such a concurrence in the English literature and could lead to further studies on the possible pathophysiologic or genetic links between these entities, although a chance association cannot be excluded.

Indications for penetrating keratoplasty and associated procedures, 2001-2005.

PURPOSE: To identify current indications and trends in indications for penetrating keratoplasty (PKP) and associated procedures. METHODS: Retrospective chart review of all patients who underwent PKP at Wills Eye Institute from January 1, 2001, to December 31, 2005. RESULTS: 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. CONCLUSIONS: 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.

Graft rejection risk and incidence after bilateral penetrating keratoplasty.

PURPOSE: To assess the risk and incidence of rejection in patients who underwent bilateral penetrating keratoplasty (PK) and had at least one rejection episode. METHODS: The records of patients undergoing PK between January 1994 and December 2003 were retrospectively reviewed. Inclusion criteria were bilateral PKs and at least one rejection within 18 months of either PK. Exclusion criteria were PK performed elsewhere, multiple grafts in the same eye, and fewer than 18 months between PKs in the two eyes. RESULTS: Eighty-three patients had bilateral PKs (56 women and 27 men) at least 18 months apart and had a rejection episode within 18 months of at least one surgery. Their mean age was 57.1 +/- 17.7 years. The mean follow-up after the first and second PKs were 9.6 +/- 5.4 and 4.1 +/- 3.7 years, respectively. Kaplan-Meier survival analysis showed that PK in the second eye did not impose an increased risk of rejection in the first eye. There was no significant difference in the frequency of rejections between the two eyes during the first 18 months after the second PK. The first eye was significantly more likely to have a rejection during the first 18 months after the first PK than during the first 18 months after the second PK (P=0.01). CONCLUSIONS: Having a PK in the second eye does not impose a significantly increased risk of rejection for the first eye when PKs are performed at least 18 months apart. There is an indefinite risk for rejection. Lifelong regular follow-up and immediate evaluation for new symptoms are essential.

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

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