1/337. blindness as a consequence of a paraneoplastic syndrome in a woman with clear cell carcinoma of the ovary.BACKGROUND: paraneoplastic syndromes are rare conditions associated with cancer that result in serious disease states at unique sites. In 1982, a report of bilateral diffuse uveal melanocytic proliferation associated with nonocular cancers which resulted in blindness was reported. We present a case of a woman with recurrent ovarian cancer who developed this paraneoplastic syndrome. CASE: A 55-year-old woman had been diagnosed in 1990 with an ocular melanoma of her right eye and in 1994 with clear cell carcinoma of the ovary. With recurrence of ovarian cancer, new eye lesions were identified in both eyes. After enulcleation of her right eye, an ocular melanoma and diffuse bilateral melanocytic proliferation (BDUMP) were found. The sight in her left eye continued to deteriorate as other signs of BDUMP occurred in the eye. Within 1 month of diagnosis, the patient was blind. She subsequently succumbed to progression of ovarian cancer. CONCLUSION: Recurrent ovarian cancer is usually an intraabdominal disease that results in gastrointestinal dysfunction. This case illustrates a rare paraneoplastic syndrome associated with ovarian cancer that mimics metastatic disease to the eye, but has a different pathophysiology.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
2/337. Postprandial transient visual loss.BACKGROUND: Temporary loss of vision after ingestion of a large meal is a phenomenon that has been reported in only a small number of cases in the ophthalmic literature. CASE REPORT: A 60-year-old man reported episodes of blurred vision associated with visual aberrations in his left eye for 5 minutes after eating large meals. His medical history was significant for carotid artery disease, hypertension, "diet-controlled" diabetes, and hypercholesterolemia. The patient's symptoms abated when he ate in smaller quantities and was placed on a regimen of IOP-lowering eye drops. CONCLUSIONS: Postprandial transient visual loss can occur when blood gets shunted to the mesenteric system after eating, leaving the eye hypoperfused. It most commonly takes place in individuals whose ocular perfusion is already compromised by carotid disease, in combination with other vascular conditions that result in the inability to compensate for decreased perfusion to the eye. The signs and symptoms of postprandial visual loss--as well as the mechanisms thought to contribute to it--are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = ocular (Clic here for more details about this article) |
3/337. Norrie disease and exudative vitreoretinopathy in families with affected female carriers.PURPOSE: Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness, which is often associated with sensorineural hearing loss and mental retardation. X-linked familial exudative vitreoretinopathy (FEVR) is a hereditary disorder characterized by an abnormality of the peripheral retina and is not associated with systemic diseases. X-linked recessive disorders generally do not affect females. Here we show that female carriers can be associated with manifestation of an X-linked disorder. methods: A four-generation family with an affected female, and a history of congenital blindness and hearing loss, was identified through the pro-band. A second family, with a full-term female infant, was evaluated through ophthalmic examinations and found to exhibit ocular features, such as retinal folds, retinal detachment and peripheral exudates. Peripheral blood specimens were collected from several affected and unaffected family members. dna was extracted and analyzed by single-strand conformation polymorphism (SSCP) following polymerase chain reaction (PCR) amplification of the exons of the Norrie disease gene. The amplified products were sequenced by the dideoxy chain termination method. RESULTS: In an X-linked four-generation family, a novel missense (A118D) mutation in the third exon of the Norrie disease gene, was identified. The mutation was transmitted through three generations and cosegregated with the disease. The affected maternal grandmother and the unaffected mother carried the same mutation in one of their alleles. In an unrelated sporadic family, a heterozygous missense mutation (C96Y) was identified in the third exon of the Norrie disease gene in an affected individual. Analysis of exon-1 and 2 of the Norrie disease gene did not reveal any additional sequence alterations in these families. The mutations were not detected in the unaffected family members and the 116 normal unrelated controls, suggesting that they are likely to be the pathogenic mutations. CONCLUSIONS: The results further strengthen the proposal that X-linked disorders can occur in female carriers, due likely to an unfavorable X-inactivation.- - - - - - - - - - ranking = 0.33333333333333keywords = ocular (Clic here for more details about this article) |
