Retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.

A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.

A randomized study of methanol-extraction residue of bacille Calmette-Guerin as postsurgical adjuvant therapy of uveal melanoma.

A randomized controlled clinical trial of methanol-extracted residue of bacille Calmette-Guerin adjuvant treatment of posterior uveal melanoma was undertaken. Of 113 patients, 34 patients received adjuvant immunotherapy and 79 patients received no treatment. No difference in survival was observed between the adjuvant-treated group and the control group of patients. This study found that the size of the tumor was a highly significant risk factor for death caused by metastasis of uveal melanomas. The standard deviation of the nucleolar area of the neoplastic cells was a significant risk factor, even though patients with tumors composed of Callender's spindle-type cells were not included in the study.

Posterior polymorphous dystrophy--a histopathologic presentation.

The corneal button from a 34-year-old woman with posterior polymorphous dystrophy was studied by light and electron microscopy. Ultrastructural findings showed coexistence of epithelial-like cells and abnormal endothelial cells, and Descemet's membrane composed of a normal anterior banded layer, and an irregular posterior homogeneous and collagenous layer. We suggest that these abnormal endothelial cells are in the process of transforming into epithelial-like cells.

Iris nevus syndrome.

A case of iris nevus (Cogan-Reese) syndrome is presented. Our patient's condition was recognized clinically and followed for many years. The unusual amount of proliferated endothelium and abnormal basement membrane and the pedunculated pigment nodules and ectropion uvea were most interesting. If there is a spectrum of disease that includes essential iris atrophy, Chandler's syndrome, and iris nevus syndrome, one would have to say this case fits at the iris nevus end.

Radiotherapy for choroidal melanoma. An 18-year experience with radon.

Twenty-three patients were treated with radon therapy for choroidal melanoma at the Ohio State University Hospitals, Columbus, between 1968 and 1976. We present an 18-year experience, including follow-up of at least eight years, in all those receiving therapy. Three patients (13%) died of metastatic disease. Four patients (17.4%) died of other causes. Sixteen patients (69.6%) were alive, with no signs of metastatic disease. Eight patients subsequently required enucleation due to inadequate tumor response. Of the 15 patients who demonstrated successful tumor destruction and retained their eyes, 13 (86.7%) developed substantial irradiation-induced retinopathy, including hard exudates, telangiectasias, neovascularization, microaneurysms, intraretinal and vitreous hemorrhages, secondary glaucoma, and irradiation-induced cataract. Our long-term results indicate a high incidence of both vascular complications and decreased visual acuity.

Uveitis in older patients.

Uveitis usually occurs in patients between 20 and 50 years of age. We determined the percentage of patients over 50 years old who were referred to a major medical center uveitis clinic and what types of uveitis affected persons in this age group. We found that the older patients had uveal inflammatory diseases similar to those of younger persons. Uveitis after 70 years of age was found to be rare. The conditions of 34% of the patients were misdiagnosed before referral with uveitis; the majority of these patients had senile degeneration.

Metastatic carcinoid to the choroid.

This article describes the seventh published case of metastatic carcinoid to the choroid. Only two prior cases have been treated conservatively, one using chemotherapy and the other using photocoagulation and proton beam irradiation. Our patient was unique since she was the first to have conservative therapy by implantation of a radon ring. Initial results were clinically promising, although repeated ultrasound testing actually showed minimal decrease in tumor size. Subsequently, a large retinal detachment caused marked visual deterioration. Persistent pain from neovascular glaucoma prompted later enucleation, and silver staining as well as electron microscopic studies were performed on the choroidal lesion. It was found that the radon ring had little histologic effect on the viability of the tumor cells. This contrasted with an earlier report of the successful treatment of two much smaller choroidal lesions, using external proton beam irradiation. From our pathologic evaluation, we believe that the metastatic carcinoid tumor should be considered relatively radioresistant.

Cryptococcosis in a phthisical eye.

A case of an enucleated phthisical eye was examined pathologically and was diagnosed as uveitis, subretinal granuloma, detached retina, phthisis bulbi. Twenty years later a more detailed study of multiple sections with differential stains revealed the existence of yeast-like bodies morphologically compatible with Cryptococcus neoformans. Systemic involvement was not found in this case, and the patient died 17 years later from an unrelated disease of the cardiovascular system. However an autopsy was not performed. Indirect fluorescent antibody staining technique on unstained deparaffinized sections of the eye tissue was positive for C. neoformans.

Detachment of descemet's membrane with insertion of an intraocular lens.

The complication of Descemet's membrane detaching during cataract surgery is rare. Awareness that this can happen is especially important now that so many intraocular lenses are being implanted. A case is presented where a detachment was not noticed and an intraocular lens stripped Descemet's membrane from the corneal stroma.

Spontaneous corneal perforation with expulsion of the lens and retina in a premature infant.

This case presents an additional ophthalmological complication in the infant under 1000 gm. The potential relationship of spontaneous corneal perforation suggests the need for extreme care in eye management in these infants.

Multifocal ocular histoplasmosis in a dog and cat.

In two cases of naturally occurring disseminated histoplasmosis, multifocal inflammatory lesions were seen in the posterior segment of the eyes. Histologic examination of both animals revealed lesions of active choroiditis (cat) and retinitis (dog) in association with numerous Histoplasma capsulatum.

Detachment of Descemet's membrane (an early complication of cataract surgery).

Two cases are presented where the corneal endothelium and Descemet's membrane spontaneously stripped off the stroma as an early operative complication of cataract surgery. Treatment is discussed. If recognized this problem can be corrected and the operation can proceed without incident.

Sympathetic ophthalmia. A long-term follow-up.

Seventeen cases of sympathetic ophthalmia have been followed up for as long as 23 years (average 10.6 years). Sixty-five percent of those patients treated with corticosteroids retained a visual acuity of 20/60 or better. Complications were frequent and included secondary glaucoma, cataract, exudative retinal detachment, and choroidal scarring. Uneventful cataract extraction was difficult to manage, requiring frequent changes in the steroid dosage, and in one patient two glaucoma procedures. The duration of steroid therapy was quite variable and ranged from a few months to six years or longer. Relapses were common and several occurred many years after the initial episode had resolved. If the histopathologic picture were moderate or severe, the clinical course most often would be difficult and protracted.

Cyclopian monster with anophthalmos.

A cyclopian monster with anophthalmos is a rare occurrence. Such a case is presented along with a case of a cyclops with synophthalmos. The many abnormalities result from an anomalous development of structures derived from the prosencephalon.

Radiotherapy of malignant melanoma of the choroid.

Twenty-two patients with choroidal melanomas were treated conservatively using radioactive radon rings with a mean follow-up of 43 months. In 14 patients there was successful tumor regression. Eight eyes were enucleated because of inadequate tumor response. Of the patients whose tumors were apparently destroyed, two have subsequently died of metastases and one eye was enucleated due to scleral rupture. Of the eight cases unsuccessfully treated, one has subsequently died of metastases. Radiation therapy is likely to be effective in patients with tumors 7 mm in diameter and 2 mm in elevation or less. Tumors larger than 12 mm in diameter and 3 mm in elevation do not respond adequately to irradiation. Relatively large doses of radiation (at least 8,000 rads to the tumor apex) must be used if the tumor is to be effectively obliterated. We believe that radiation effectively can destroy small choroidal melanomas.