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Purpose: To evaluate high-risk histopathological features (HRHF) following primary enucleation of eyes with retinoblastoma (RB) and assess the patient outcomes across continents., Methods: Retrospective study of 1426 primarily enucleated RB eyes from five continents., Results: Of all, 923 (65%) were from Asia (AS), 27 (2%) from Australia (AUS), 120 (8%) from Europe (EUR), 162 (11%) from North America (NA), and 194 (14%) from South America (SA). Based on the continent (AS vs. AUS vs. EUR vs. NA vs. SA), the histopathology features included massive choroidal invasion (31% vs. 7% vs. 13% vs. 19% vs. 27%, p=0.001), post-laminar optic nerve invasion (27% vs. 0% vs. 16% vs. 21% vs. 19%, p=0.0006), scleral infiltration (5% vs. 0% vs. 4% vs. 2% vs. 7%, p=0.13), and microscopic extrascleral infiltration (4% vs. 0% vs. <1% vs. <1% vs. 4%, p=0.68). Adjuvant chemotherapy with/without orbital radiotherapy was given in 761 (53%) patients. Based on Kaplan-Meier estimates in different continents (AS vs. AUS vs. EUR vs. NA vs. SA), the 6-year risk of orbital tumor recurrence was 5% vs. 2% vs. 0% vs. 0% vs. 12% (p<0.001), systemic metastasis was reported in 8% vs. 5% vs. 2% vs. 0% vs. 13% (p=0.001), and death in 10% vs. 3% vs. 2% vs. 0% vs. 11% (p<0.001) patients., Conclusion: There is a wide variation in the infiltrative histopathology features of RB across continents, resulting in variable outcomes. SA and AS had a higher risk of orbital tumor recurrence, systemic metastasis, and death compared to AUS, EUR, and NA., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: To report the histologic findings in the eyes of two patients with bilateral retinoblastoma who underwent chemoreduction therapy and enucleation of one eye., Methods: Clinical histories were obtained for both patients. The enucleated eyes were routinely processed and sections were stained with hematoxylin and eosin., Results: The first patient underwent two cycles of carboplatin, vincristine, and etoposide, and the second patient underwent one cycle of carboplatin, vincristine, and etoposide before enucleation. The eyes of both patients exhibited a clinical type 3 regression pattern. Histopathologic examination showed a gliotic mass with interspersed calcifications in one eye and necrotic tumor adjacent to histologically intact retinoblastoma in the other eye., Conclusion: Chemoreduction has variable effects on retinoblastoma and the clinical type 3 regression pattern has several histologic counterparts.

Purpose: To report two cases of regional lymphatic spread of primary uveal melanoma., Methods: The clinical records of two patients who underwent enucleation for uveal melanoma and later developed regional lymph node metastases were reviewed. One of the two eyes was initially treated with proton beam irradiation. Histologic sections of the enucleated eyes and excised lymph nodes were examined., Results: The melanomas arose in the choroid and ciliary body of the two patients and spread to regional lymph nodes 2 years after enucleation. The choroidal melanoma recurred after irradiation, diffusely infiltrated the uveal tract, and extended into the conjunctiva via an emissary canal. The ciliary body melanoma spread through the trabecular meshwork to the conjunctiva., Conclusions: Choroidal and ciliary body melanoma may rarely exhibit regional lymph node metastasis. This mode of metastasis may occur after extraocular spread and invasion of conjunctival lymphatics.

Purpose: The authors describe the clinicopathologic features of an anterior variant of diffuse retinoblastoma., Methods: The clinical history of a child with an anterior chamber infiltrate and cells in the anterior vitreous was reviewed. An anterior chamber aspirate was processed by a Millipore filter technique. The eye was enucleated and routinely processed for light and transmission electron microscopic examination., Results: An 8-year-old girl was treated for anterior uveitis in her right eye that failed to resolve. Examination of an anterior chamber aspirate showed cells suggestive of retinoblastoma. The eye was enucleated, and the enucleation specimen showed retinoblastoma confined to the iris, ciliary body, and anterior vitreous with one microscopic focus of tumor in the peripheral retina., Conclusion: This variant of diffuse retinoblastoma involved only anterior ocular structures with minimal involvement of the retina.

