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To determine the long-term effects of cyclocryotherapy, we conducted a follow-up with a mean of 24.9 +/- 13.9 months in 50 eyes of 46 patients with neovascular glaucoma. On final examination, intraocular pressure in 16 (32%) of the eyes was greater than 26 mm Hg and in 17 (34%), less than or equal to 25 mm Hg; 17 (34%) developed phthisis bulbi. Enucleation was performed in three of the eyes. Anterior segment necrosis occurred immediately postoperatively in four eyes. Twenty-four of 41 eyes (58.5%) with vision lost light perception during our study. All patients were comfortable except the three who required enucleation.

A case of acute posterior multifocal placoid pigment epitheliopathy is presented with the unusual feature of the exposure of deep choroidal vessels which filled with dye in the early phase of the fluorescein angiogram in the center of many of the placoid lesions. Visualization of these vessels with surrounding hypofluorescence implies nonperfusion of choriocapillaris presumably due to inflammation, rather than retinal pigment epithelial blockage. The pathology of acute posterior multifocal placoid pigment epitheliopathy is probably a vasculitis of the choriocapillaries, which causes transient occlusion of these vessels and the initial hypofluorescence in the typical lesions during angiography. In many lesions the ischemia is mild enough to cause only temporary disturbance of visual function with subsequent recovery. Only in the center of some of the lesions is the inflammation severe enough to allow for the findings observed in the case presented and to account for the permanent visual defects found in some patients.

Four reports of a rare syndrome characterized by severe, early-onset chorioretinopathy, trichosis, and evidence of pituitary dysfunction are reviewed. Recently obtained follow-up information about these four patients is presented. A new example of this disease, the fifth case to be reported, to our knowledge, is described.

An unusual type of rhegmatogenous retinal detachment is described and compared with a control group of patients with detached retina. Features of the condition, in addition to retinal detachment, include severe anterior and posterior uveitis, choroidal detachment, hypotony, deepened anterior chamber, posterior synechiae, iridophakodonesis, and a poor surgical and visual prognosis due to massive periretinal proliferation. The disease occurs in a disproportionately high ratio in blacks. These cases may be mistaken for uveitis, Harada's syndrome, the uveal effusion syndrome, dislocated lens, or malignant melanoma. It is postulated that the detached retina initiates a series of exaggerated pathophysiological changes in the eye, with the severe inflammation leading to choroidal detachment and hypotony. Such eyes are inoperable until the inflammation, hypotony, and choroidal detachment are reversed with corticosteroid treatment. The surgical and visual prognosis is very poor.

A 51-year-old white man had the B-K mole syndrome (multiple large atypical cutaneous nevi of the upper part of the trunk and extremities, inherited as an autosomal dominant trait, and thought to be more susceptible to malignant transformation), so named after two patients in whom the syndrome was first observed. Two cutaneous malignant melanomas (thigh and back) and an ocular malignant melanoma (ciliary body and iris) simultaneously developed. Patients with B-K mole syndrome have been known to have a very high risk for the development of cutaneous melanoma (including multiple primary cutaneous melanomas) and multiple primary malignancies. There may be a propensity in these patients for development of ocular melanomas.

The acquired chorio-retinal anastomoses occur when the rupture of the Bruch's membrane happens. In these arterial of venous anastomoses, the blood circulates in one way or the other according to the local conditions, as showed in the nine reported cases.

A 30-year-old woman with funduscopic and electrophysiologic findings of retinitis pigmentosa had in addition several asymptomatic serous detachments of the retinal pigment epithelium. She later had a typical serous detachment of the sensory retina which was recurrent, and finally required argon laser therapy.

Description of one case of melanocytosis oculi with malignant melanoma of the choroid of the same eye in a white patient. Microscopical examination confirmed the clinical diagnosis. Recent statistical work which shows that the risk of uveal melanoma is greater in patients with melanocytosis oculi than in the normal white population is recalled.

Ocular manifestations of hypertension involve both retinal and choroidal circulation. Clinical and pathological studies suggest that choroidal changes are present in benign and malignant hypertension, though significantly greater in the later. Lesions classically described as Siegrist and Elschnig spots are described. Clinical evidence of choroidopathy from hypertension and arteriosclerosis has a significant prognostic value for life.

Thirteen patients had a new syndrome and entity in intraocular inflammation called birdshot retinochoroidopathy, which was characterized by a white, painless eye with minimal anterior segment inflammation but particulate debris in the anterior and posterior vitreous. There was profuse retinal vascular leakage with resultant retinal, macular, and disk edema, which are the primary causes of the decreased vision in this syndrome. Long-term follow-up (mean of ten years) revealed that only two patients seemed to respond to anti-inflammatory therapy (systemic corticosteroids) and five patients progressed to 6/60 (20/200) or less visual acuity in at least one eye.

