Cases reported "Iris Diseases"

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1/45. 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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2/45. Treatment of fibrinous pupillary membrane occlusion following cataract surgery with microruptor laser.

    A 79-year-old female with rheumatoid arthritis suffered from severe induced iritis with dense fibrinous pupillary membrane occlusion and high intraocular pressure (57 mmHg) following cataract surgery. We used a Nd:YAG laser technique to dissect and move the dense pupillary membrane away and avoid invasive surgical procedures. Postoperatively, we recorded IOP 14 mmHg and visual acuity 20/30.
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3/45. Long-term outcome of aqueous shunt surgery in ten patients with iridocorneal endothelial syndrome.

    PURPOSE: To report the long-term outcome of ten patients with iridocorneal endothelial (ice) syndrome who underwent aqueous shunt surgery for uncontrolled glaucoma. DESIGN: Noncomparative, retrospective case series. PARTICIPANTS: The authors reviewed charts of ten patients with ice syndrome-related glaucoma who underwent aqueous shunt surgery at one institution between 1987 and 1996. MAIN OUTCOME MEASURES: intraocular pressure (IOP), number of glaucoma medications, and further surgical interventions were measured. RESULTS: With a median follow-up of 55 months, four eyes had adequate IOP control (IOP <21 mm Hg) with one or two medications after the initial aqueous shunt surgery. An additional three eyes achieved adequate IOP control after one or more tube repositionings or revisions of the initial aqueous shunt. In this series, the aqueous shunt surgery most often failed because of blocking of the tube ostium by iris, ice membrane, or membrane-induced tube migration. CONCLUSION: Aqueous shunt surgery appears to be an effective method for IOP lowering in some eyes with ice syndrome-related glaucoma when medical treatment or conventional filtration surgeries fail, but additional glaucoma procedures and/or aqueous shunt revisions and tube repositionings are not uncommon.
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4/45. Successful treatment of tractional corectopia using 2 mJ of energy with an Nd:YAG laser.

    Although the Nd:YAG laser is most commonly used to perform posterior capsulotomies after cataract surgery, it has also been used to treat a variety of other anterior segment abnormalities including tractional corectopia, iridocorneal adhesions, persistent pupillary membranes, and posterior synechiae. Numerous reports on the use of the Nd:YAG laser to treat structures in the anterior segment have emphasized the need to use higher pulse energy for pupillary membranes, compared with the lower settings required for posterior capsulotomy. Steinert and Puliafito noted that single pulses of 4 to 12 mJ may be required to treat pupillary membranes "in a manner similar to that of a stonemason chipping at marble" in their description treating a membrane considerably thicker than what we describe. We report the successful treatment of tractional corectopia due to an anterior membrane strand in a child with only 2 mJ of total energy.
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5/45. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.

    BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.
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6/45. Triangular transchamber suture.

    A 64-year-old woman with a fibrous membrane at the lens plane after traumatic loss of all the iris and massive intraocular hemorrhage had posterior chamber intraocular lens (PCIOL) implantation anterior to the fibrous membrane with a triangular transchamber suture to prevent possible PCIOL-corneal touch and enhance the stability of the PCIOL. After 3 years, the PCIOL remained in a good position and visual rehabilitation was satisfactory and without complications.
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7/45. Demonstration of retroiridal extension of an epithelial membrane by ultrasound biomicroscopy: a clinicopathologic correlation.

    PURPOSE: To report the utilization of ultrasound biomicroscopy (UBM) in identifying the extension into the posterior segment of an epithelial membrane, as correlated by the histopathologic findings. METHOD: Case report. UBM findings before enucleation with histopathologic correlation. RESULTS: UBM demonstrated a membrane tracking from the posterior iris surface over the ciliary body and towards the retina. light microscopic examination disclosed the epithelial nature of this membrane. CONCLUSION: UBM can identify extension of an epithelial membrane into the posterior segment and thereby guide the approach to surgical management.
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8/45. Differential diagnosis of corneal oedema assisted by in vivo confocal microscopy.

