Cases reported "Syndrome"

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1/84. Pseudocapsulorrhexis in a patient with iridocorneal endothelial syndrome.

    We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome.
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2/84. Capsular block syndrome with external blockage of the capsular opening by a ciliary sulcus fixated posterior chamber lens.

    PURPOSE: To report capsular block syndrome with external blockage of the capsular opening by a posterior chamber lens fixated in the ciliary sulcus. METHOD: Case report. RESULTS: In an 89-year-old man who had undergone cataract surgery, a posterior chamber lens was accidentally fixated in the ciliary sulcus after continuous curvilinear capsulohhexis and phacoemulsification/aspiration. The next day, capsular block syndrome was noted along the posterior chamber lens optic, which was blocking the capsular opening from the outside. CONCLUSION: In all previously reported eyes with capsular block syndrome, the posterior chamber lens had been placed inside the lens capsule to block the continuous curvilinear capsulorhexis opening from the inside.
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3/84. Increased density of oligodendrocytes in childhood ataxia with diffuse central hypomyelination (CACH) syndrome: neuropathological and biochemical study of two cases.

    We report neuropathological, biochemical and molecular studies on two patients with childhood ataxia with diffuse central nervous system hypomyelination (CACH) syndrome, a leukodystrophy recently defined according to clinical and radiological criteria. Both had severe cavitating orthochromatic leukodystrophy without atrophy, predominating in hemispheric white matter, whereas U-fibers, internal capsule, corpus callosum, anterior commissure and cerebellar white matter were relatively spared. The severity of white matter lesions contrasted with the rarity of myelin breakdown products and astroglial and microglial reactions. In the white matter, there was an increase in a homogeneous cell population with the morphological features of oligodendrocytes, in many instances presenting an abundant cytoplasm like myelination glia. These cells were negative for glial fibrillary acidic protein and antibodies PGM1 and MIB1. Some were positive for myelin basic protein, proteolipid protein (PLP), and myelin oligodendrocyte glycoprotein, but the majority were positive for human 2'-3' cyclic nucleotide 3' phosphodiesterase and all were positive for carbonic anhydrase ii, confirming that they are oligodendrocytes. Myelin protein and lipid content were reduced. The PLP gene, analyzed in one case, was not mutated or duplicated. The increased number of oligodendrocytes without mitotic activity suggests an intrinsic oligodendroglial defect or an abnormal interaction with axons or other glial cells. This neuropathological study supports the notion that CACH syndrome constitutes a specific entity.
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4/84. Peters' anomaly with the fetal transfusion syndrome.

    Of identical twins with the fetal transfusion syndrome, the second twin who was anemic and hypoxemic from early gestation had Peters' anomaly by histologic examination of the host cornea excised during corneal transplantation at 7 months of age. The absence of a normally positioned lens with the incorporation of lens epithelium, capsule, and cortex within the posterior corneal stroma suggested a developmental disturbance in the separation of the lens from the cornea. The severe lenticular disturbance, microphthalmos, and fetal growth retardation in this case reflected early anemia and the resultant hypoxemia. To our knowledge, this is the first time that the fetal transfusion syndrome and Peters' anomaly have been associated.
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5/84. neodymium: YAG laser parabolic anterior capsulotomy in extreme capsule contraction syndrome.

    We report a technique of neodymium: YAG laser parabolic anterior capsulotomy in an extreme case of capsule contraction syndrome with complete occlusion of the central opening and intraocular lens (IOL) displacement. This technique achieves both optical and mechanical benefits of a clear visual axis and possible prevention of further IOL decentration.
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keywords = capsule
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6/84. Two cases of late postoperative capsular block syndrome.

    Two cases of late postoperative capsular block syndrome that occurred 4 and 8.5 years, respectively, were encountered. One case underwent phacoemulsification after continuous curvilinear capsulorhexis in his left eye. The other case had a can opener type capsulorhexis and underwent extracapsular cataract extraction with trabeculectomy. One-piece posterior chamber lenses were implanted in both cases. Upon slit-lamp examination, the posterior capsules were found distorted posteriorly; the capsular openings were apparently sealed by the lens optic. A whitish material existed between the intraocular lens optic and posterior capsule, with thick aggregation in a lower fifth space in case 1. After Nd:YAG laser anterior capsulotomy in case 1, the thick aggregate spread diffusely on the posterior capsule which was sunken completely for 4 weeks. After Nd:YAG capsulotomy, the distorted posterior capsule disappeared and the best corrected visual acuity was restored to 20/20 in both cases.
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keywords = capsule
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7/84. Capsular block syndrome and pseudoexpulsive hemorrhage.

    Capsular block syndrome (CBS) has been recognized as a cause of immediate or delayed postoperative accumulation of fluid behind an intraocular lens/capsulorhexis complex. Hydrodissection-related rupture of the posterior capsule may be considered a variant of CBS that can manifest intraoperatively. We describe another intraoperative situation related to CBS in which fluid loculation during hydrodissection mimics a threatened expulsive hemorrhage.
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8/84. Complete capsular bag distension syndrome.

    After conventional extracapsular cataract extraction and in-the-bag intr aocular lens (IOL) implantation 3 years previously, the partially cut su perior one-third of the anterior capsulotomy flap resealed in the eye of a 55-year-old woman. With the integrity of the capsular bag restored, it distended fully; the IOL was in the center and milky fluid accumulated inside. A neodymium:YAG laser was used to drain the milky fluid and di srupt the opacified anterior and posterior capsules, restoring vision.
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9/84. Ocular findings in Fryns syndrome.

    PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. methods: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.
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keywords = capsule
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10/84. Localization of clinical syndromes using DWI: two examples of the "capsular" warning syndrome.

    The capsular warning syndrome (CWS) is a subtype of transient ischemic attack characterized by its recurrent nature, absence of cortical signs, and high probability of early capsular stroke. Currently, standard imaging techniques have identified only internal capsule lesions in this entity. The authors present 2 cases with an otherwise typical CWS in whom a brainstem stroke was detected by diffusion-weighted imaging (DWI). DWI's ability to differentiate between acute and chronic infarcts may assist in more accurate localization of clinical syndromes.
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