Congenital glaucoma with sickle cell anemia.
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Biomedical subjects
Publications and source records attributed to L M Jampol.
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Coats' disease is characterized by vascular anomalies in the retina that are usually associated with exudates. In the absence of treatment the disease almost always progresses toward loss of vision and, often, loss of the eye. This report describes three patients in whom some of the retinal lesions apparently regressed spontaneously; only two other such patients are mentioned in the literature. These patients may have had a form of Coats' disease that has a better prognosis than usual for the eye.
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In two patients with sickle cell disease (one hemoglobin SC and one hemoglobin SS), central retinal artery occlusions developed. In one case, the occlusion followed a retrobulbar injection of lidocaine hydrochloride. Although the central retinal artery reperfused in each patient, many secondary peripheral retinal arteriolar occlusions remained. During the subsequent days, multiple salmon-patch hemorrhages developed in the distribution of these occluded arterioles. In one patient, the salmon-patch hemorrhages evolved into atrophic schisis cavities. These unusual cases allowed us to document the origin of salmon-patch hemorrhages after peripheral retinal arteriolar occlusions. The development of the hemorrhages was a delayed phenomenon that occurred hours to days after the initial vascular occlusion. Reperfusion of the damaged ischemic vessels with a blowout of the wall of the vessels seems the most likely explanation for this phenomenon.
Delayed development of choroidally fed neovascularization represents a potentially serious complication of feeder vessel photocoagulation of proliferative sickle cell retinopathy (PSR). Of the 53 photocoagulated eyes, choroidally fed neovascularization developed in 21 within one month to seven years (mean, 32.8 months). This complication appeared in eyes treated with argon laser and xenon arc. In 11, neovascular tissue remained flat in the chorioretinal scar (chorioretinal neovascularization), but in ten, the vessels grew into the vitreous (choriovitreal neovascularization). In many cases of chorioretinal neovascularization, the only subsequent complication was local vitreous hemorrhage. Visual acuities remained near normal. The development of choriovitreal neovascularization was associated with vitreous hemorrhages or retinal detachment in six of ten cases. Final visual acuities, however, were 20/50 or better in nine. Photocoagulation in some cases converted chorioretinal neovascularization to choriovitreal neovascularization or seemed to stimulate further growth of choriovitreal neovascularization. We now recommend no treatment for most cases of choroidally fed neovascularization. Photocoagulation techniques for PSR should attempt to minimize the development of choroidally fed neovascularization.
Four rhesus monkeys received intravenous injections of talc twice weekly for 3 1/2 to ten months. Within one month, talc particles were visible in fine perifoveal retinal vessels in the posterior pole. Continued deposition of talc could then be seen after subsequent injections. Hemorrhages in the nerve fiber layer, cotton-wool patches, and whitish plaques in the choroid were visible ophthalmoscopically. Fluorescein angiography revealed precapillary arteriolar occlusions, capillary nonperfusion, an abnormal foveal avascular zone, and retinal vascular leakage. Vitreous fluorophotometric findings were abnormal in all five eyes tested, while electroretinograms were normal in two eyes with advanced talc retinopathy. Talc retinopathy in the primate is similar to ischemic retinopathies in humans, including human talc retinopathy, sickle cell retinopathy, and hypertensive retinopathy. Subsequent reports will describe the light microscopic and ultrastructural changes in these eyes using tracer studies with in these eyes using tracer studies with horseradish peroxidase.
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Fort-five phakic eyes with open angle glaucoma received argon laser therapy to the trabecular meshwork (laser goniotherapy). The average IOP dropped from 27.5 to 20.3 mm Hg in 22 eyes that were followed up for seven to fourteen months. Tonographic data obtained from 13 eyes prior to and approximately one month after laser treatment demonstrated a 50% increase in the facility of aqueous outflow. Several eyes experienced hypertensive episodes following treatment, in which the IOP increased as high as 60 mm Hg on one occasion. Two eyes developed prolonged uveitis with peripheral anterior synechiae formation. Laser goniotherapy appears to be a beneficial mode of therapy for open angle glaucoma in many patients. Longer follow-up is needed, however, and its role in secondary and aphakic glaucomas needs to be defined.
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A 66-year-old man had a pigmented choroidal lesion in his right eye. Over a subsequent period of 8 1/2 years, the lesion showed slight growth. At autopsy, the diagnosis was spindle B melanoma of the choroid without evidence of systemic metastasis. A very unusual feature of this case was the presence in this patient of seven primary malignant tumors and five benign neoplasms. In addition to the choroidal melanoma, these included three distinct pulmonary carcinomas, leiomyosarcoma of the stomach, adenocarcinoma of the sigmoid colon, and basal cell carcinoma. The literature on multiple malignancies in association with uveal melanoma is reviewed.
