Thomas P. Link

Ranibizumab Combined With Low-Dose Sorafenib for Exudative Age-Related Macular Degeneration

Angiogenesis is a common factor in the pathogenesis of cancer and in exudative age-related macular degeneration (AMD). Therefore, angiogenesis inhibition has been developed as a therapeutic strategy. We report 2 cases of recurrent exudative AMD in which oral sorafenib, a tyrosine kinase inhibitor approved for cancer, was added to intravitreal ranibizumab, an antibody to vascular endothelial growth factor. These 2 patients were followed up by determination of visual acuity, fluorescein angiography, fundoscopy, and optical coherence tomography. The visual acuity of 1 patient improved from 20/70 to 20/60 while he was receiving sorafenib therapy; that of the other did not. Marked improvement was noted in both patients on optical coherence tomography. Additionally, both patients appeared to receive some benefit when low-dose oral sorafenib was used as monotherapy after its initial addition to ranibizumab therapy. Randomized trials of adding sorafenib to standard therapy for patients with neovascular AMD should be considered.

Correlation of fundus autofluorescence with fluorescein and indocyanine green angiography in choroidal melanocytic lesions.

Purpose: To correlate fundus autofluorescence (FAF) patterns with fluorescein/indocyanine green angiographic (FA/ICGA) features in choroidal melanocytic lesions. Methods: Retrospective chart review of 30 consecutive patients with choroidal nevi and melanoma who underwent FAF photography and FA/ICGA. The FAF pattern was classified as patchy or diffuse. The FA images were evaluated at the arterial, early venous, late venous, midphase, and late phases. The ICGA images were evaluated at the early and midlate phase. The fluorescence within the tumor was classified as hyperfluorescent, pinpoint hyperfluorescent, isofluorescent, or hypofluorescent with respect to the surrounding retina or choroid. Statistical analysis was performed using two sample t test for continuous data. For categorical or ordinal data, Pearson chi-square or Fisher’s exact test was used depending on the sample size being studied. Results: Nineteen of 30 tumors (63.3%) were choroidal melanoma and 11 (36.7%) were choroidal nevus. Thirteen choroidal melanomas had a diffuse FAF pattern. Six choroidal melanomas and 11 choroidal nevi had a patchy FAF pattern. The diffuse FAF pattern was significantly associated with the clinical diagnosis of choroidal melanoma versus choroidal nevus (P = 0.00001), increased tumor thickness (P = 0.00001), and increased tumor base diameter (P = 0.001), partially pigmented or amelanotic versus pigmented lesion color (P = 0.006), early venous hyperfluorescence on FA (P = 0.015), and late hyperfluorescence on FA (P = 0.018). Conclusion: Diffuse FAF is more often associated with larger choroidal melanomas as well as early venous and late hyperfluorescence on FA angiography.

Snowflake degeneration of the retina.

Ten patients in four families with white or yellow-white granular-like deposits of the peripheral retina, consistent with the description of snowflake degeneration are reported. The granular changes are observable near the equatorial fundus as numerous ophthalmoscopically discrete deposits commonly up to 100 to 200 microns in size that focally thicken the retina. They can be rather evenly distributed about the entire circumference of the eye, but sometimes they may show a predilection for the inferior quadrants. The lesions need not be associated with recognizable vitreous liquefaction, vitreoretinal traction, retinal breaks, or retinal pigmentation, and in some instances the condition appears relative stable for intervals as long as five years. The similar appearance in a mother and her 9-year-old son suggests further the relative stability of the condition. In its pure form, snowflake degeneration may be an innocent dominant familial finding but when associated with other conditions such as lattice degeneration, snowflake degeneration may be of clinical significance. Affected members may have co-existing extensive white with and without pressure changes and/or lattice degeneration.

Fundus autofluorescence in choroidal metastatic lesions: a pilot study.

