Idiopathic preretinal macular gliosis: a retrospective study of 200 patients.

The charts of the last 200 patients with idiopathic preretinal macular gliosis (IPRMG) seen in the Retina Service of Montefiore Medical Center, Albert Einstein College of Medicine, were reviewed. We found that IPRMG is a disease of older people, tends to have a nonprogressive course, and rarely causes severe visual loss. In our series, 91.5% of the patients were older than 50 years of age, 80% of eyes lost less than two lines of vision, and only 4% of eyes had a visual acuity 20/200.

Mortality and morbidity in patients with central retinal vein occlusion.

We investigated the risk of mortality and morbidity in 78 patients with central retinal vein occlusion (CRVO) followed for more than 2 years (mean follow-up 7.2 years). Thirteen subjects died at an average of 7.0 years after CRVO. Five subjects developed myocardial infarction at an average of 2.8 years after CRVO, and 1 developed a cerebrovascular accident 3 years after CRVO. Patients with CRVO do not carry a higher risk of mortality and morbidity than matched controls derived from national surveys.

Role of diabetes mellitus on the natural history of central retinal vein occlusion.

In order to elucidate the role of diabetes mellitus on the natural history of central retinal vein occlusion, we compared two groups of central retinal vein occlusion, one with diabetes mellitus (32 patients) and one without diabetes mellitus (113 patients). The two groups were quite similar with respect to age, sex, systemic hypertension and glaucoma. No fellow eye had proliferative diabetic retinopathy. The diabetic group had angiographically a more ischemic type of central retinal vein occlusion (72%) than the nondiabetic group (39%; p less than 0.002). Diabetics with central retinal vein occlusion should be closely monitored.

Optic disc size in central retinal vein occlusion.

Predisposing factors in central retinal vein occlusion (CRVO) have included various systemic disorders and glaucoma. Recent reports have suggested a possible relation between specific anatomic configurations of the optic nerve head and the development of anterior ischemic optic neuropathy. The authors analyzed several optic disc parameters in 57 ocular normotensive patients with unilateral CRVO and in 27 control subjects. There was no significant difference in the horizontal disc diameter and cup-to-disc ratio between eyes with unilateral CRVO, fellow eyes with CRVO, and control eyes. The current data suggest that there is no relation between optic disc size and CRVO.

Photostress recovery in chronic open angle glaucoma.

Photostress recovery time was measured in 30 eyes from 15 patients with chronic open angle glaucoma, and 30 eyes from 15 individuals of a similar age group with no ophthalmological disorder. The average recovery time in patients with glaucoma was 70.47 (SD 35.39) seconds. The average recovery time in the control population was 41.97 (SD 17.34) seconds. This difference was statistically significant (p less than 0.001). There was a small positive correlation between age and recovery time in the control population, whereas there was no correlation between age and recovery time in the glaucoma group. There was no correlation between visual acuity and recovery time for either group. There was also no correlation between intraocular pressure and recovery time for the glaucoma group. It was not possible to control for pupillary dilatation in this study. However, it has been previously demonstrated that pharmacological meiosis will not delay photostress recovery time in normal subjects. This is the first report of photostress recovery testing in patients with chronic open angle glaucoma. The results are discussed in terms of the pathophysiology of glaucoma and previous photostress studies in patients with macular disease.

Multiple myeloma and its ocular manifestations.

Multiple myeloma is a malignancy of the cells of the immune system that frequently involves the eye. Cysts of the ciliary body have been reported in 33-50% of myeloma patients, and retinal vascular lesions have been reported in up to 66%. Corneal and orbital involvement is less common. In this review, systemic and immunologic aspects of multiple myeloma are outlined and ocular manifestations are illustrated.

Arteriovenous anastomoses of the retina.

The authors reviewed the clinical findings in 13 patients followed at Montefiore Medical Center and in 85 cases described in the literature with primary arteriovenous communication of the retina (AVCR). The characteristics of AVCR include: young age at detection; both sexes affected; detected on routine examination, and less frequently after decreased vision or referral after diagnosing systemic arteriovenous communication (AVC); unilateral involvement; single or multiple sites in the same fundus; and predilection for the papillomacular area and the superotemporal quadrant. Vision correlated inversely with the grade of AVCR. The presence of concomitant systemic AVC correlated directly with the grade of AVCR. It is concluded that grade I AVCR and the majority of grade II AVCR represent isolated retinal vascular anomalies, and there is no need for extensive search for systemic AVC if these patients remain asymptomatic.

Recurrent conjunctival melanoma with neuroidal spindle cell features.

