Robert L. Schultze

Long-term outcomes of Boston type 1 keratoprosthesis implantation: A retrospective multicenter cohort

PURPOSE To study the long-term outcomes of Boston type 1 keratoprosthesis (KPro) surgery. DESIGN Retrospective, multicenter case series. PARTICIPANTS A total of 158 eyes of 150 patients underwent KPro implantation at 5 participating tertiary centers in the United States between January 2003 and December 2006. Of those, 139 eyes of 133 patients were included in the analyses. METHODS The medical records of consecutive adult patients who received KPro surgery were reviewed. All patients with at least 1 postoperative visit were retained in the outcomes analyses. In eyes in which a repeat KPro procedure was performed, only the outcomes of the initial surgery were analyzed. MAIN OUTCOME MEASURES Visual acuity (VA) outcomes, postoperative complications, and device retention. RESULTS The mean follow-up was 46.7 ± 26 months with all but 4 eyes having at least 6 months of follow-up. Preoperatively, only 10.8% of the eyes had VA of ≥ 20/200. Postoperatively, the VA in 70% of eyes improved to ≥ 20/200. The probability of maintaining VA of ≥ 20/200 at 7 years was 50%. The device retention rate was estimated at 67% at 7 years. The 7-year cumulative incidence of complications was 49.7% for retroprosthetic membrane formation, 21.6% for glaucoma surgery, 18.6% for retinal detachment, and 15.5% for endophthalmitis. CONCLUSIONS Although the risk for complications with longer follow-up seemed to increase, this large multicenter cohort demonstrates favorable outcomes with KPro, with a large number of patients achieving and retaining useful vision over a 7-year period.

Elevated intraocular pressure-induced interlamellar stromal keratitis

PURPOSE To describe a series of cases (Elevated Intraocular Pressure Induced Interlamellar Stromal Keratitis (PISK)) that appears to be identical to post-laser in situ keratomileusis (LASIK) diffuse lamellar keratitis (DLK), but was present at a later time frame and was associated with a significant elevation of intraocular pressure (IOP). Unlike DLK, this syndrome is not steroid responsive, but resolves with a lowering of the IOP. DESIGN Retrospective, noncomparative, small case series. PARTICIPANTS The medical records of four LASIK patients with IOP-induced interface changes for the 1-year period March 2000 to March 2001 were reviewed retrospectively. MAIN OUTCOME MEASURE Slit-lamp appearance. RESULTS In the four cases presented, the slit-lamp findings and visual degradation appeared identical to DLK. All cases, however, presented outside of the first postoperative week and were not associated with any antecedent trauma. All four cases failed to respond to high-dose topical steroids. Significant IOP elevations were noted in all cases, and the interface changes responded dramatically to both a lowering of the IOP and a discontinuation or lowering of the topical steroids. CONCLUSIONS Elevated IOP-induced post-LASIK interface keratitis (PISK) is a poorly documented phenomena. Because the condition may be caused by or worsened by frequent topical steroids, early recognition is important. Treatment consists primarily of normalizing the IOP.

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.

Impact of donor characteristics on 2-year Descemet stripping automated endothelial keratoplasty outcomes in patients with Fuchs endothelial dystrophy.

Purpose: The aim of this study was to determine the relationship between donor tissue characteristics and surgical outcomes in patients with Fuchs endothelial dystrophy (FED) 2 years after undergoing Descemet stripping automated endothelial keratoplasty (DSAEK). Methods: A retrospective chart review was performed identifying 70 eyes with FED that underwent DSAEK. Donor endothelial cell density (ECD), donor death to preservation time, donor storage time, donor age, and postoperative recipient ECD were evaluated. Statistical analysis was performed using univariate linear regression analysis and bidirectional elimination multiple stepwise linear regression. Results: Cornea donor age was a predictor of postoperative ECD at postoperative years 1 and 2 (P = 0.02, adjusted R2 = 0.07 and P = 0.002, adjusted R2 = 0.13, respectively). Donor age was also a predictor of postoperative ECD at years 1 and 2 after taking into account donor ECD, death to preservation time, and donor storage time (P = 0.02, adjusted R2 = 0.62 and P = 0.002, adjusted R2 = 0.12, respectively). Donor death to preservation time was a predictor of postoperative ECD at only postoperative year 2 (P = 0.03, adjusted R2 = 0.05). Donor storage time and donor ECD did not significantly impact postoperative ECD. Conclusions: Although donor age seems to have a statistically significant impact on postoperative ECD in patients with FED undergoing DSAEK, the relationship is weak. Other donor parameters did not have a consistent or significant impact on postoperative ECD.

Nd:YAG photodestruction of a presumed corneal Toxocara canis larva.