4/337. Surgical treatment of children blinded by stevens-johnson syndrome.PURPOSE: The surgical treatment of severe stevens-johnson syndrome is considered to be very difficult, especially in children. However, ocular surface reconstruction is possible in certain cases. methods: We have performed ocular surface reconstruction by allogeneic corneal epithelial stem cell transplantation in four children blinded by stevens-johnson syndrome. RESULTS: Two cases failed, and the other two had excellent results. The successful cases had good lacrimal function and conjunctival epithelium, with clear corneal stroma and pathology limited to the superficial ocular tissue, whereas the failures did not. CONCLUSIONS: The successful ocular surface reconstruction has been stable for more than 1 year in two cases, suggesting that some patients with stevens-johnson syndrome are very good candidates for ocular surface reconstruction, especially when the patients have good tear function and healthy conjunctival epithelium.- - - - - - - - - - ranking = 1.6666666666667keywords = ocular (Clic here for more details about this article) |
5/337. Sorsby's fundus dystrophy in two Japanese families with unusual clinical features.PURPOSE: To describe two Japanese families with Sorsby's fundus dystrophy (SFD) with unusual clinical features. methods: Two families from Kagoshima Prefecture with senile-onset macular dystrophy were examined. Three affected individuals through three successive generations of one family and three affected siblings in another family were examined and followed. RESULTS: The initial symptom of these patients was a rapid or slow central visual loss that occurred at an average age of 67.4 years. The major ophthalmoscopic changes consisted of soft drusen and hemorrhagic or atrophic lesions in the macula, which were progressive and ultimately led to disciform scarring. They had no difficulty with night vision. All the patients had normal peripheral retina with intact peripheral fields. They maintained good ambulatory vision and could walk unguided until late in life. These patients had a novel mutation in the tissue inhibitor of the metalloproteinases-3 (TIMP3) gene. CONCLUSIONS: This is the first report of SFD from the East. Its clinical features differ from those of SFD patients of the West, appearing closer to features of age-related macular degeneration. These two unrelated Japanese families with an identical mutation in the TIMP3 gene might be descendants of a common ancestor who carried the mutant gene.- - - - - - - - - - ranking = 0.05341235321239keywords = dystrophy (Clic here for more details about this article) |
6/337. Childhood blindness and visual loss: an assessment at two institutions including a "new" cause.PURPOSE: This study was initiated to investigate the causes of childhood blindness and visual impairment in the united states. We also sought a particular etiology--congenital lymphocytic choriomeningitis virus (LCMV)--which has been considered exceedingly rare, in a fixed target population of children, the severely mentally retarded. methods: We undertook a library-based study of the world literature to shed light on the causes of childhood blindness internationally and to put our data in context. We prospectively examined all consented children (159) at 2 institutions in the united states to determine their ocular status and the etiology of any visual loss present. One of the institutions is a school for the visually impaired (hereafter referred to as Location V), in which most of the students have normal mentation. The other is a home for severely mentally retarded, nonambulatory children (hereafter referred to as Location M). This institution was selected specifically to provide a sample of visual loss associated with severe retardation because the handful of cases of LCMV in the literature have been associated with severe central nervous system insults. Histories were obtained from records on site, and all children received a complete cyclopleged ophthalmic examination at their institution performed by the author. patients at Location M with chorioretinal scars consistent with intrauterine infection (a possible sign of LCMV) had separate consents for blood drawing. Sera was obtained and sent for standard TORCHS titers, toxoplasmosis titers (Jack S. Remington, MD, Palo Alto, Calif), and ELISA testing for LCMV (Centers for Disease Control and Prevention, Atlanta, Ga). RESULTS: The diagnoses at Location V were varied and included retinopathy of prematurity (19.4%), optic atrophy (19.4%), retinitis pigmentosa (14.5%), optic nerve hypoplasia (12.9%), cataracts (8.1%), foveal hypoplasia (8.1%), persistent hyperplastic primary vitreous (4.8%), and microphthalmos (3.2%). The most common diagnosis at Location M was bilateral optic atrophy, which was found in 65% of the patients examined who had visual loss. Of these, the insults were most often congenital (42.6%), with birth trauma, prematurity, and genetics each responsible for about 15% of the optic atrophy. The second most common diagnosis was cortical visual impairment (24%), followed by chorioretinal scars (5%), which are strongly suggestive of intrauterine infection. Of 95 patients examined at Location M, 4 had chorioretinal scars. Two of these had dramatically elevated titers for LCMV, as did one of their mothers. One of the other 2 children died before serum could be drawn, and the fourth had negative titers for both TORCHS and LCMV. CONCLUSIONS: At both locations studied, visual loss was most often due to congenital insults, whether genetic or simply prenatal. The visual loss at Location V was twice as likely as that at Location M to be caused by a genetic disorder. The genetic disorders at Location V were more often isolated eye diseases, while those among the severely retarded