Cases reported "Glaucoma, Open-Angle"

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1/55. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.

    OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.
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2/55. Juvenile onset primary open-angle glaucoma: three case studies and review.

    BACKGROUND: Common clinical characteristics of juvenile onset primary open-angle glaucoma (JPOAG) include increased intraocular pressure, optic nervehead damage, visual-field loss, and a normal-appearing iridocorneal angle by gonioscopy. Histologic analysis of the angle structures may show varying developmental abnormalities that are observed to be less obvious as age of onset increases. Individuals who are African-American, male in gender, and myopio, are at highest risk--particularly if a positive family history exists. A genetic locus for juvenile onset primary open-angle glaucoma has been isolated to the long arm of chromosome 1. case reports: Three separate cases of JPOAG diagnosed in a 16-year-old African-American girl, a 9-year-old African-American boy, and a 28-year-old African-American woman are presented and reviewed. The symptoms, clinical presentation, disease progression, and treatment options are discussed in detail. CONCLUSION: Early recognition of this disease is possible through screening on all patients--particularly those who exhibit increased cupping with increased intraocular pressures and risk factors, such as youthful age, male gender, myopic refractive error, and African-American heritage with a positive family history of glaucoma. family members should also be examined.
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3/55. Posner-Schlossman syndrome: a case report.

    BACKGROUND: Glaucomatocyclitic Crisis (AKA Posner-Schlossman syndrome) is a syndrome that affects patients between the ages of 20 and 50 years. It is characterized by a mild recurrent anterior uveitis associated with an out-of-proportion intraocular pressure spike in the same eye. The cause is unclear, but many theories exist. Some researchers have tried to connect it with primary open-angle glaucoma. CASE REPORT: A review of the record of a 41-year-old Hispanic woman with Posner-Schlossman syndrome is discussed. She sought treatment on multiple occasions with symptoms of mild pain, photophobia, and a blurry left eye. Examination revealed mild anterior chamber reaction and an intraocular pressure (IOP) spike in the same eye. She was treated with topical anti-inflammatory and pressure-lowering agents. Each individual flare-up was treated successfully, but the patient began to show equivocal signs of primary open-angle glaucoma (PDAG). She is currently treated prophylactically for POAG with a beta-blocker. No etiologic factor was identified in this patient. The differential diagnosis is straight-forward and the treatment is general uveitic therapy--minus cycloplegics--combined with an IOP-lowering drop. CONCLUSION: This is an interesting and relatively rare uveitic condition. The list of differential diagnoses is long, as is the theorized etiologies; however, clinically the condition is relatively quickly identifiable by the presence (and absence) of signs and symptoms.
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4/55. Increased intraocular pressure and visual field defects in high resistance wind instrument players.

