Cases reported "Retinal Diseases"

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1/16. hydroxychloroquine and chloroquine retinopathy: screening for drug toxicity.

    PURPOSE: To report hydroxychloroquine and chloroquine retinopathy and consider screening for drug toxicity. DESIGN: Retrospective observational case series. methods: review of clinical records, visual fields, fundus photographs, and fluorescein angiography of six patients from a retina referral practice. RESULTS: All cases arose because of failure by physicians to avoid dosing above published safe levels. Five cases developed despite accepted ophthalmologic patterns of screening for toxicity. All cases developed parafoveal retinal pigment epithelial atrophic changes and paracentral scotomas to threshold visual field testing. CONCLUSIONS: New cases of hydroxychloroquine and chloroquine toxicity continue to develop in a screening environment. Increased ophthalmologic attention to dosing, awareness of location and nature of early visual field defects, and traditional attention to presence or absence of maculopathy can reduce the incidence of this avoidable condition.
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2/16. growth failure with pericardial constriction. The syndrome of mulibrey nanism.

    The features of the syndrome of mulibrey nanism, an autosomal recessive disorder of unknown pathogenesis, include severe growth failure, yellow pigmentation of the retina, evidence of pericardial constriction, J-shaped sella turcica, and fibrous dysplasia of bones. To date, 24 individuals from finland and a boy from egypt have been reported with the syndrome. The patient reviewed in this article is the first known affected child from the united states. It is important that physicians look for this disorder in children with severe growth failure and hepatomegaly because of the potential seriousness of undetected pericardial constriction.
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3/16. West African crystalline maculopathy.

    OBJECTIVE: To report new observations in West African crystalline maculopathy. DESIGN: Retrospective, observational case series. PARTICIPANTS: Three patients drawn from a private retina practice. methods: review of clinical charts and photographic studies. MAIN OUTCOME MEASURES: Distribution of intraretinal crystals and changes after laser photocoagulation, and history of ingesting foods typical in a West African diet but atypical for an American diet. RESULTS: All patients were older than 50 years, had diabetic retinopathy, ate green vegetables not found in American diets, and showed no deleterious effects of the crystals. Kola nut ingestion in 2 patients was remote and sparse, and was unknown in a third patient. The first 2 affected patients originating outside the Ibo tribe of nigeria are reported. The pattern of retinal crystals can be changed, and the quantity of crystals reduced, by laser photocoagulation of associated diabetic retinopathy. CONCLUSIONS: West African crystalline retinopathy is distinguishable from other causes of crystalline retinopathy. It may reflect a component of the West African diet, seems to have diabetic retinopathy as a promoting factor via breakdown of the blood-retina barrier, and can be modified by laser photocoagulation of diabetic retinopathy. Increased awareness of the condition will allow physicians seeing West African immigrants to make the diagnosis and treat the patients appropriately.
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4/16. Subfoveal nodule in Coats' disease.

    An atypical presentation of Coats' disease is reported with a prominent subfoveal nodule with peripheral retinal exudates. A 6-year old boy presented with 6/120 vision in the left eye associated with an elevated 1 mm subfoveal, circular lesion with peripheral exudates. The fluorescein angiogram showed peripheral retinal telangiectasias with leakage consistent with Coats' disease. The prominent subfoveal nodule is an uncommon initial presentation of Coats' disease and physicians should be aware of this atypical finding.
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5/16. Visual loss due to central serous chorioretinopathy during corticosteroid treatment for giant cell arteritis.

    giant cell arteritis (GCA) can be a devastating disease resulting in blindness if not promptly diagnosed and treated. The only proven treatment for GCA is systemic corticosteroids; however, there are many side-effects associated with this therapy including ocular side-effects such as ocular hypertension, cataract formation and central serous chorioretinopathy. To raise physician awareness, a patient with biopsy-proven GCA is reported who lost vision during corticosteroid therapy because of central serous chorioretinopathy.
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6/16. hypertensive retinopathy caused by a rare ovarian tumor: case report and photo essay.

    A 38-year-old female presented for an optometric exam complaining that her "vision had occasionally gone blank while reading during the past 2 months". Although visual acuity was 20/20 in both eyes, hypertensive retinopathy was present in both eyes. blood pressure was measured to be 210/142. After inadequate response to initial treatment, her family physician referred her to an internist. Ultimately, a four pound Sertoli-Leydig ovarian tumor was found to be causing malignant hypertension. Removal of the tumor and medical treatment of the hypertension cleared the hypertensive retinopathy within 4 months. Prompt optometric care led to the timely discovery of this tumor, and probably prolonged this woman's life. This case illustrates the valuable role optometric care can have in the management of hypertension from any cause. Coordination with the primary physician as to the status of the hypertensive retinopathy is an integral part of the total management. Retinal photographs at presentation and at various stages of treatment dramatically show how the level of fundus pathology is related to the level of hypertension.
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7/16. Investigative studies in von hippel-lindau disease.

