Vitreoretinal juncture; epiretinal membranes and vitreous.

This report reviews current knowledge of the ultrastructural features of the vitreoretinal juncture in its normal state, in eyes with posterior vitreous detachment (PVD), and in eyes with epiretinal membranes (ERM's) and then presents new concepts concerning the interrelationship of ERM's and the vitreous. Results show no vitreous residues on retinal surface following uncomplicated rhegmatogenous (senescent) PVD. In eyes that develop ERM's before vitreous detachment, however, a layer of vitreous may be entrapped beneath the ERM and on adjacent membrane-free retina when PVD ocurs. ERM's may be a cause as well as an effect of PVD.

Detection of cytoplasmic S-100 protein in primary and metastatic intraocular melanomas.

The authors sought S-100 protein in sections cut from formalin- or glutaraldehyde-fixed, paraffin-embedded tumors from the eye and other sites by an immunoperoxidase technique, using rabbit antibovine S-100 protein antiserum, swine antirabbit serum, a peroxidase-antiperoxidase preparation, and amino-ethylcarbizole as developer. Tissue from 26 intraocular tumors was examined, the pathologic diagnosis being unknown to the investigators at that time that the tests were performed. Thirteen of 16 malignant melanomas of the choroid contained S-100 protein (81%), while only one of seven retinoblastomas contained S-100 protein (14%). One cutaneous malignant melanoma metastatic to the eye contained S-100 protein, while none of three metastatic nonmelanocytic, nonneural tumors contained the protein. These results are similar to our findings with nonocular tumors, in which 56 of 56 cutaneous melanomas contained S-100 protein (100%) and only 1 of 51 nonneural, nonmelanocytic tumors contained S-100 protein (2%). S-100 protein may be useful as a marker for ocular malignant melanoma.

Necrotizing retinopathy after intraocular inoculation of murine cytomegalovirus in immunosuppressed adult mice.

A light microscopic study was done to investigate retinal changes in healthy and immunosuppressed mice after intraocular inoculation of murine cytomegalovirus (MCMV). A 0.01-ml inoculum containing 10(5) plaque-forming units of MCMV was placed behind the lens in 138 4-week-old Swiss Webster mice. Ninety-eight mice were immunosuppressed with 0.2 mg/g of cyclophosphamide given intraperitoneally at the time of inoculation and 0.1 mg/g of cyclophosphamide every 5 days thereafter. Selected eyes were examined on postinoculation days 5, 10, 15, and 16-20. Evidence of viral infection was most prominent in uveal tissue. Uveal infection developed whether or not animals received cyclophosphamide, but retinal necrosis developed only in immunosuppressed mice. Focal retinal necrosis, primarily involving the outer retinal layers and retinal pigment epithelium, was first observed in an eye examined on day 10. Retinopathy from MCMV was present in three of five eyes (60%) examined on day 15, and in six of 16 eyes (37.5%) examined between days 16-20. Retinal disease was characterized by full-thickness retinal necrosis, scattered cytomegalic cells, intranuclear and intracytoplasmic viral inclusions, and acute and chronic inflammation. These results indicate that MCMV can produce a necrotizing retinopathy in mice and that immunosuppression facilitates infection. Although ocular MCMV infection in immunosuppressed adult mice is a potential model for study of human CMV retinopathy, many differences exist between human CMV and MCMV and between the ocular diseases they produce.

Respirator retina.

Retinas from 14 comatose patients, who had been sustained with a respirator for one or more days before death, had selective characteristic alterations (ie, autophagy, cell swelling, and coagulation necrosis) of inner nuclear layer in a patchy pattern in posterior fundus. Bipolar cells were most often affected, but amacrine and horizontal cells also were substantially damaged. Cells of Müller and vascular cells were largely spared. These lesions are ascribed to oligemia (ischemia) since the inner nuclear layer is a microscopic vascular watershed (boundary zone) between the choroidal and retinal circulations.

Chorioretinal juncture. Vascularization of Bruch's membrane in peripheral fundus.

Pathologic changes in chorioretinal juncture (pigment epithelium, drusen, Bruch's membrane, and choriocapillaris) were studied in the peripheral fundus of eyes removed at autopsy and surgically enucleated eyes by gross, light microscopic, and electron microscopic methods. Vascularization of Bruch's membrane (VBM) occurred in 430 (43%) of eyes at autopsy, was age related, and was most common in the ora zone and temporal sectors; a significant correlation was found with systemic oligemic conditions. Vascularization of Bruch's membrane occurs in the inner collagenous layer of the membrane, which always demonstrates collagenous thickening and often shows intrusion of interstitial-type cells; the vessels emanate from adjacent pars plana. While many degenerative changes of the pigment epithelium accompany VBM, only direct ophthalmoscopic visualization of linear-dendritic depigmentation of overlying pigment epithelium provides diagnostic evidence of vessels. Thus, VBM seems pathogenically related to the fact that the peripheral fundus is a vascular watershed and is selectively vulnerable to senile involutional changes.