at Location M were more generalized genetic disorders. Our study identified optic atrophy as a common diagnosis among the severely mentally retarded with vision loss, a finding that is supported by previous studies in other countries. In our population of severely retarded children, the target etiology of lymphocytic choriomeningitis virus was responsible for half the visual loss secondary to chorioretinitis from intrauterine infection. This is more common than would be predicted by the few cases previously described in the literature, and strongly suggests that LCMV may be a more common cause of visual loss than previously appreciated. We believe that serology for LCMV should be part of the workup for congenital chorioretinitis, especially if the TORCHS titers are negative, and that perhaps the mnemonic should be revised to "TORCHS L." Childhood blindness and visual impairment are tragic and co- - - - - - - - - - ranking = 0.33333333333333keywords = ocular (Clic here for more details about this article) |
7/337. Progressive visual loss and ophthalmoplegia.A 51-year-old woman with hyperthyroidism presented with a 4-week history of bilateral progressive visual loss despite treatment with oral prednisone. Her visual function improved after bilateral orbital decompression. The indications for and advantages and disadvantages of radiation therapy and orbital decompression in TAO are discussed. The management of intraocular pressure, strabismus, and lid abnormalities in TAO is also addressed.- - - - - - - - - - ranking = 1.3924536427164keywords = ocular, ophthalmoplegia (Clic here for more details about this article) |
8/337. "A little reminder does no harm": case report.A case of falsified non-accidental ocular injury of a 29 year old male is presented. The importance of an accurate history and appropriate investigations in the management of ocular injuries is highlighted to promote a high standard of health care delivery in zimbabwe.- - - - - - - - - - ranking = 0.66666666666667keywords = ocular (Clic here for more details about this article) |
9/337. Visual loss from arterial steal in patients with maxillofacial arteriovenous malformation.OBJECTIVE: To determine whether an arterial "steal" from the ophthalmic artery accounts for the ocular manifestations associated with maxillofacial arteriovenous malformation (AVM) outside the orbit. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Seven patients with maxillofacial AVM who had been previously treated, unsuccessfully, with proximal ligation of the supplying external carotid artery branches were evaluated clinically and by superselective cerebral angiography. No cases had intraorbital arteriovenous shunting or abnormal venous drainage to the orbit. INTERVENTION: Endovascular embolization. MAIN OUTCOME MEASURES: signs and symptoms of ocular ischemia were correlated with findings on cerebral/orbital angiography. RESULTS: Four of seven patients had signs of ocular ischemia. By selective angiography, these four patients were found to have a significant ophthalmic artery supply to the AVM. In contrast, the three patients without signs of ocular ischemia had minimal or no ophthalmic artery supply to the AVM. CONCLUSIONS: When the ophthalmic arterial blood supply is recruited, ophthalmic artery "steal" phenomenon occurs in patients with maxillofacial AVMs that do not directly involve the orbit. This mechanism appears to be the cause of ocular ischemia. It is possible that this "steal" is precipitated or worsened by previous surgical proximal ligation of external carotid arterial branches that are potential collaterals with the ophthalmic artery but fail to occlude the arteriovenous (AV) shunts.- - - - - - - - - - ranking = 1.6666666666667keywords = ocular (Clic here for more details about this article) |
10/337. Transient visual loss after licorice ingestion.The authors present a spectrum of clinical presentations of transient visual loss/aberrations and show that these occurrences were attributable to ingestion of significant amounts of licorice. A case series of five patients was seen at Geisinger Medical Center between October 1986 and May 1996. Details of patient presentation, treatment, and outcome are discussed. The literature on the effects of licorice derivatives on vascular smooth muscle function is reviewed. Of the five patients, two had documented visual loss by visual acuity testing and visual fields. All patients reported a recent ingestion of significant amounts of licorice (1/4-2 lbs.). All patients had resolution of their visual symptoms; the aid of hyperbaric oxygen was required for one patient. Thorough review of the literature shows recent in vitro and in vivo evidence of glycyrrhetinic acid (the active component of licorice) and carbenoxolone (the hemisuccinate derivative of glycyrrhetinic acid) directly and indirectly enhancing vasoconstrictor action in vascular smooth muscle. It is believed that licorice derivatives can cause retinal or occipital vasospasm, giving rise to transient monocular or binocular visual loss/aberrations. This has not been described before in the literature. It is important to ask about licorice ingestion with patients who present with these visual symptoms.- - - - - - - - - - ranking = 0.66666666666667keywords = ocular (Clic here for more details about this article) |
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