Purpose: To evaluate a tissue sealant (autologous cryoprecipitate activated with bovine thrombin) as an adjuvant in macular hole surgery., Methods: Sixty-nine patients with stage 2, 3, or 4 full-thickness macular hole were enrolled consecutively in a prospective pilot study. Anatomic closure of the macular holes with a single operation was the primary outcome. Fifty-eight patients had pre- and postoperative standardized measurements including best refracted visual acuity, reading speed, and contrast sensitivity. Group A patients (45) had primary macular holes; Group B patients (13) had recurrent macular holes or macular holes with "other" retinal pathology. Surgical technique was standardized and membrane dissections were optional., Results: The anatomic closure rate was 80% with a minimum of 6 months follow-up. Mean improvement in visual acuity for Group A (2.9+/-0.4 lines) was significantly better than for Group B (0.8+/-0.5 lines; P = 0.008). Eyes that underwent internal limiting membrane (ILM) dissections had an anatomic closure rate of 96% (23/24), compared with 71% (32/45) in "non-ILM" cases (P = 0.034). Adverse reactions included sterile hypopyon (10%), intraretinal hemorrhage (9%), pigmentary hyperplasia (3%), and retinal detachment (3%)., Conclusion: Tissue sealants should be evaluated as an adjuvant in macular hole surgery in a randomized clinical trial. Inflammatory reactions may occur in some patients. Internal limiting membrane dissection may improve anatomic closure rates without adversely affecting the visual acuity.

Purpose: The authors report the clinicopathologic features of a recurrent retinoblastoma after radioactive iodine 125 (125l) plaque therapy., Methods: The clinical and histopathologic findings of a 1-year-old boy with a recurrent retinoblastoma after 125l radioactive plaque application were reviewed. The patient's enucleated right eye was processed for routine histologic evaluation. Immunostains for glial fibrillary acid protein and S-100 protein were performed., Results: Examination of the enucleated eye revealed a retinal tumor composed of two populations of cells. One population consisted of poorly differentiated cells, and the second population at the base of the tumor contained well-differentiated cells with numerous fleurettes. Immunohistochemical stains were positive for glial fibrillary acid protein and S-100 protein in the well-differentiated portion of the tumor., Conclusion: This case illustrates a recurrent retinoblastoma that may have arisen from radioresistant well-differentiated cells after 125l plaque therapy.

Purpose: The authors report one patient with definitive and one patient with presumed metastatic cutaneous malignant melanoma to the vitreous and retina diagnosed by cytologic evaluation of the vitreous and subretinal fluid., Methods: A diagnostic vitrectomy was performed on two patients with atypical vitreous and retinal infiltrates. Additionally, subretinal fluid was obtained from the second patient. The specimens were processed for cytologic examination. The cytology specimens were compared with previously excised cutaneous melanomas from the patients., Results: Examination results showed cells in the vitreous with high nuclear-to-cytoplasm ratios, coarse chromatin, and intracytoplasmic melanin pigment granules. The cytologic features were compared with the cutaneous malignant melanoma, and a diagnosis of metastatic melanoma was made. One patient eventually required enucleation, and the metastatic tumor in the other patient was controlled by local irradiation., Conclusions: Cytologic evaluation of the vitreous and subretinal fluid is helpful in establishing a diagnosis in patients with metastatic cutaneous melanoma to the vitreous and retina.

Objective: The authors describe the clinicopathologic features of surgically excised choroidal neovascular membranes imaged by indocyanine green angiography (ICG)., Design: Fifteen eyes from 15 patients with surgically excised subfoveal choroidal neovascular membranes underwent preoperative and postoperative ICG angiography. All excised membranes were examined by light microscopy for overall configuration and the presence of cellular and extracellular constituents. One membrane was examined by transmission electron microscopy., Results: Fourteen patients had age-related macular degeneration and one had ocular histoplasmosis syndrome. Constituents in more than 50% of membranes included retinal pigment epithelium (15/15), vascular endothelium (14/15), inflammatory cells (10/15), and basal laminar deposit (8/15). Basil laminar deposit was present only in membranes of patients with age-related macular degeneration. Eleven membranes were located primarily under the retinal pigment epithelium and four were located primarily under the neurosensory retina. There appeared to be no correlation between the ICG appearance and the histopathologic configuration of the membranes., Conclusion: Surgically excised ICG-imaged membranes correspond to subretinal pigment epithelium and subneurosensory retinal choroidal neovascularization.