Three patients had unusually large capillary aneurysms. Two of the patients had had previous branch vein occlusions, while a third had had a central retinal vein occlusion. The large capillary aneurysms were located in areas of ischaemic retina that were drained by the previously obstructed veins. Retinal ischaemia and, possibly, increased hydrostatic pressure following vein occlusion were postulated to result in the development of these atypical capillary aneurysms. They are similar in size to arterial macroaneurysms, but originate from the venous side of the capillary bed. Like typical capillary microaneurysms and arterial macroaneurysms, these large capillary aneurysms may result in visual loss from macular oedema, serous elevation of the macula, and circinate lipid exudation. Argon laser obliteration of the aneurysms appears to be effective therapy.
Choroidal neovascularisation occurred in 35 out of 57 (61%) patients during a trial of xenon are photocoagulation in proliferative sickle retinopathy. Nineteen patients manifested abnormal vessel systems in the plane of the retina which tended to be benign, not enlarging, and without complications (choroidoretinal neovascularisation). In 16 patients the abnormal vessel systems grew into the vitreous, tended to increase in size, and to be associated with vitreous haemorrhage and retinal traction (choroidovitreal neovascularisation). Both forms of neovascularisation were significantly more common at younger ages. Treatment was generally unsatisfactory, frequently resulting in further enlargement of the lesions. Although the natural history of choroidal neovascularisation and its ultimate effect on visual function remains to be recorded, these lesions constitute a common and potentially serious complication of xenon are photocoagulation in proliferative sickle retinopathy as currently performed.
Three patients had spontaneous separation of an epiretinal membrane from the macular region. Two of the patients had had argon laser photocoagulation of peripheral retinal lesions three to four months earlier. Vitreous contraction, which had led to the fundus conditions that required photocoagulation, may have created alterations in the vitreous or in the membrane itself, which enhanced the peeling of the membranes. Although photocoagulation may sometimes worsen macular puckers, the presence of an epiretinal membrane near the macula should not be considered an absolute contraindication to peripheral fundus photocoagulation when the pathologic process that is to be coagulated has a substantially worse prognosis if left untreated.
Retinal breaks developed in four eyes of four patients with hemoglobin SC disease and advanced proliferative sickle cell retinopathy (PSR) after argon laser photocoagulation of feeder vessels. These cases represented 8.7% of the 46 eyes with PSR treated with the argon laser at our institution in 1977 and 1978. In three patients, the retinal breaks were immediately adjacent to the site of photocoagulation. A scleral buckling procedure was necessary in only one of the four eyes; the breaks were successfully managed in the other three cases by encirclement with further laser treatment. None of the four eyes lost vision. In patients with vitreous traction in association with seafan neovascularization, argon laser photocoagulation may result in retinal breaks and retinal detachment.
Six black patients had fundus lesions diagnostic of the presumed ocular histoplasmosis syndrome. All patients had peripapillary changes or peripheral punched-out chorioretinal lesions, or both. Five of the six had macular subretinal neovascularization, disciform scarring, or an atrophic macular lesion, and one patient had a macular hole; four of five patients tested had a positive histoplasmin skin test. The macular involvement was bilateral in three of the six cases. HLA B-7 was present in one of four patients who had HLA typing. Macular involvement can occur in blacks with the presumed ocular histoplasmosis syndrome.
A 2,948-g full-term male infant, the product of an uncomplicated pregnancy, developed fundus changes consistent with retrolental fibroplasia in the absence of supplemental oxygen therapy. He had no associated illnesses or congenital anomalies, and had not received exchange transfusions. There was no family history of ocular disease. Changes similar to those of retrolental fibroplasia do occasionally occur in fullterm infants who have had no supplemental oxygen therapy. A relative hyperoxia caused by the increase in arterial oxygen saturation occurring at birth is one possible explanation for these events. This rise, plus susceptibility factors apart from prematurity, may account for these unusual cases. Additionally, these cases may represent sporadic examples of familial exudative vitreoretinopathy.
A 38-year-old male drug abuser had multiple emboli in the retinal circulation of the posterior pole of both eyes. He showed widespread peripheral retinal capillary nonperfusion and neovascular proliferation at the junction of perfused and nonperfused retina. The emboli were considered to be talc particles from the intravenous administration of suspensions of oral medications. The presumed mechanism of development of neovascularization in this case was the filtering out of the particles by the retinal vasculature with vaso-occlusion, ischemia and subsequent retinal neovascularization.