Purpose: To describe the technique of Nd:YAG photodestruction of a presumed Toxocara canis corneal larval migrans and to report the unique clinical combination of a corneal nematode and diffuse unilateral subacute neuroretinitis (DUSN). Methods: Clinical case report. Results: A 63-year-old white male presented with idiopathic neuroretinitis treated empirically with systemic anti inflammatory therapy. Subsequently, he developed keratouveitis with an obvious corneal larva. Serology was positive for T. canis. Laser destruction of the larva combined with topical steroid and oral albendazole therapy led to initial resolution, followed by 1 episode of recurrent keratouveitis without identified ocular surface or intraocular larvae. This was cleared with topical steroids. A second empiric course of oral albendazole was also administered. The patient has shown no ocular recurrences or evidence of systemic involvement 1 year after the second course of treatment. Conclusions: Corneal larvae can be safely and successfully destroyed by Nd:YAG photodestruction. T. canis may be one of several nematodes responsible for DUSN. A careful examination of the anterior segment is essential in the management of patients with DUSN.

Donor, Recipient, and Operative Factors Associated With Increased Endothelial Cell Loss in the Cornea Preservation Time Study

Importance Determining factors associated with endothelial cell loss after Descemet stripping automated endothelial keratoplasty (DSAEK) could improve long-term graft survival. Objective To evaluate the associations of donor, recipient, and operative factors with endothelial cell density (ECD) 3 years after DSAEK in the Cornea Preservation Time Study. Design, Setting, and Participants This cohort study was a secondary analysis of data collected in a multicenter, double-masked, randomized clinical trial. Forty US clinical sites with 70 surgeons participated, with donor corneas provided by 23 US eye banks. Individuals undergoing DSAEK for Fuchs dystrophy or pseudophakic/aphakic corneal edema were included. Interventions The DSAEK procedure, with random assignment of a donor cornea with a preservation time of 0 to 7 days or 8 to 14 days. Main Outcomes and Measures Endothelial cell density at 3 years as determined by a reading center from eye bank and clinical specular or confocal central endothelial images. Results The study included 1090 participants (median age, 70 years) with 1330 affected eyes (240 bilateral cases [22.0%]), who underwent DSAEK for Fuchs dystrophy (1255 eyes [94.4%]) or pseudophakic/aphakic corneal edema (PACE) (75 eyes [5.6%]). Of these, 801 eyes (60.2%) belonged to women and 1207 (90.8%) to white individuals. A total of 749 participants (913 eyes; 164 [21.9%] bilateral cases) had functioning grafts with acceptable endothelial images preoperatively and at 3 years postoperatively and were included in this analysis. Factors associated with a lower ECD at 3 years (estimated effect with 99% CI) in the final multivariable model included donors with diabetes (−103 [−196 to −9] cells/mm2), lower screening ECD (−234 [−331 to −137] per 500 cells/mm2), recipient diagnosis of PACE (−257 [−483 to −31] in cells/mm2), and operative complications (−324 [−516 to −133] in cells/mm2). Endothelial cell loss (ECL) from a preoperative measurement to a 3-year postoperative measurement was 47% (99% CI, 42%-52%) for participants receiving tissue from donors with diabetes vs 43% (99% CI, 39%-48%) without diabetes; it was 53% (99% CI, 44%-62%) for participants diagnosed with PACE vs 44% (99% CI, 39%-49%) for those diagnosed with Fuchs dystrophy, and 55% (99% CI, 48%-63%) in participants who experienced operative complications vs 44% (99% CI, 39%-48%) in those who did not. No other donor, recipient, or operative factors were significantly associated with 3-year ECD. Conclusions and Relevance Donor diabetes, lower screening ECD, a PACE diagnosis in the recipient, and operative complications were associated with lower ECD at 3 years after DSAEK surgery and may be associated with long-term graft success. While causation cannot be inferred, further studies on the association of donor diabetes and PACE in recipients with lower 3-year ECD warrant further study.

Evaluating the effects of Botulinum Toxin A on tear metrics in patients with hemifacial spasm

ABSTRACT Hemifacial spasm has been previously shown to result in dry eye and ocular surface diseases. This study was performed to assess the impact of chemodenervation with botulinum toxin on clinically-relevant metrics of tears. Tear osmolarity and lipid layer thickness were measured in patients with hemifacial spasm after they achieved relief from hemifacial spasm via botulinum toxin injections. Twelve eyes of 6 patients with hemifacial spasm (2 men and 4 women; mean age 55.5 years) were assessed in accordance with the Declaration of Helsinki and IRB approval. The mean tear osmolarities were 300.8 mOsm (standard deviation = 7.44 mOsm) and 293.0 mOsm (standard deviation = 7.01 mOsm) for treated and untreated eyes, respectively, and this difference was not statistically significant (p = 0.097). The mean lipid layer thicknesses of the tear film were 78.7 nm (standard deviation = 18.0 nm) and 71.5 nm (standard deviation = 25.2 nm) for treated and untreated eyes, respectively, and these values were not statistically significantly different (p = 0.671). Although previous investigations have demonstrated significant dry eye disease in patients with hemifacial spasm, successful chemodenervation with botulinum toxin appears to result in tear osmolarities and lipid layer thicknesses that were comparable in treated and untreated eyes. This finding may represent an additional benefit to treatment of hemifacial spasm.