Taylor R. Smith

The Corneal Endothelium and the Spectrum of Essential Iris Atrophy

We found that the spectrum of essential iris atrophy (progressive essential iris atrophy, Chandlers syndrome, and the Cogan-Reese syndrome) began as a corneal endothelial degeneration, which, with ectopic endothelial membrane overgrowth over an open angle, followed by contraction of this membrane and further growth onto the iris, accounted for all aspects of the syndrome. The contractile membrane caused the synechiae, the glaucoma, the corectopia, the ectropion uveae, the occasional iris nodules, and the iris atrophy. Thus we found this disease to be primarily of the cornea, and only secondarily of the iris. The results of this study led us to propose a new designation for this spectrum: primary proliferative endothelial degeneration.

Intraocular reticulum cell sarcoma: a case report and literature review.

A 66-year-old white woman with a confusing spectrum of central nervous system signs and symptoms had a diagnostic enucleation of her blind left eye showing retinal infiltration. Histopathologic examination showed reticulum cell sarcoma involving the retina. The patient died one month later and autopsy revealed diffuse central nervous system reticulum cell sarcoma along with right eye and systemic involvement. A review of the literature indicates intraocular reticulum cell sarcoma to be an apparently uncommon occurrence, although ocular examination may be frequently overlooked.

Clinical Manifestations of Brawny Scleritis

We studied four patients with posterior brawny scleritis. Two underwent enucleation for suspected melanoma, and in the other two, the correct diagnosis was made and effective therapy begun. Of seven other eyes with brawny scleritis from other sources, five were enucleated after diagnosis of choroidal melanoma and one for suspected intraocular tumor. This experience and other previous reports indicate the high incidence of diagnostic confusion regarding brawny scleritis. We therefore emphasized clinical symptoms and signs of brawny scleritis: inflammation, tenderness or pain of the globe, history of collagen vascular disease, proptosis, bilaterality, and retinal and choroidal detachment. A preserved normal choroidal vascular pattern over an elevated subretinal mass may be indicative of posterior brawny scleritis. Scleral biopsy is useful for tissue diagnosis. Radioactive phosphorus uptake tests and ultrasonography may erroneously indicate choroidal melanoma and lead to enucleation of a potentially salvageable globe.

Broad Scleral Buckle in the Management of Retinal Detachments with Giant Tears

One of us (T.R.S.) operated on 24 patients with 26 retinal detachments caused by giant tears between 1969 and 1975 with the use of a broad silicone implant and diathermy. Minimum follow-up was six months. The technique included a scleral dissection extending at least three circumferential rows of diathermy posterior to the folded edge of the retinal flap, up onto the ora serrata, and two hours of the clock beyond each end of the tear. The retina was intentionally incarcerated on the buckle when subretinal fluid was drained. Sixty-nine percent of the cases were successfully reattached. If those patients with radial tears extending from the circumferential tears to the disk were excluded, the success rate rose to 75%. Seventeen percent of the successful reattachments required a second scleral buckle operation. Giant tears with radial tears extending to the disk, tears greater than 180 degrees, and immobile posteriorly everted flaps were poor prognostic signs. Thirty-five percent of the eyes with giant tears and a detachment had other holes or tears in the retina of that eye.

Cholesterosis Bulbi: The Ocular Abnormality Known as Synchysis Scintillans

Review of inpatient records at the Massachusetts Eye and Ear Infirmary for ten years and the inpatient and outpatient records at the Childrens Hospital Medical Center for eight years revealed no diagnosed cases of synchysis scintillans. The pathology reports from the Ophthalmic Pathology Laboratory at the Massachusetts Eye and Ear Infirmary for the last ten years revealed 12 eyes with cholesterol crystals in the vitreous cavities or subretinal spaces, or both. All were blind, long-term damaged eyes; there were no vitreous cholesterol crystals observable clinically. This study corroborates previous reports and suggests that synchysis scintillans occurs only in severely damaged, blind eyes not visible to observation. Both the current concept and terminology of synchysis scintillans are inaccurate. Cholesterosis bulbi is a more appropriate term. Cholesterol crystals are clinically different from asteroid bodies since the former occurs only in a damaged, functionless eye.

Rubeosis Iridis in "Pseudogliomas"

A total of 85 eyes (75 patients) with pseudogliomas (i.e., certain conditions which simulate retinoblastoma, were examined histopathologically to determine the incidence of rubeosis iridis. Neovascularization of the anterior surface of the iris was found in 70 eyes (82%). The principal associated changes in these eyes were moderate to severe inflammation of the uveal tract and retinal detachment. These findings are interpreted as indicating that inflammation, retinal detachment, and/or ischemia are effective stimulants in producing iris neovascularization, and rubeosis is not a significant factor in differentiating eyes with retinoblastoma from those eyes with pseudogliomas.

2. Sympathetic Uveitis

Sympathetic ophthalmia isa specific bilateral granulomatous inflammation of the uvea usually following surgical or accidental penetrating trauma to the globe. While generally considered rare, its true incidence is difficult to ascertain. One recent report noted an incidence of approximately two cases per 1,000 accidental perforations, and approximately one case per 10,000 intraocular surgical procedures. 1 Another recent clinical review of cases of sympathetic ophthalmia at one center over the past 20 years showed a decrease in the number of such cases following purely accidental or purely surgical injury, and an increase in the number of cases in which accidental injury was followed by surgical intervention in addition to primary repair. 2 Five cases of sympathetic ophthalmia following trauma and vitrectomy have been reported.3 It has been suggested by some that as more attempts at secondary reconstructive surgery (ie, vitrectomy) in severely traumatized eyes are made, a higher incidence of sympathetic uveitis may be expected. 2 However, it is also possible that a single primary or early secondary repair, including vitrectomy or len sectomy if indicated, may be less likely to incite sympathetic ophthalmia than mUltiple secondary procedures. The importance of early removal of lens material after trauma in the prevention of phacoanaphylaxis (phacogenic uveitis) has been stressed. 4 This report presents an additional case of sympathetic ophthalmia following scleral buckling, vitrectomy, and cataract extraction.

Late Diagnosis of Choroidal Malignant Melanomas in Eyes with Clear Media and Low Visual Acuity

Five patients seen in a one-year period with advanced malignant melanoma of the choroid in eyes with low visual acuity and clear media are reported. The low visual acuity appeared clinically to be antecedent to the malignant tumor; although in certain cases it may have been related to the occult melanoma, this was not appreciated clinically. Because of the low visual acuity, these patients did not have vision symptoms referable to the melanoma, and the malignancy was diagnosed late in its course. All five cases had extrascleral extension of the tumor at the time of surgical treatment; three are dead of metastatic disease, and two have undergone orbital exenteration with only brief follow-up periods. This experience suggests that eyes with clear media and low vision owing to presumably unrelated causes should undergo periodic examination to rule out the presence of a growing malignant melanoma and to prevent its late diagnosis.

Melanocytic tumor of the anterior uvea.

A patient with spindle melanoma involving the iris and ciliary body refused enucleation at the time of diagnosis in 1933. The eye developed painful glaucoma 13 years later and was enucleated. Histopathologic examination indicated spread of the tumor intrasclerally and subconjunctivally. The patient is still alive without evidence of metastases, 32 years after enucleation and 45 years after initial diagnosis.