    von hippel-lindau disease (VHLD) is an autosomal dominant disorder characterized by cerebellar, spinal cord, and retinal hemangioblastomas; cysts of the kidney, pancreas, liver, and epididymis; and an increased frequency of renal cancer (renal cell carcinoma or hypernephroma), pancreatic cancer, and pheochromocytoma. Since expression of the disorder is markedly variable, patients presenting with only one of these abnormalities should be investigated further to determine if the lesion is isolated or is indicative of VHLD. A retrospective review of charts of patients diagnosed at the Mayo Clinic between 1976 and 1981 as having VHLD or a retinal or central nervous system hemangioblastoma demonstrates that adequate investigations were not performed routinely in patients presenting with either type of hemangioblastoma. Of 28 patients with an apparently isolated central nervous system hemangioblastoma, 21 patients had ophthalmologic examinations. Of 7 patients with retinal hemangioblastoma, only 3 had computerized tomography (CT) of the head. Among 35 patients with either isolated central nervous system or retinal hemangioblastoma, only 8 had intravenous pyelograms and none had ultrasonography or CT of the abdomen. Recently, there has been increasing awareness of the need for additional investigations in patients presenting with apparently isolated hemangioblastomas. However, not all physicians are aware of this need. Furthermore, it must be recognized that a family history of an isolated lesion may be the first clue that a person has undiagnosed VHLD.
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8/16. psoriasis and intraocular inflammation.

    Presented in this series were seven men and three women. Ages when seen, ranged from 32 to 68 years (average 54). psoriasis had begun in childhood in the women and in the late 20's and 30's in the men. arthritis (ankylosing spondylitis) was present in only one. Their ocular inflammations began from ages 26 to 62 (average 41). The onset of the inflammation was acute iritis in four and in indolent iridocylcitis in six. All but one were bilateral and chronic. The vitreous had heavy debris in nine of the ten patients. The retina was normal in only three. Boggy congestion was present in two with cystoid edema. Patches of edema. fluorescein leaking, depigmentation of both maculae, pars plana exudate, and retinal vessel obliteration to grey-white, shaggy cords was present in at least one of the remaining five patients. Systemic corticosteroid therapy has been used in eight of the ten patients described in this report. Doses no higher than 30 mg of prednisone per day were used to initiate reversal of the inflammatory response. In case 10, knowledge of the sensitivity of the process to steroids led to the successful rapid reversal of a recalcitrant iritis with only 20 mg of prednisone as a first dose and 20 mg per day for less than three weeks. maintenance corticosteroid therapy ranged from 40 mg of prednisone every other day in case 9, prednisone 12.5 mg one day and 5 mg the next in case 8, to 8 mg of Aristocort or methylprednisolone acetate (M-edrol) daily in cases 1 and 2. In summary, these patients are older, have an indolent onset bilateral uveitis with dense vitreous debris, retinal abnormalites, and are extremely sensitive to systemic corticosteroids. Many of these patients had undergone the series of clinical evaluations known as a "uveitis survey." Many different systemic abnormalities were found and merited treatment which rarely made a difference in their ocular disease, though two improved after infected teeth were treated. Assuming that these ocular diseases were related to psoriasis, one can then challenge the value of anything found by a "uveitis survey." The author uses information gained from such studies to insure that the patient is in the best possible heatlh and then makes clinical correlations such as presented in this paper. The rarity of this combination of ocular inflammations in patients with psoriasis makes it risky to propose that this is a significant association. Arguments that this is a disease entity began with the clinical similarities: older age, indolent onset, vitreous and retinal involvement, and the extreme sensitivity to systemic corticosteroids. None of these patients had psoriatic arthritis and only one had ankylosing spondylitis. The best explanation for the fact that this disorder has not been emphasized in the past would be an attitude of ophthalmologists and physicians that the eye and skin disease were coincident.
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9/16. Hard Retinal exudates and visual loss due to papilledema.

    Bilateral papilledema developed in a patient with a cystic, grade 3 astrocytoma of the right frontal lobe. Despite successful neurosurgical treatment, 60Co radiotherapy, and oral corticosteroid therapy, progressive visual loss occurred. At examination one year later, visual activity was 20/200 and 20/70, and extensive lipid exudates in the peripapillary retina and central macula of each eye were noted. Retinal lipid exudates rarely complicate the course of surviving patients who had papilledema from intracranial tumor; physicians involved in the multispecialty care of such patients should be aware of the possible ocular residuals of persistent papilledema in an otherwise successfully treated patient.
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10/16. Tuberculous retinal vasculitis.

    A case of central retinal vasculitis associated with culture proven active pulmonary tuberculosis is presented. The ocular condition responded dramatically to systemic isoniazid and ethambutol. The clinical picture of central retinal vein occlusion should alert the physician to the possibility of an inflammatory etiology. Appropriate studies to rule out treatable inflammatory conditions should be ordered.
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