Recent Progress in Retinal Pigment Epithelium Cell-Based Therapy for Retinal Disease.

Age-related macular degeneration and retinitis pigmentosa are degenerative retinal diseases that cause severe vision loss. Early clinical trials involving transplantation of retinal pigment epithelial cells and/or photoreceptors as a treatment for these conditions are underway. In this review, we summarize recent progress in the field of retinal pigment epithelium transplantation, including some pertinent clinical trial results as well as preclinical studies that address issues of transplant immunology, cell delivery, and cell manufacturing.

Recent Progress in Photoreceptor Cell-Based Therapy for Degenerative Retinal Disease.

Age-related macular degeneration and retinitis pigmentosa are degenerative retinal diseases that cause severe vision loss. Early clinical trials involving transplantation of photoreceptors as treatment for these conditions are underway. In this review, we summarize recent progress in the field of photoreceptor transplantation, including some pertinent results regarding photoreceptor manufacture, photoreceptor transplantation, mechanisms of donor-host cell integration such as material transfer and photoreceptor transplant immunology. We conclude by proposing several approaches that may provide a rational basis for selecting a vision restoration strategy (eg, donor-host synapse formation vs donor-host nanotube formation) and improved transplant efficiency.

Unilateral acute iris transillumination syndrome with glaucoma and iris pigment epithelium dispersion simulating iris melanoma.

Purpose: To report a patient with a unilateral presentation of glaucoma, pain, and acute iris transillumination syndrome simulating iris melanoma. Observations: A 53-year-old male presented with blurred vision and pain in his right eye several weeks following a respiratory sinus infection managed by oral azithromycin. Examination of the right eye was notable for elevated intraocular pressure of 46 mm Hg, an irregular mid-dilated pupil, and diffuse iris transillumination with pigmentary seeding on the iris surface, in the anterior chamber angle, and on the sclera, suspicious for diffuse iris melanoma with glaucoma and extrascleral extension. Ultrasound biomicroscopy (UBM) of the right eye revealed circumferential anterior chamber angle and trabecular meshwork involvement by an infiltrative process corresponding to the pigmented cells noted clinically, while the ciliary body was unremarkable. Following enucleation, histopathology showed extensive necrosis of the iris pigment epithelium, sphincter, and dilator muscles with melanophagic infiltration in the anterior chamber angle and episclera, mild chronic non-granulomatous iridocyclitis, and no evidence of a melanocytic neoplasm. Although immunohistochemical studies for herpes simplex virus (HSV) types 1 and 2, varicella-zoster virus, and cytomegalovirus were negative, qualitative real-time polymerase chain reaction on paraffin-embedded tissue detected HSV-1 DNA. The combined clinical, pathologic, and molecular findings were compatible with unilateral acute iris transillumination syndrome, likely HSV-1 associated. Conclusion and Importance: Unilateral acute iris transillumination syndrome with diffuse iris pigment epithelial loss can simulate iris melanoma. Prompt herpes viral studies may be informative.

Adjuvant Intravitreal Bevacizumab for Retinal Neovascularization in Eales' Disease Associated With Latent Mycobacterium Tuberculosis.

We report a case of bilateral Eales' disease managed with intravitreal bevacizumab. A 32-year-old woman with a history of bacillus Calmette-Guerin vaccine, administered when she was 10 years old, presented with a five-day history of a scotoma in the temporal field of her right eye. A dilated fundus exam and fluorescein angiography showed bilateral retinal peripheral capillary non-perfusion, retinal neovascularization in the right eye, and deep intraretinal hemorrhages in the left eye. Her laboratory workup resulted in a positive QuantiFERON-TB Gold test (Cellestis Ltd, Carnegie, Victoria, Australia). Chest computed tomography showed a calcified granuloma in her right lung. Angiographic-guided pan-retinal photocoagulation was performed, and intravitreal injections of bevacizumab (1.25 mg/0.05 mL) were administered in both eyes over the course of three months. The intraretinal hemorrhages resolved after three months of therapy. Three months following treatment, the patient showed normal fundus findings without any evidence of recurrence and a visual acuity of 20/20 in both eyes. Intravitreal bevacizumab in combination with angiography-guided pan-retinal photocoagulation may be efficacious in select patients with Eales' disease.

Intravitreal Bevacizumab for Choroidal Neovascular Membrane in Macular Telangiectasia Type 2.

We report a case of macular telangiectasia type 2 with an associated choroidal neovascular membrane and its treatment. A 54-year-old female presented with a history of gradual vision loss in both eyes. A physical exam showed visual acuity of 20/40 in both eyes and significant metamorphopsia in the right eye. A fundus examination of the right eye was remarkable for right-angled vessels in the juxtafoveal region and subretinal fibrosis temporal to the fovea. A fundus examination of the left eye revealed intraretinal crystals in the juxtafoveal region and right-angled vessels. Optical coherence tomography and angiography confirmed the diagnosis of macular telangiectasia type 2 as well as the presence of a choroidal neovascular membrane in the right eye. The patient was treated with 18 intravitreal injections of anti-vascular endothelial growth factor agent in the right eye for two years, with five to six weeks between each treatment, which resulted in the membrane's stability. Our report suggests that anti-vascular endothelial growth factor therapy via intravitreal injection may be beneficial in treating choroidal neovascular membranes in patients with macular telangiectasia type 2.