    OBJECTIVE: In this twofold study, part 1 aimed to determine whether the playing of high resistance wind instruments elevates intraocular pressure (IOP) and if so, to investigate the mechanism of IOP elevation and whether its magnitude differs while playing high resistance versus low resistance instruments. The purpose of part 2 was to evaluate whether high resistance players have a greater incidence of glaucomatous changes than other musicians. DESIGN: Three case reports and a cross-sectional study. PARTICIPANTS: Two players of high resistance instruments and one player of high and low resistance wind instruments participated in part 1 of the study. Nine high resistance wind players, 12 low resistance wind players, and 24 nonwind players were recruited among professional musicians in the boston area to participate in part 2. INTERVENTION: In part 1, IOP and uveal thickness changes were measured by pneumatonometry and ultrasound biomicroscopy in two participants playing their high resistance wind instruments (trumpet and oboe) and in a third participant playing both high (trumpet) and low (clarinet and saxaphone) resistance instruments. Each musician in part 2 underwent medical and musical history, measurement of IOP, Humphrey visual field testing, slit-lamp examination, gonioscopy, and dilated examination. MAIN OUTCOME MEASURES: intraocular pressure and uveal thickness changes, and visual field loss and optic nerve head appearance were the main parameters measured in part 1 and part 2, respectively. RESULTS: In part 1, pneumatonometry showed IOP elevation dependent on the force of blowing, and ultrasound biomicroscopy revealed uveal thickening associated with IOP elevation. The magnitude of IOP elevation was dependent on the amount of expiratory resistance provided by the particular instrument. Part 2 showed that life hours of high resistance wind instrument playing had a significant relationship to abnormal visual field (P = 0.03) and corrected pattern standard deviation (CPSD) scores (P = 0.007) in univariate logistic regression and univariate linear regression, respectively. A 0.011-unit increase in CPSD for each 1000 life hours of high resistance wind playing was found. CONCLUSIONS: High and low resistance wind musicians experience a transient rise in their IOP while playing their instruments as a result least in part of uveal engorgement. The magnitude of IOP increase is greater in high resistance wind players versus low resistance wind players. High resistance wind musicians had a small but significantly greater incidence of visual field loss (abnormal fields and increased CPSD scores) than other musicians, which was related to life hours of playing. The cumulative effects of long-term intermittent IOP elevation during high resistance wind instrument playing may result in glaucomatous damage, which could be misdiagnosed as normal-tension glaucoma.
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5/55. Optical coherence tomography and localized defects of the retinal nerve fiber layer.

    PURPOSE: To test the capability of the optical coherence tomography (OCT) to demonstrate and quantitate retinal nerve fiber layer (RNFL) defects. methods: The authors examined 6 eyes of 6 chronic open angle glaucoma patients with the OCT. The patients had abnormal Humphrey 30-2 visual fields which corresponded to RNFL defects visible in monochromatic fundus images taken with a digital imaging system. The RNFL images were used for directing the OCT scans to areas where most information was believed to be obtainable. Several linear scans of different lengths across healthy and abnormal RNFL regions were made. RESULTS: When the OCT images were compared to RNFL photographs, the defective areas showed reduced backscattering with the OCT, being distinctly different from the adjacent normal RNFL. Except for one case the RNFL thickness values were smaller in the areas of abnormal appearance compared to areas of normal appearance. CONCLUSION: This preliminary study suggests that the OCT examination results of the RNFL are in good agreement with the RNFL appearance in monochromatic fundus images.
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6/55. Progressive optic nerve cupping and neural rim decrease in a patient with bilateral autosomal dominant optic nerve colobomas.

    PURPOSE: To document progressive optic nerve cupping and neural rim decrease in a patient with normal intraocular pressures and bilateral autosomal dominant optic nerve colobomas. methods: The ophthalmology records, stereoscopic fundus photographs, and visual fields of a 27-year-old woman with familial (autosomal dominant) optic nerve colobomas were reviewed. The appearance of the optic nerves was documented over a 13-year period (1985 to 1998). RESULTS: Despite repeatedly normal intraocular pressures, the patient showed progressive optic nerve cupping and neural rim decrease in both eyes. Visual field testing was available over a 5-year period (1993 to 1998) and was abnormal, but no progression was seen. CONCLUSIONS: This case of progressive cupping and neural rim decrease in a patient with autosomal dominant optic nerve coloboma in both eyes may provide insight into the optic nerve cupping associated with normal tension glaucoma. Careful follow-up of patients with optic disk colobomas or patients is indicated to detect possible optic nerve changes or field loss.
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7/55. Hereditary motor and sensory neuropathy with myelin folding and juvenile onset glaucoma.

    OBJECTIVE: We describe three patients from a family with motor and sensory neuropathy accompanied by open-angle glaucoma. BACKGROUND: Autosomal recessive demyelinating hereditary motor and sensory neuropathies (HMSN) include different disorders. To our knowledge, autosomal recessive HMSN has not been associated with juvenile onset glaucoma. methods: sural nerve pathology of the three patients were examined, and genetic analysis of the family was performed. Result:- The most prominent pathologic finding was a highly unusual myelin abnormality consisting of irregular redundant loops and folding of the myelin sheath. The family survey supports autosomal recessive inheritance. The molecular analysis failed to demonstrate either linkage of the disease to MPZ gene, PMP22 gene, Cx32 gene, orEGR2 gene. Analysis did not establish linkage of the disease to the locus of CMT4A, 4B, and 4C genes. CONCLUSION: The present cases may represent a new type of HMSN accompanied by juvenile onset glaucoma.
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8/55. serum methanol levels in subjects with or without optic nerve head disease.

