Topical bevacizumab treatment in aniridia.

PURPOSE: To report the results of long-term topical treatment with bevacizumab (Avastin) 5 mg/mL eyedrops in a case of aniridia-related neovacularization of the cornea. METHODS: Interventional case report. A female patient with aniridia had a decrease in the best corrected visual acuity from 0.32 to 0.02 in the OS over the course of 4 years, secondary to central corneal neovascularization and epithelial breakdown. Vision in the OD was 0.2. In 2008, at age 28, a shared decision was made to start off-label treatment with bevacizumab eyedrops 0.5% in both eyes. After 9 years, the visual acuity in the OD remained stable, with stability of the macropannus and maintenance of central corneal clarity. In the OS, the central corneal neovascularization regressed, the epithelium regained its clarity, and after cataract surgery visual acuity was regained to 0.32. RESULTS: After 9 years of treatment with topical bevacizumab, vision acuity is comparable to the situation of 12 years previously: Visual acuity remained stable in the ODS. CONCLUSION: In a young patient with progressive corneal neovascularization secondary to aniridia, stability of central corneal neovascularization was obtained and corneal clarity was preserved by adding a daily drop of bevacizumab 5 mg/mL. No adverse events occurred. Vessel growth was inhibited, and as such, the progression of the natural history of the patient's disease was halted. More clinical study with longer follow-up is needed to investigate the applicability of treatment with topical VEGF inhibitors in aniridia.

The role of a labial salivary gland biopsy in the diagnostic procedure for Sjögren's syndrome; a study of 94 cases.

OBJECTIVES: The purpose of the present study is to examine the role of the outcome of the labial salivary gland biopsy (LSGB) in the diagnostic procedure of patients suspected of suffering from Sjögren's syndrome (SS). MATERIAL AND METHODS: In a retrospective study the result of histopathological assessment of 94 consecutively taken labial salivary gland biopsies has been examined. For the diagnosis of SS the American-European Consensus Group classification (AECG, 2002) have been used. The outcome of the assessment has been discussed in relation to a recently reported classification provided by the American College of Rheumatology (ACR, 2012). RESULTS: In the 94 LSGBs support for a diagnosis of SS has been encountered in 24 out of 26 patients with SS. In the 68 patients with a negative diagnosis of SS only six positive LSGBs were observed. The sensitivity of the labial biopsy amounted 0.92; the specificity was 0.91, while the positive predictive value and the negative predictive value amounted 0.80 and 0.97 respectively. LSGBs taken by or on the request of the departments of Rheumatology or Internal Medicine had a significant higher yield compared to LSGBs taken in other clinical departments. CONCLUSIONS: The LSGB may play a role in the diagnostic procedure of Sjögren's syndrome when using either the AECG classification or the ACR classification. A LSGB should preferably taken after counseling for the possible presence of SS by a department of Rheumatology or Internal Medicine since the yield of such biopsies is much higher than in patients who have not been counseled by these departments prior to the taking of a LSGB. When using the ACR classification, a positive serologic result and a positive ocular test make the taking of a LSGB redundant. Only in case of a negative serologic outcome or a negative result of the ocular test a LSGB is indicated. Since both the serologic test and the ocular test carry hardly any morbidity, these tests should, indeed, be performed first before considering to take a LSGB.

Epidemiology and Clinical Management of Fusarium keratitis in the Netherlands, 2005-2016.

