Brandon D. Ayres

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.

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.

Referrals to the Wills Eye Institute Cornea Service after laser in situ keratomileusis: Reasons for patient dissatisfaction

PURPOSE: To review the symptoms, findings, and management options in patients referred to the Cornea Service who were unsatisfied with results after laser in situ keratomileusis (LASIK). SETTING: Cornea Service, Wills Eye Institute, Philadelphia, Pennsylvania, USA. METHODS: A retrospective chart review was conducted of all patients seen for consultation between January 1, 2004, and December 31, 2006, who had LASIK performed elsewhere. The parameters extracted were demographic data, history, symptoms, postoperative best corrected and uncorrected visual acuities, surgical complications, examination findings, and treatment recommendations. The data were also compared with previously unpublished data collected at Wills Eye from 1998 to 2003. RESULTS: One hundred fifty‐seven eyes of 109 patients seen in consultation after LASIK were identified. Twenty‐eight percent were referred by the LASIK surgeon and 54%, by another eye doctor; 17% were self‐referred. The most common chief complaints were poor distance vision (63%), dry eyes (19%), redness/pain (7%), and glare and halos (5%). Forty‐four eyes (28%) had surgical complications or enhancements. The most common diagnoses were dry eye or blepharitis (27.8%), irregular astigmatism (12.1%), and epithelial ingrowth (9.1%). Eleven percent were referred in the first month after LASIK; 23% and 10% were referred between 1 and 6 months and 7 and 12 months, respectively. Medical management (eg, artificial tears, steroids, other dry‐eye treatment) was offered in 39% of cases, surgical intervention in 27%, and observation only in 7%. Nonsurgical therapy was offered in 73% of cases. CONCLUSIONS: Most patients who came for consultation were referred by a doctor other than their LASIK surgeon. Poor distance vision, dry eye, redness/pain, and glare and halos were the most common chief complaints and dry eye or blepharitis, irregular astigmatism, and epithelial ingrowth, the most common diagnoses.

A multicenter study to map genes for Fuchs endothelial corneal dystrophy: Baseline characteristics and heritability

Purpose: To describe the methods for family and case–control recruitment for a multicenter genetic and associated heritability analyses of Fuchs endothelial corneal dystrophy (FECD). Methods: Twenty-nine enrolling sites with 62 trained investigators and coordinators gathered individual and family information, graded the phenotype, and collected blood and/or saliva for genetic analysis on all individuals with and without FECD. The degree of FECD was assessed in a 0 to 6 semiquantitative scale using standardized clinical methods with pathological verification of FECD on at least 1 member of each family. Central corneal thickness was measured by ultrasonic pachymetry. Results: Three hundred twenty-two families with 330 affected sibling pairs with FECD were enrolled and included a total of 650 sibling pairs of all disease grades. Using the entire 7-step FECD grading scale or a dichotomous definition of severe disease, heritability was assessed in families via sib–sib correlations. Both binary indicators of severe disease and semiquantitative measures of disease severity were significantly heritable, with heritability estimates of 30% for severe disease, 37% to 39% for FECD score, and 47% for central corneal thickness. Conclusions: Genetic risk factors have a strong role in the severity of the FECD phenotype and corneal thickness. Genotyping this cohort with high-density genetic markers followed by appropriate statistical analyses should lead to novel loci for disease susceptibility.

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.

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.

Excimer laser phototherapeutic keratectomy.

The argon-fluoride 193 nm excimer laser is currently under investigation in the United States for both refractive and therapeutic indications. We report the preliminary results of phototherapeutic keratectomy in 15 patients. Patients were divided as follows: group 1 (11 patients with anterior corneal pathology) and group 2 (four patients with refractive error after ocular surgery). Mean follow-up was 4.7 and 4.0 months in the two groups, respectively. Although results in group 1 are difficult to quantify because of varied indications, visual acuity improved in six of 11 patients (55%) and remained the same in five patients (45%). In group 2 uncorrected visual acuity improved in two patients (50%) and remained the same in two patients. Best corrected vision was the same or better in three patients but decreased four lines in one patient at 1-month follow-up, possibly related to retinal pathology. Overall, the results of excimer laser phototherapeutic keratectomy are quite promising, especially in light of alternative treatments that are more invasive.

Corneal ulcers associated with aerosolized crack cocaine use.

Purpose: We report 4 cases of corneal ulcers associated with drug abuse. The pathogenesis of these ulcers and management of these patients are also reviewed. Methods: Review of all cases of corneal ulcers associated with drug abuse seen at our institution from July 2006 to December 2006. Results: Four patients with corneal ulcers associated with crack cocaine use were reviewed. All corneal ulcers were cultured, and the patients were admitted to the hospital for intensive topical antibiotic treatment. Each patient received comprehensive health care, including medical and substance abuse consultations. Streptococcal organisms were found in 3 cases and Capnocytophaga and Brevibacterium casei in 1 patient. The infections responded to antibiotic treatment. Two patients needed a lateral tarsorrhaphy for persistent epithelial defects. Conclusions: Aerosolized crack cocaine use can be associated with the development of corneal ulcers. Drug abuse provides additional challenges for management. Not only treatment of their infections but also the overall poor health of the patients and increased risk of noncompliance need to be addressed. Comprehensive care may provide the patient the opportunity to discontinue their substance abuse, improve their overall health, and prevent future corneal complications.

Giant fornix syndrome: a case series.

Purpose: To describe the demographics, characteristics, and treatment of giant fornix syndrome, a rare cause of chronic purulent conjunctivitis in the elderly. Methods: Retrospective chart review of five patients with giant fornix syndrome evaluated by the Cornea Service, Oculoplastics and Orbital Surgery Service and the Department of Pathology at the Wills Eye Institute. Results: The median age of the 5 female patients was 75 years (mean 80, range 70–95). The median duration of eye symptoms before presentation was 2 years (mean 2.4, range 1–4). Before referral, the chronic conjunctivitis was treated with topical antibiotics in all 5 cases and with additional dacryocystorhinostomy in one case. The right eye was affected in 2 cases, and the left eye was affected in the other 3 cases. Floppy eyelids were present in 2 cases. The superior fornix was involved in 4 cases, and the inferior fornix was involved in one case. Pseudomembranes and superficial punctate keratitis (SPK) were seen in 3 cases. Diagnosis of giant fornix syndrome was made in all 5 cases. Conjunctival culture grew methicillin-resistant Staphylococcus aureus (MRSA), Pseudomonas aeruginosa, and S. aureus in singular cases. Case 1 was treated with topical moxifloxacin, Case 2 was treated with topical vancomycin and repair of the upper eyelid, Case 3 was treated with topical besifloxacin, and Case 4 was treated with dacryocystorhinostomy and topical vancomycin. Case 5 was treated with reconstruction of the left upper eyelid. The median duration of follow up was 4 months (mean 21.6, range 1–84). Conclusions: Giant fornix syndrome can lead to chronic relapsing conjunctivitis in the elderly. Deep conjunctival fornices in affected patients can be a site for prolonged sequestration of bacteria causing recurrent infections. Removing the infected debris from the superior fornix and reconstruction of the upper eyelid may prevent the recurrent chronic persistent infection.

Long-Term Results of the Boston Keratoprosthesis for Unilateral Corneal Disease.

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.