Coronal adenomas.

The incidence and morphologic features of coronal adenomas, benign epithelial tumors of the ciliary processes, were studied in 500 autopsy cases. Macroscopically, coronal adenomas are white, globular, often cystic in appearance, of limited growth potential, and found only on the ciliary processes. Microscopically, they contain convoluted sheets or tubes of nonpigmented epithelium, between which are found varying amounts of amorphous eosinophilic, PAS-positive, extracellular material. Coronal adenomas were present in 153 (31%) of 500 cases, were bilateral in 23 (15%), and were thus present in 176 (18%) of the 1,000 eyes examined. Two clinically important complications of coronal adenomas were found, sectoral cataracts and misdiagnosis as iris tumor.

Vitreous cylinders.

Vitreous cylinders, a rarely discussed ophthalmologic entity, were studied in two clinical cases and in five enucleated eyes. The cylinders were found in four cases of ocular toxoplasmosis, one case of vitreous hemorrhage complicating a malignant melanoma, and one case of pars planitis and one of uveitis, both of unknown causes. Results of scanning electron microscopy in one specimen suggested that cylinders are formed by condensation of vitreous collagen fibers. We emphasize the nonspecific nature of this interesting vitreous finding.

Orbital embryonal rhabdomyosarcoma and intracranial schwannoma.

A 39-year-old woman had a large benign intracranial schwannoma of the Gasserian ganglion. Thirty-two years previously, she had an embryonal rhabdomyosarcoma of the orbit. She had been treated by enucleation, local excision, and low-dose supplemental irradiation (1,400 rads). This is the longest survival of which we are aware following any treatment modality in such tumors. No correlation between the two tumors has been established.

Fungal endophthalmitis following intraocular lens implantation. A surgical epidemic.

Thirteen cases of fungal endophthalmitis were caused by Paecilomyces lilacinus. In all cases an intraocular lens was inserted that had been sterilized in sodium hydroxide and neutralized in sodium bicarbonate. In 12 of the 13 cases, it was absolutely determined that a manufacturer's lot 128 was the neutralizing solution used. The same fungus that caused the endophthalmitis was cultured from several of the neutralizing solutions from lot 128. Eight of the 13 eyes eventually required enucleation. Of the remaining five eyes, one eventually recovered 20/25 vision, one recovered 20/80 vision, one had light perception, and two lost light perception (one of these became phthisical). This surgically induced epidemic of fungal endophthalmitis clearly shows the major consequences of a breakdown in quality control for any substance or material used intraocularly.

Small malignant melanoma of the choroid with extraocular extension.

A disciform macular lesion in the hyperpigmented eye of a patient with melanosis oculi demonstrated slowly progressive increase in size during eight years. Clinical observation and serial fundus photography indicated increase in anteroposterior thickness of the lesion to 2.0 mm and in greatest diameter from 3.5 to 6.0 mm. Subsequent echography revealed an overall anteroposterior dimension of 3.4 mm and an increase to 5.5 mm in 20 months. Enucleation of the affected eye with the intact extraocular tumor mass and a local tenonectomy demonstrated a choroidal malignant melanoma of the epithelioid cell type with extraocular extension, peripapillary scleral infiltration, focal invasion of the juxtalaminar optic nerve, and tumor cells in the subarachnoid space. This experience emphasizes the importance of echography in the periodic evaluation of small choroidal malignant melanomas to detect growth not apparent by ophthalmoscopy.

Autoenucleation.

Autoenucleation is a rare occurrence. An unusual case is presented of a young woman who manually extracted her right globe and was left with a temporal hemianopsia in the remaining eye. Legends surrounding autoenucleation and the medical literature are reviewed, with emphasis on psychiatric aspects and pathologic findings in ocular avulsion.

Intraocular coccidioidomycosis.

A case of unsuccessfully treated coccidioidomycosis with intraocular manifestations is presented. Fever, skin lesions and a variety of constitutional symptoms dominated the clinical course. At autopsy there were extensive systemic dissemination and conspicuous intraocular lesions involving the uveal tract. The ocular and systemic features and the immunologic aspects of Coccidioidomycosis are discussed. Previously reported intraocular cases are reviewed and compared to our case.

Isolated metastasis to the optic nerve.