A 66-year-old white woman had had a 20-year history of flat primary acquired melanosis involving the left inferior forniceal and palpebral conjunctiva. Over the ensuring 12 years, the patient experienced multiple recurrences of invasive malignant melanoma that emerged from the progressive primary acquired melanosis. Two of these recurrences were composed of nonpigmented spindle cells, and in the most florid invasive malignant melanoma that developed, the spindle cells formed a nodule 7.5 mm thick. The spindle cells were organized into fascicles and small bundles, the latter separated by a loose stroma that was devoid of mucopolysaccharides. The fascicular and neuroidal features in this case were sufficiently well developed to suggest the incorrect diagnosis of a neural tumor or a neurofibroma. However, the presence of intraepithelial atypical melanocytes at the edge of the spindle cell lesion, the absence of mucopolysaccharides in the stroma, the mitotic activity, and the absence of intercellular reticulin fibers favored the diagnosis of a spindle cell invasive malignant melanoma. This morphologic variant of conjunctival melanoma is compared with related cutaneous lesions of melanoma featuring a spindle cell population.

Ocular complications in strabismus fixus convergens.

Two cases of strabismus fixus are described. Histopathological examination of the eyeballs from both patients revealed the following complications of this rare disorder: ocular ischemia, atrophy of the optic nerve, and old central retinal artery occlusion. These complications are likely to be secondary to the mechanical stretching and torsion of the optic nerve with subsequent strangulation of its blood supply. Early surgical correction of strabismus fixus is recommended.

Fibrinogen-induced vitreous membranes.

Fibrinogen was injected in the vitreous cavity of 29 rabbits with follow-up for 75 days. Vitreous membrane formation was detected by indirect ophthalmoscopy and confirmed by histopathological study. Membrane formation was significantly more common in the fibrinogen group than in the control group. Fifteen days after injection, the membranes decreased in size and gave place to vitreous liquefaction in the majority of eyes in the fibrinogen group. Histochemical stains for fibrinogen were positive in half of the vitreous membranes. Fibrinogen is thought to be transformed into a long fibrin polymer forming a matrix for surrounding cells to proliferate in the vitreous. An effective fibrinolytic system in the vitreous explains the ultimate resolution of most of the fibrinogen-induced membranes. Simple fibrinogen injections do not provide a good model for long-term vitreous band formation, yet they add more evidence to the role of fibrin in the pathogenesis of vitreoproliferative diseases. The spontaneous resolution of some proliferative vitreoretinopathies parallels the reversibility of most of the fibrinogen-induced vitreous membranes.

Ultrastructure of Bruch's membrane after krypton laser photocoagulation. I. Breakdown of Bruch's membrane.

Previous observations on rats suggested that subretinal neovascularization does not require a prior break in Bruch's membrane (BM). We verified this observation by using scanning electron microscopy to examine large expanses of BM that became exposed following ophthalmoscopically white krypton laser burns. Bruch's membrane appeared intact in the acute phase after injury. Subsequently, slitlike defects in BM were associated with penetrating choroidal capillaries. These observations were consistent with transmission electron microscopic findings of cellular protrusions arising from the choriocapillary endothelial cells and from regenerating retinal pigment epithelial (RPE) cells invading BM. These cell-formed defects in BM differed from thermal defects in appearance, size, and onset of occurrence. Endothelial cells penetrated all layers of BM, including the RPE basement membrane. We conclude that endothelial cells can erode their own basement membrane and the RPE basement membrane, and krypton laser burns with or without immediate rupture of BM induce cellular activity that may result in defects in BM.

Ultrastructure of Bruch's membrane after krypton laser photocoagulation. II. Repair of Bruch's membrane and the role of macrophages.

The accompanying ultrastructural study showed that ophthalmoscopically white krypton laser photocoagulation in rats is followed by cellular invasion causing breakdown of Bruch's membrane (BM). We have expanded these observations, using scanning electron microscopy, transmission electron microscopy, and high-voltage transmission electron microscopy to describe the healing process. The repair of BM involves regenerating retinal pigment epithelial cells and choriocapillaris (CC) that form new basement membranes and fibroblasts that secrete collagen and elastin. The reformation of the CC is also associated with subretinal neovascularization. The involvement of macrophages was evident throughout the process of BM repair and formation of new vessels. We propose that the macrophages act as a common factor linking the diverse diseases associated with choroidal subretinal neovascularization, deduced from our evaluation of the healing process and the reformation of choriocapillaris.

Cellular processes causing defects in Bruch's membrane following krypton laser photocoagulation.

Krypton laser photocoagulation of the posterior fundus of the rat created white retinal lesions comparable with therapeutic burns in the human. Twenty-four hours after injury, the retinal pigment epithelium was destroyed and the majority of the endothelial cells survived. The choriocapillary lumen was occluded. Bruch's membrane was not fractured nor had it undergone thermal lysis in any area studied but choriocapillary endothelial cell buds invaded the choroidal side of Bruch's membrane. After seven days, cellular protrusions arising from retinal pigment epithelial cells traversed Bruch's membrane through full-thickness defects and entered the choroid. Although intense laser burns may destroy Bruch's membrane, we conclude from this data that many so-called "breaks" in Bruch's membrane following laser therapy are formed by cellular processes and are not caused primarily by laser coagulation.

Basal infolding density in the normal pigmented rat.