A 27-year-old white woman with anorexia, abdominal pain, arthralgias, spiking fever, diarrhea, and bizarre neurologic symptoms developed yellowish-gray plaques surrounded by hemorrhage in the posterior choroid of both eyes. Progressive thrombocytopenia, hypofibrinoginemia, and accelerated fibrinolysis supported the diagnosis of disseminated intravascular coagulation (DIC). Death occurred from complications of DIC. Widespread thrombotic occlusion of small blood vessels was present in numerous organs, including the posterior choriocapillaris and larger choroidal vessels of both eyes. Other ocular vessels were normal.

A patient's corneal graft rejection arose at the site of an anterior iris adhesion and was reduced by removal of the adhesion.

The authors have followed the evolution of disseminated choroidal tuberculosis during a period of 16 months. At the beginning of the disease, fluorescein angiography showed an early 'screen effect' and later a 'focus effect'. During the later stage of the disease, the pigmented epithelium was seen to be involved as well, as demonstrated by (1) the presence of advanced pigmented chorioretinitis at the sites where the tubercles were observed at the beginning of the disease and (2) the presence of fluorescent areas early during fluorescein angiography.

A report is given of a case of a unilateral condition of this type in a 30-year-old woman, who was observed and repeatedly photographed for more as 7 years.

Two patients had nanophthalmos with uveal effusion and angle-closure glaucoma. They were treated with a method based on the use of the laser, not only to perform iridotomy but to shrink the iris stroma, which appears to open the anterior chamber angle even without iridotomy. Additionally, we introduced three factors that may be diagnostic of nanophthalmos.

Three patients had during pregnancy visual disturbances caused by central serous choroidopathy. One of them had a central scotoma in her first and second pregnancy. The 2 other patients had a central scotoma in their first pregnancy. Symptoms disappeared spontaneously after delivery. Except for the ocular abnormalities the pregnancies were without complications. The complaints can be misinterpreted as pregnancy-related optic neuritis or compressive optic neuropathy, but careful biomicroscopy of the ocular fundus should avoid superfluous diagnostic and therapeutic measures.

Clinical specular microscopic examination of 17 patients with features common to the iridocorneal endothelial syndrome (including Chandler's syndrome, iris nevus syndrome, and essential iris atrophy) showed pathognomonic endothelial changes and no areas of normal endothelial mosaic. Even in those patients with minimal anterior synechiae, gross central endothelial changes could be found, suggesting that the endothelial changes are primary and not secondary to the iris-corneal adhesions. Specular microscopy of the clinically uninvolved contralateral eye in these patients revealed frequent endothelial cell pleomorphism incommensurate with the patient's age. This study also confirmed the value of the specular microscope for qualitative endothelial observations.

We studied 41 young and middle-aged adults who developed innumerable, small, round, uniformly sized, yellow, subretinal nodules that were often arranged in clusters throughout the posterior fundus. Angiographically, they fluoresced discretely during the early arteriovenous phase. In middle or later life 17 of these patients developed visual loss caused by vitelliform retinal detachment in the macula of one or both eyes. Spontaneous resolution of the detachment and retention of good visual acuity occurred frequently. We found evidence indicating that the multiple, small, subretinal nodules are probably focal areas of pigment epithelial attenuation overlying nodular thickenings of the basement membrane of the pigment epithelium. The term basal laminar drusen would distinguish these nodules from those of typical drusen, which are caused by focal detachments of attenuated pigment epithelium and its usually normal-thickness basement membrane from the inner collagenous zone of Bruch's membrane.

A 43-year-old black woman with acquired immunodeficiency syndrome developed bilateral multifocal choroidopathy characterized by slowly enlarging round to oval lesions in the posterior pole and midperiphery. Systemic evaluation revealed no evidence of mycobacterial, fungal, or spirochetal disease. Fluorescein angiography of the lesions showed early hypofluorescence with late staining of the lesions, which appeared deep to the retinal circulation. There was no evidence of retinal involvement. Over a 4-month period of observation, the lesions appeared to enlarge slowly, with no evidence of vitreous cells or debris in the overlying retina. A transscleral choroidal biopsy was performed, and electron microscopy showed numerous cystic structures characteristic of Pneumocystis carinii within necrotic choroid. The lack of inflammatory changes clinically, by fluorescein angiography, and histopathologically was striking.

A case of bilateral CSC is reported: a 36-year-old man, whose fluorogram showed a peculiar change of leak type, namely smokestack phenomenon to ink-blot, and then to descending type in succession. As the leak type changed to descending type, serous detachment of the macula extended to the inferior periphery, and shifting of subretinal fluid occurred.

A 44-year-old woman had a fundus appearance similar to that of gyrate atrophy, a macular lesion, and excessive urinary excretion of proline, hydroxyproline, and glycine. The patient also had abnormal ciliary processes and patchy atrophy of the irides. To our knowledge, this is the first reported case of ocular manifestations associated with iminoglycinuria.