Background: There is a spectrum of benign and malignant ciliary body tumors that can be classified by histopathologic examination., Methods: A 55-year-old woman with a history of ovarian carcinoma underwent excision of a ciliary body tumor in her right eye. The tumor was submitted for light microscopic, immuno-histochemical, and ultrastructural examination., Results: The tumor was composed of sheets and cords of cells with bland cytologic features. Immunohistochemical stains were positive for S-100 protein and vimentin in tumor cells. Ultrastructural examination showed intercellular junctions and thick basement membrane within the tumor., Conclusion: The tumor was classified as an adenoma of the ciliary body nonpigmented epithelium, a benign tumor with variable histopathologic features.

The ultrastructural features of three surgically excised idiopathic subfoveal neovascular membranes were examined. Ultrastructural features of the cellular components in the membranes included retinal pigment epithelium, endothelial-lined vascular channels, photoreceptor cells, macrophages, myofibroblasts, glial cells, erythrocytes, and ghost erythrocytes. Extracellular constituents included 200-nm to 250-nm collagen fibrils, 100-nm collagen fibrils and fibrin. The findings in this study are consistent with previously reported findings of subfoveal neovascular membranes in age-related macular degeneration and the ocular histoplasmosis syndrome, with the exception of the presence of basal laminar deposit in membranes from age-related macular degeneration.

A 48-year-old woman with a previous a liver transplant and a cataract extraction developed a necrotizing retinochoroiditis in her right eye. Vitreous and serum cultures failed to yield a causative organism. As the retinochoroiditis progressed, visual acuity worsened to the point of no light perception, and the eye became blind and painful. An enucleation was performed and histologic examination showed Toxoplasma gondii necrotizing retinochoroiditis. This is the first histologically documented case of T. gondii retinochoroiditis after liver transplantation. The fulminant clinicopathologic appearance of the retinochoroiditis is similar to that previously reported to occur in acquired immune deficiency syndrome (AIDS) and other forms of immunosuppression, unlike the reactivated congenital retinochoroiditis that occurs in immunocompetent patients.

A retrospective study was conducted of 308 eyes with pathologic myopia obtained from 202 patients (23 surgical eyes; 285 post mortem eyes) over a 67-year period. Histopathologic findings and percentage of eyes affected, in decreasing order of frequency, were myopic configuration of the optic nerve head, 37.7%; posterior staphyloma, 35.4%; degenerative changes of the vitreous, 35.1%; cobblestone degeneration, 14.3%; myopic degeneration of the retina, 11.4%; retinal detachment, 11.4%; retinal pits, holes, or tears, 8.1%; subretinal neovascularization, 5.2%; lattice degeneration, 4.9%; Fuchs spot, 3.2%; and lacquer cracks, 0.6%. The reasons for enucleation in the surgically obtained eyes included, in decreasing order of frequency: degeneration after retinal detachment; secondary glaucoma; endophthalmitis; postsurgical epithelial ingrowth; expulsive hemorrhage; degeneration after cataract extraction; and presumed intraocular tumor. Clinicopathologic correlations are discussed.

We retrospectively studied eight eyes of seven patients sustaining indirect choroidal rupture secondary to blunt trauma. Clinical and fluorescein angiographic findings were reviewed. The ruptures were single in five eyes and multiple in three eyes. Four eyes contained macular ruptures, and three eyes contained extramacular ruptures. Peripapillary ruptures were noted in three eyes. Three eyes developed subretinal neovascularization as a complication. Location of the rupture but not visual acuity at presentation was predictive of visual outcome. All ruptures were concentric with the optic nerve and vertically oriented, consistent with a break in Bruch's membrane and an associated tethering effect by the optic nerve.

The authors examined an eye obtained post-mortem from a patient with chronic granulomatous disease of childhood and clinically apparent chorioretinal scars. Histologic examination disclosed numerous chorioretinal scars with associated retinal pigment epithelial changes and glial proliferation. Special stains for bacteria and fungi were negative. Additional findings were scattered pigment-containing macrophages found in the patient's spleen, liver, lymph nodes, bone marrow, lungs, kidney, thymus, choroid, and retina. The significance of the pigmented macrophages is unknown, however they may represent an abortive macrophage response to infectious agents.