A variation in retinal pigment epithelial (RPE) basal infolding (BI) density in normal rats was observed and quantified. Serial electron micrographs of the midequatorial region of all quadrants from both eyes of litter-mate pigmented rats, 58 days old, were prepared. The lengths of BI and Bruch's membrane (BM) were determined by tracing enlarged micrographs to a Zeiss MOP-3 analyser. BI density per unit length of BM was assessed over the choriocapillary (CC) lumen, nucleated cytoplasma and intercapillary septum (ICS), and expressed as the dimensionless ratio BI/BM. Fenestrae were counted. The average BI/BM was 8.7; the BI/BM over the ICS, 6.1, was significantly lower than over the CC, 10.7 (P less than 0.001). BI/BM over the lumen and nucleated CC cytoplasm was the same. The number of fenestrae was not strongly associated with BI density (R2: 0.33). These data support the notion that the density of BI is related to diffusion within BM and is proportional to the distance from the CC lumen.

Bilateral total corneal and conjunctival choristomas associated with epidermal nevus.

A 4-year-old boy had been noted from birth to have bilateral masses obscuring the entire outer portion of the eyes. Systemic findings included mild growth retardation, multiple cortical lesions of the distal metaphyseal ends of the lower extremities, and diffuse skin pigmentation of the right side of the body. The epibulbar lesions were excised; on the right side, there was a thin cornea that necessitated corneal transplantation. The diagnosis of composite epibulbar choristomas associated with linear epidermal nevus was established by pathologic examination. This case report as well as a literature review establish the association of epibulbar choristomas with cutaneous linear nevi of the epidermal and sebaceous types.

Ophthalmology consultation in the diagnosis and treatment of sarcoidosis.

We reviewed 204 cases of biopsy-proved sarcoidosis at our institution from 1967 through 1983 to determine the extent of this disease in our catchment area and the degree of awareness by the various medical specialties of sarcoid manifestations. The ophthalmology service was used as an example. Our results showed that there were familial clusters of sarcoid; that one half of the patients born in the United States were from the southeast; that the mode of initial presentation varied considerably; and that only 44% of patients were referred to an ophthalmologist even though more than one half of those examined by an eye doctor manifested signs and symptoms of ocular sarcoidosis. We advocate a more cooperative interdisciplinary approach to the diagnosis and treatment of sarcoidosis because of its broad spectrum of clinical manifestations.

Vascular permeability and the optic disc. Changes after bilateral common carotid ligation in the rat.

The permeability of retinal blood vessels to intravenously injected horseradish peroxidase was examined two to seven days after bilateral common carotid ligation in rats. Light and electron microscopy revealed leakage from the blood vessels of the optic disc. This leakage appeared to result from vesicular transport across the endothelium; the interendothelial tight junctions we examined remained intact and there was no evidence of endothelial cell death. The intraretinal blood vessels and the retinal pigment epithelium retained their normal impermeability to horseradish peroxidase. Cellular edema was present at the optic disc, manifested as swollen axons. Glial cells remained normal in appearance and the extracellular space was not enlarged. These observations clarify those previously derived from funduscopy and fluorescein angiography, which revealed disc edema and hyperfluorescence.

Choriocapillaris atrophy after experimental destruction of the retinal pigment epithelium in the rat. A study in thin sections and vascular casts.

Rats that receive intravenous injections of sodium iodate develop a retinopathy characterized by the partial loss of the retinal pigment epithelium (RPE). In thin sections examined by transmission electron microscopy the choriocapillaris atrophied adjacent to areas of RPE destruction. The endothelial cells thickened and lost their fenestrae and the lumen of the capillary was reduced. At sites where the RPE remained normal in appearance the choriocapillaris did not atrophy. Scanning electron microscopy of vascular casts of the choriocapillaris showed the coexistence of atrophic and normal choriocapillaris throughout the retina, presumably adjacent to sites where the RPE was destroyed or spared, respectively. Our observations support the concept that the RPE exerts some control over the structure and function of the choriocapillaris.

Ocular findings in the facioauriculovertebral sequence (Goldenhar-Gorlin syndrome).

We reviewed the ocular findings in 57 consecutive patients with the facioauriculovertebral sequence (Goldenhar-Gorlin syndrome). Epibulbar choristomas were detected in 18 cases (32%), a much lower occurrence than reported previously. Various motility disorders (11 cases, 19%), blepharoptosis or narrow palpebral fissures (seven cases, 12%), eyelid colobomas (six cases, 11%), and lacrimal drainage system anomalies (six cases, 11%) were more frequent than previously noted. These ocular findings were more common in the patients with epibulbar choristomas. Of the various features of the Goldenhar-Gorlin syndrome (skin tags, microtia, hemifacial microsomia, and vertebral anomalies), only skin tags correlated positively with the laterality of epibulbar choristomas. Preauricular and facial tags represent choristomas, explaining their association with epibulbar choristomas and the laterality they share.