A case of isolated optic nerve metastasis from breast carcinoma is described. Intraocular tumor was discovered three months following a radical mastectomy and was confined to the optic nerve head and distal optic nerve. Following X-irradiation treatment, central retinal vascular occlusion, rubeosis, and neovascular glaucoma developed during the fifteen months before enucleation. The clinicopathologic features of this case are discussed and the literature reviewed.

Solitary retinal astrocytoma.

The clinicopathologic features of retinal or optic disc astrocytomas are discussed based on eighteen reported cases. In addition, a new case of solitary retinal astrocytoma in a patient without other stigmata of phakomatosis is described. The tumor was prominently vascular and caused total exudative retinal detachment. It doubled in diameter over the seven months prior to enucleation, which was dictated by the occurrence of neovascular glaucoma. The astrocytic nature of the lesion was confirmed by immunohistochemical techniques (glial fibrillary acidic protein stain) and by electron microscopy.

Retinal holes.

Holes of the peripheral retina, defined as full-thickness breaks of trophic origin with no associated flap or free operculum, were found in 136 (2.4%) eyes from 2,800 autopsied subjects. Primary retinal holes (those with no indication of a proximal causative lesion and with no lattice degeneration in either eye) occurred in only eight of the 5,600 eyes studied; all were unilateral, single, less than 0.25 disk diameter in size, within the basal zone, and in eyes from elderly subjects. Secondary holes were found in 128 (2.3%) of eyes and of these, lattice degeneration was the most common cause (103). Other lesions complicated by hole formation included zonular traction tufts (10), chorioretinitis (9), meridional folds (3), and pavingstone degeneration (2). Retinal holes in surgically aphakic eyes did not differ qualitatively or quantitatively from those in age-matched phakic eyes.

Drusen of the optic disk in retinitis pigmentosa.

To clarify the nature of mulberry-like excresences of the optic disk, we histopathologically studied the eye of a 66-year-old woman with retinitis pigmentosa. Examination showed that the optic nerve excresences were ordinary drusen and not hamartomas. There was a marked predisposition to mineralization of other intraocular structures.

Traumatic pigmentary retinopathy.

A 56-year-old man with unilateral pigmentary retinopathy had sustained an ocular injury ten years previously. Pathologic examination of the enucleated eye disclosed a spicular pigmentary pattern of the retina in the temporal and inferior sectors. The macular region showed only selective loss of photoreceptor cells and epiretinal membrane formation. The nasal and superior sectors were unremarkable. These findings suggested that traumatic pigmentary retinopathy is not a progressive lesion that always leads to total retinal destruction.

Clinicopathologic findings in anterior hyaloidal fibrovascular proliferation after diabetic vitrectomy.

Two young patients (27 and 30 years old, respectively) with long-standing diabetes mellitus underwent vitreoretinal surgery for traction retinal detachment and vitreous hemorrhage. The postoperative course in each was characterized by early recurrence of the hemorrhage into the vitreous cavity, hypotony, and severe visual loss. Histopathologic examination of both operated on eyes showed anterior extraretinal fibrovascular proliferation extending along the anterior hyaloid to the posterior lens surface, causing traction detachments of the peripheral retina and ciliary body. The vessels originated from the anterior retina. There was no evidence of excess fibrous tissue at the well-healed sclerotomy wounds.

Comparison of lesions predisposing to rhegmatogenous retinal detachment by race of subjects.

Because rhegmatogenous retinal detachments are thought to be much less common in blacks than in whites, we compared the incidence of various lesions known to cause or predispose to this condition (synchysis senilis, posterior vitreous detachment, breaks, tears, and holes of the peripheral fundus, and lattice degeneration of the retina) in a series of postmortem eyes on the basis of race. Our statistical analysis also included trauma, myopia, and chorioretinitis. The series included 322 black subjects and 2,012 white subjects. The subjects ranged in age from 20 to 93 years at the time of death. Although the initial data showed a racial difference in the incidence of synchysis senilis of grade 3 (50% destruction) or higher and posterior vitreous detachment (P = .033 and P = .021, respectively), we found no difference when the data were age-corrected.

Miliary retinitis in coccidioidomycosis.

We studied the pathologic features of a rare case of coccidioidomycosis retinitis in a 12-year-old girl. At autopsy there were multiple small lesions throughout the vital organs. Ocular lesions were restricted to the posterior part of the fundus. Step-level serial sectioning including the optic axes of both eyes showed 29 discrete retinal and 22 choroidal granulomas. Topographic analysis demonstrated random distribution of lesions throughout the fundus. Retinal granulomas were centered within layers served by the retinal circulation. Choroidal lesions most often involved the middle vascular layer and in only two areas focally disrupted Bruch's membrane. The pigment epithelium was intact. Retinal granulomas were not topographically associated with choroidal lesions.