Consultations in Eye Pathology: Experience at the Ophthalmology Specialty Hospital.

CONTEXT.­: Ophthalmic pathology is a discipline that relies heavily on a knowledge of clinical ophthalmology. The diagnosis of ocular and periocular lesions can be challenging because some lesions and diseases are unique to this region, whereas others may demonstrate site-specific differences from nonocular counterparts. Because of these challenges, ocular and periocular biopsies are frequently referred to specialized ophthalmic pathology centers for second opinion diagnoses. OBJECTIVE.­: To analyze the referral patterns, diagnostic challenges, and diagnostic discrepancies for second opinion referrals at a dedicated ophthalmic pathology laboratory with an emphasis on lesions of special interest in ophthalmic pathology. DATA SOURCES.­: Data sources included the pathology records of all slides and blocks received in consultation at the referral eye pathology center between December 1, 2015, and December 1, 2022, the personal experience of senior authors, and published peer-reviewed literature. CONCLUSIONS.­: Corneal, intraocular, and conjunctival biopsies are the most common types of cases received in consultation without the referring pathologist's diagnosis, likely reflecting diagnostic challenges. Degenerative intraocular processes occasionally raise concern for a neoplasm. Conjunctival melanocytic lesions are the most common conjunctival biopsies referred for second opinion diagnosis and require careful tissue sampling and clinical-pathologic correlation. Careful clinical-pathologic correlation, a high level of suspicion, and adequate sampling also are required for the accurate diagnosis of periocular sebaceous carcinoma. The diagnostic discrepancies involving uveal, retinal, conjunctival, eyelid, and temporal artery biopsies are most likely to adversely influence patient management and possible outcome. Such specimens may benefit from referral to specialized ophthalmic pathology laboratories.

Identification of the Vibrational Optical Coherence Tomography Corneal Cellular Peak.

Purpose: Our team previously identified the presence of five corneal resonant frequency (RF) peaks in healthy volunteers using vibrational optical coherence tomography (VOCT). Prior studies have suggested that the ≤100 Hz RF peak represents the cellular element of tissue. The aim of this study was to confirm that this peak reflects the human corneal cellular component using VOCT and histological analysis. Methods: Two human research globes were obtained from the same donor, and VOCT measurements were collected from the full-thickness corneas. A microkeratome was then used to create serial-free corneal caps from each cornea, with VOCT performed on the residual stromal bed after each excision. All lamellar sections from both globes were sent for histological analysis to determine cellularity. Cell counts on the specimens were performed by two independent observers. Results: The average of the normalized ≤100 Hz peak values before lamellar sectioning was significantly higher than the average of this peak values after the first, second, and third cuts (P = 0.023), which was 33.9% less than before any cuts. The cell count values in the first slice were significantly higher than the average cell count values of the three deeper slices (P < 0.001), and the cell count dropped 84.4% after the first slice was removed. Conclusions: The findings of this study suggest that the ≤100 Hz corneal peak identified by VOCT corresponds to the cellular component of the cornea. Translational Relevance: This work furthers our understanding of the origin of the corneal ≤100 Hz peak identified using VOCT.

Characteristics Upon Presentation of Patients with Type 2 Macular Telangiectasia in Puerto Rico.

OBJECTIVE: To describe the systemic disease associations and clinical features upon initial presentation of a cohort of patients with type 2 macular telangiectasia who live in Puerto Rico. METHODS: A retrospective review of patients with macular telangiectasia was performed in 4 private retina practices in Puerto Rico. The demographic and clinical characteristics were recorded. RESULTS: Twenty-one patients who were diagnosed with macular telangiectasia were included in the analysis. The median age of presentation was 62 years; 86% were female, and all patients were Hispanics. The median visual acuity at presentation was 20/50. A prior medical diagnosis of type II diabetes mellitus was found in 15 (71.4%) patients, essential hypertension in 12 (57.1%), and dyslipidemia in 9 (42.9%). All patients had bilateral disease. The most common ocular findings were the presence of right-angle vessels in 32 (76.2%) eyes and angiographic hyperfluorescence temporal to the fovea, found in 22 (52.4%) of the affected eyes. One eye had evidence of choroidal neovascularization. CONCLUSION: Our cohort showed a higher prevalence of type 2 diabetes in patients with type 2 macular telangiectasia than in other cohorts. It also supports the findings of other studies showing that macular telangiectasia patients are more likely to have type 2 diabetes and hyperlipidemia. However, the increased prevalence of diabetes and hyperlipidemia may be due to selection bias, and further studies are needed to assess the significance of these findings.