Introduction: Recognizing fungal keratitis based on the clinical presentation is challenging. Topical therapy may be initiated with antibacterial agents and corticosteroids, thus delaying the fungal diagnosis. As a consequence, the fungal infection may progress ultimately leading to more severe infection and blindness. We noticed an increase of fungal keratitis cases in the Netherlands, especially caused by Fusarium species, which prompted us to conduct a retrospective cohort study, aiming to describe the epidemiology, clinical management, and outcome. Materials and Methods: As fungi are commonly sent to the Dutch mycology reference laboratory for identification and in vitro susceptibility testing, the fungal culture collection was searched for Fusarium isolates from corneal scrapings, corneal swabs, and from contact lens (CL) fluid, between 2005 and 2016. All Fusarium isolates had been identified up to species level through sequencing of the ITS1-5.8S-ITS2 region of the rDNA and TEF1 gene. Antifungal susceptibility testing was performed according to the EUCAST microbroth dilution reference method. Antifungal agents tested included amphotericin B, voriconazole, and natamycin. In addition, susceptibility to the antisepticum chlorhexidine was tested. Ophthalmologists were approached to provide demographic and clinical data of patients identified through a positive culture. Results: Between 2005 and 2016, 89 cases of Fusarium keratitis from 16 different hospitals were identified. The number of cases of Fusarium keratitis showed a significant increase over time (R2 = 0.9199), with one case in the first 5 years (2005-2009) and multiple cases from 2010 and onwards. The male to female ratio was 1:3 (p = 0.014). Voriconazole was the most frequently used antifungal agent, but treatment strategies differed greatly between cases including five patients that were treated with chlorhexidine 0.02% monotherapy. Keratitis management was not successful in 27 (30%) patients, with 20 (22%) patients requiring corneal transplantation and seven (8%) requiring enucleation or evisceration. The mean visual acuity (VA) was moderately impaired with a logMAR of 0.8 (95% CI 0.6-1, Snellen equivalent 0.16) at the time of Fusarium culture. Final average VA was within the range of normal vision [logMAR 0.2 (95% CI 0.1-0.3), Snellen equivalent 0.63]. CL wear was reported in 92.9% of patients with Fusarium keratitis. The time between start of symptoms and diagnosis of fungal keratitis was significantly longer in patients with poor outcome as opposed to those with (partially) restored vision; 22 vs. 15 days, respectively (mean, p = 0.024). Enucleation/evisceration occurred in patients with delayed fungal diagnosis of more than 14 days after initial presentation of symptoms. The most frequently isolated species was F. oxysporum (24.7%) followed by F. solani sensu stricto (18%) and F. petroliphilum (9%). The lowest MICs were obtained with amphotericin B followed by natamycin, voriconazole, and chlorhexidine. Conclusion: Although Fusarium keratitis remains a rare complication of CL wear, we found a significant increase of cases in the Netherlands. The course of infection may be severe and fungal diagnosis was often delayed. Antifungal treatment strategies varied widely and the treatment failure rate was high, requiring transplantation or even enucleation. Our study underscores the need for systematic surveillance of fungal keratitis and a consensus management protocol.

Toric phakic intraocular lens for the correction of hyperopia and astigmatism.

PURPOSE: To evaluate the Artisan toric phakic intraocular lens (pIOL) for the correction of hyperopia and astigmatism. SETTING: Department of Ophthalmology, Erasmus MC, Rotterdam, The Netherlands, and Department of Ophthalmology, Sint Truiden, Belgium. METHODS: In this prospective study of 47 eyes of 28 patients with hyperopia and astigmatism, Artisan toric pIOLs were implanted between April 1999 and June 2004. Uncorrected visual acuity (UCVA), best corrected visual acuity, refraction, astigmatism, safety, and predictability were analyzed. Change in astigmatism was analyzed with vector analysis. Refractive cylinders are expressed in minus form. RESULTS: Mean preoperative spherical equivalent was +4.33 diopters (D) +/- 2.26 (SD). Mean follow-up was 11.1 months (range 6 to 36 months). A gain of 1 or more lines in best spectacle-corrected visual acuity (BSCVA) was seen in 36.2%. Safety index and efficacy index after 6 months were 1.06 and 0.87, respectively. The mean postoperative astigmatism at 6 months was 0.19 D at an axis of 144 degrees. At 6 months, about three quarters (76.6%) of the eyes had a UCVA of 20/40 or better. One eye lost 2 lines of BSCVA. In 1 eye, the lens position had to be changed because of a large axis misalignment. No serious complications developed in any of the treated eyes during follow-up. CONCLUSIONS: Artisan toric pIOLs can correct moderate to high hyperopia combined with astigmatism with good refractive results. In this study, there were no serious complications. However, the predictability of the refractive results appeared to be lower than those in the correction of myopia and astigmatism with toric Artisan lenses.