In a patient with the clinical features of the iris nevus syndrome who was treated for glaucoma a cataract developed later. The sector of iris removed during cataract extraction was examined by electron microscopy and was found to be covered by ectopic corneal endothelium with an underlying atypical Descemet's membrane. Abnormalities in the iris included aggregates of melanophagic cells in the anterior stroma and fibrosis in relation to the ectopic endothelium. These findings suggest that many of the iris abnormalities seen in this disorder occur on a reactive basis.

Six eyes developed angle-closure glaucoma secondary to total hemorrhagic retinal detachment that occurred as a rare complication of senile disciform macular degeneration. The patients were all elderly with known senile macular choroidal degeneration. They had no history of eye surgery and experienced sudden loss of vision followed by pain. Balloons of retinal detachment were pushed tightly against the lens in a characteristic "Y suture" appearance causing shallowing of the anterior chamber and glaucoma. Pathologic examination of three eyes revealed that the choroid was not detached but that a massive subretinal hemorrhage was present. Neovascular tissue in macular disciform lesions was found in every case and was the probable source of hemorrhage. Sheets of avulsed retinal outer segments were seen incarcerated in subretinal blood in two cases, and drainage of this subretinal blood in three cases did not restore useful vision.

A 79-year-old man initially had a perforated corneal ulcer of his blind right eye. The next day a spontaneous expulsive choroidal hemorrhage occurred just before scheduled enucleation. Histopathologic examination of the enucleated eye revealed a severe necrosis of several posterior ciliary arteries in the expulsive choroidal hemorrhage, as well as a large occult spindle B malignant melanoma of the choroid. The necrosis of the posterior ciliary arteries may have been the cause of the choroidal hemorrhage. The finding of an occult malignant melanoma may be more than a chance occurrence of two unrelated events, inasmuch as both are associated with necrosis of the posterior ciliary arteries.

The clinical and fluorescein angiographic findings in 2 cases of retinal detachment occurring in toxaemia of pregnancy are presented. Both cases showed angiographic evidence of abnormal choroidal leakage of dye unassociated with signs of retinal angiospasm. Both cases also showed angiographic evidence of delayed filling of the choriocapillaris. We believe this finding has not previously been reported. These observations provide further clinical evidence that retinal detachment in toxaemia of pregnancy is secondary to choroidal damage.

Age-related macular degeneration (AMD) is an important cause of vision loss among the elderly. Because patients with early subretinal neovascular membranes due to AMD can be treated by laser photocoagulation it becomes critical to find tests which can detect early changes. Inasmuch as AMD involves the retinal pigment epithelium, one might expect the electro-oculogram (EOG) to be sensitive to AMD pathology, but the ganzfeld EOG has been found to be normal in most cases. We designed a focal EOG apparatus to try to emphasize the contribution of the central retina, where AMD is found, to the EOG. Seventeen patients with various stages of drusen and AMD were tested and compared with normals. The focal EOG did not separate the AMD patients from the normals, nor did it give different distributions for the various subgroups of AMD. The focal EOG, like the ganzfeld EOG, is not a sensitive test for AMD.

A 44-year-old man had unilateral closure of the anterior chamber angle associated with ciliary body swelling. He was treated with topical cycloplegics and corticosteroids. The shallow chamber deepended to equal the fellow eye in 6 days, and no recurrence has been noted.

In a clinical study of the essential iris atrophies, corneal edema occurred in 49 of 90 patients, and a characteristic hammered-silver appearance of the posterior cornea was visible in 54 cases. Intraocular pressure in the eyes with corneal edema ranged from normal to extreme elevation with glaucomatous damage, and lowering the pressure relieved the corneal edema in many cases with penetrating keratoplasty being effective when other measures failed. Electron microscopy of two keratoplasty specimens revealed extremely few, distinctly abnormal cells lining a collagenous layer posterior to Descemet's membrane.

Two patients who had highly elevated pigmented masses associated with a chorioretinal scar were observed clinically for several years. The scar was due to presumed histoplasmosis in one patient and toxoplasmosis in the other. Histopathological examination disclosed choroidal and subretinal masses consisting of hyperplastic retinal pigment epithelium. The difficulty in making the diagnosis of RPE hyperplasia in such instances is discussed.

The release of subretinal fluid is only required in certain complicated types of retinal detachment and should be avoided when possible. An important consideration in releasing subretinal fluid is the timing of this step in relation to the application of diathermy or cryotherapy as the primary treatment modality. Drainage should precede application of cryotherapy but follow the use of diathermy. The most satisfactory site for drainage is either immediately above or below the medial or lateral long ciliary nerve, just posterior to the equator of the globe. A technique for drainage of subretinal fluid has been developed and evaluated. An 'L'-shaped scleral flap is dissected to produce a relatively staphylomatous zone and the choroid is perforated near its center.