    We evaluated serum methanol levels in subjects with or without optic nerve head disease. serum methanol levels were determined using gas chromatography in 71 patients with optic nerve head disease and in 127 subjects without optic nerve head disease. Their ages ranged from 17 to 89 years. serum methanol levels in 127 subjects without optic nerve head disease ranged from 0.12 to 3.86 microg/ml (mean /- standard deviation, 1.72 /- 0.86 microg/ml). In the subjects without optic nerve head disease, the differences in the levels between those with cataract versus retinal detachment, men versus women, and between each age-group (50-80 years) were not significant. The methanol levels in patients with optic neuritis (n = 2), wolfram syndrome (n = 1), Leber hereditary optic neuropathy at the late stage (n = 2), retinitis pigmentosa (n = 23), and primary open-angle glaucoma (n = 16) were less than 3.86 microg/ml. methanol levels in 1 patient with Leber hereditary optic neuropathy at the acute stage was 5.28 microg/ml. Of 10 patients with primary angle-closure glaucoma, 1 had a slightly elevated level and 9 had levels less than 3.86 microg/ml. Of 17 patients with normal tension glaucoma, 5 had methanol levels higher than 3.86 microg/ml, and 12 patients had levels less than 3.86 microg/ml. The present study shows that serum methanol levels in subjects without optic nerve head disease ranged from 0.12 to 3.86 microg/ml and were much lower than the levels that produce acute ocular symptoms of methanol intoxication. It is possible that high serum methanol levels may play a part in the acute stage of Leber hereditary optic neuropathy and normal tension glaucoma in certain patients. It is unlikely that increased serum methanol levels participate in primary angle-closure glaucoma.
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9/55. Management of the Pulfrich phenomenon secondary to pigmentary glaucoma.

    BACKGROUND: The Pulfrich phenomenon can cause annoying symptoms for a patient due to a difference in interocular optic nerve conduction. There are very few reports that describe the successful use of tinted lenses as a treatment modality, while the majority of cases in the literature describe the condition without any reference to treatment. Although there are two previous reported cases of the Pulfrich phenomenon secondary to glaucoma, this article relates the first case of a patient with this phenomenon with glaucoma treated by tinted lenses. CASE REPORT: A 42-year-old woman, who was previously diagnosed with asymmetric pigmentary glaucoma, reported dramatic changes in visual perception-especially with driving and motion-related tasks. The evaluation showed asymmetric optic nerve function and a spontaneous Pulfrich phenomenon. The effects of different grades of neutral-density filters over the better eye were quantified by means of the Pulfrich phenomenon, subjective brightness comparison, and the visual-evoked potential. Relief of some of the symptoms from the Pulfrich phenomenon was achieved using an ophthalmic tint. CONCLUSION: The majority of patients (89%) reported in the literature who are symptomatic of the Pulfrich phenomenon received no treatment. This report illustrates that the traditional optometric tool of tinted lenses can be dramatically effective in relief of the motion-related symptoms secondary to the Pulfrich phenomenon in a patient with pigmentary glaucoma.
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10/55. Ockham's glaucoma.

    The combination of characteristic optic nerve head cupping, arcuate visual field loss and ocular hypertension would usually be thought sufficient to diagnose glaucoma. Only in the absence of elevated intraocular pressure, when normal tension glaucoma may be suspected, would intracranial imaging normally be performed to exclude occult pathology. A case is presented which illustrates the continuing need for vigilance, and an open mind, years after an apparently straight-forward diagnosis has been made.
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