Purpose: To correlate fundus autofluorescence (FAF) in choroidal metastases with clinical changes overlying the lesions using confocal scanning laser ophthalmoscope. Methods: Retrospective chart review of 15 metastatic tumors in 14 eyes of 11 consecutive patients. The patients underwent confocal scanning laser ophthalmoscope to compare the clinical findings and FAF. Correlation between increased FAF and clinical changes was defined as complete, partial, or no correlation. Results: Of 11 patients, 8 (73%) were women, and the average age was 56 years. All 15 metastases were amelanotic, focal hyperpigmentation was present in 8 (53%), subretinal fluid in eleven (73%), whereas drusen and orange pigment were absent. Of 8 tumors that were visualized by scanning laser ophthalmoscope, 7 tumors (87%) showed correlation between FAF and hyperpigmentation, no correlation in 1 (13%). A complete correlation between hyperpigmentation and FAF was found in 5 (70%) tumors and a partial correlation in 2 (30%). There was partial correlation between subretinal fluid and FAF in 6 metastases (46%), no correlation in 5 (39%). Three metastases without hyperpigmentation had FAF, one showed the presence of subretinal fluid. Conclusion: Amelanotic choroidal metastases with hyperpigmentation are associated with increased FAF in most cases. Subretinal fluid overlying choroidal metastases may cause FAF as well.

Heidelberg Spectralis infrared video imaging: a clinical tool for diagnosing ocular torsional instability

Heidelberg Spectralis infrared video imaging provides a diagnostic modality for the assessment of dynamic torsional instability of the eyes. It accurately detects monocular changes in both vertical and torsional eye position as fixation is switched from one eye to the other without the need for illuminating either eye. Heidelberg Spectralis infrared video imaging is useful for depicting the subtle torsional components of dissociated vertical divergence, latent nystagmus in patients with infantile esotropia.

Uveal Tumors: Diagnostic Techniques: Angiography

Fluorescein (FA) and indocyanine green angiography (ICGA) are usually not necessary to diagnose ocular tumors except for retinal tumors, when these ancillary tests assist in establishing the correct diagnosis. Among choroidal tumors, FA and ICGA are particularly useful in establishing the diagnosis of choroidal hemangioma and for revealing choroidal folds. In addition, over the past few decades, these diagnostic procedures have played a pivotal role in expanding our understanding of retinal and choroidal anatomy, pathology, and pathophysiology. These tools are also used to assess disease progression and treatment side effects (radiation retinopathy).

Retinal mosaicism in a girl with an X–Y translocation

To the Editor X-linked ocular albinism is a common disorder of melanosome biogenesis.1 In affected male subjects, it manifests in the form of reduced visual acuity, infantile nystagmus and ocular hypopigmentation.1 Carrier female subjects are minimally affected but may show iris translumination and coarse pattern of blotchy hypopigmentation and hyperpigmentation of the retinal pigment epithelium due to lyonisation.2 ,3 X-linked ocular albinism is caused by mutations in the G protein-coupled receptor 143 (GPR143) gene, also known as the ocular albinism 1 gene located at Xp22.32.1 About 48% of reported mutations in the GPR143 gene are intragenic …

Utility of large spot binocular indirect laser delivery for peripheral photocoagulation therapy in children.

The purpose of this article is to demonstrate the utility of the large spot size (LSS) setting using a binocular laser indirect delivery system for peripheral ablation in children. One patient with bilateral retinopathy of prematurity received photocoagulation with standard spot size burns placed adjacently to LSS burns. Using a pixel analysis program called Image J on the Retcam picture, the areas of each retinal spot size were determined in units of pixels, giving a standard spot range of 805 to 1294 pixels and LSS range of 1699 to 2311 pixels. Additionally, fluence was calculated using theoretical retinal areas produced by each spot size: the standard spot setting was 462 mJ/mm2 and the LSS setting was 104 mJ/mm2. For eyes with retinopathy of prematurity, our study shows that LSS laser indirect delivery halves the number of spots required for treatment and reduces fluence by almost one-quarter, producing more uniform spots.