    The purpose of this study was to demonstrate microstructural differences between clinically similar, but aetiologically different, cases of corneal oedema in four subjects. In vivo confocal microscopy highlighted oedema of the basal epithelium, prominent nerve-keratocyte interactions, and typical 'epithelialization' of the endothelium in a case of iridocorneal endothelial syndrome; however, a similar microstructural appearance was observed in a case of presumed herpetic disciform keratitis. The latter diagnosis was subsequently revised on this basis. Confocal examination of fuchs' endothelial dystrophy demonstrated oedema of the basal epithelium, prominent wing cells, anterior stromal alterations, fibrosis of Descemet's membrane and a typical 'strawberry' appearance of the endothelium. In contrast, in vivo microstructural examination of bilateral keratoconus with hydrops confirmed oedema mainly involving the epithelium and anterior stroma. In vivo confocal microscopy allows the clinician to observe the living cornea at a microstructural level and to better diagnose and differentiate borderline or unusual cases of corneal oedema.
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9/45. Deletion in the OA1 gene in a family with congenital X linked nystagmus.

    AIMS: To elucidate the molecular genetic defect of X linked congenital nystagmus associated with macular hypoplasia in three white males of a three generation family with clear features of ocular albinism in only one of them. methods: A three generation family with congenital nystagmus following X linked inheritance, and associated with macular hypoplasia was clinically examined (three males and two obligate carriers). Flash VEP was performed to look for albino misrouting. dna samples were subjected to PCR and subsequent analysis using SSCP for all exons of the OA1 gene. RT-PCR was performed on a mRNA preparation from a naevus from one patient. PCR products presenting divergent banding patterns in SSCP and from the RT-PCR were sequenced directly using cycle sequencing with fluorescent chain termination nucleotides and electrophoresis in a capillary sequencer. RESULTS: The index case (patient 1, IV.1) was diagnosed with X linked OA1 at the age of 3 months because of typical clinical features: congenital nystagmus, iris translucency, macular hypoplasia, fundus hypopigmentation, normal pigmentation of skin and hair, and typical carrier signs of OA1 in his mother and maternal grandmother. Pigmentation of the iris and fundus had increased at the last examination at age 4 years. Albino misrouting was present at this age. In the maternal uncle (III.3, 51 years) who also suffered from congenital nystagmus there was clear macular hypoplasia and stromal focal hypopigmentation of the iris but no iris translucency or fundus hypopigmentation. Patient 3 (II.3, 79 years, maternal uncle of patient III.3) had congenital nystagmus and was highly myopic. The fundus appearance was typical for excessive myopia including macular changes. The iris did not show any translucency. Molecular genetic analysis revealed a novel 14 bp deletion of the OA1 gene at nt816 in exon 6. The mutation abolishes four amino acids (Leu 253-Ile-Ile-Cys) and covers the splice site. nucleotides 814/815 are used as a new splice donor thus producing a frame shift in codon 252 and a new stop codon at codon 259. CONCLUSIONS: Macular hypoplasia without clinically detectable hypopigmentation as the only sign of X linked OA1 has been reported occasionally in African-American, Japanese, and white patients. The present family shows absent hypopigmentation in two patients of a white family with a deletion in the OA1 gene. We propose a model of OA1 that allows increase of pigmentation with age. We hypothesise that macular hypoplasia in all forms of albinism depends on the extracellular DOPA level during embryogenesis, and that in OA1 postnatal normalisation of the extracellular DOPA level due to delayed distribution and membrane budding/fusion of melanosomes in melanocytes results in increasing pigmentation.
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10/45. Fibrous congenital iris membranes with pupillary distortion.

    BACKGROUND: In 1986 Cibis and associates described 2 children with a new type of congenital pupillary-iris-lens membrane with goniodysgenesis that was unilateral, sporadic, and progressive. These membranes were different from the common congenital pupillary strands that extend from 1 portion of the iris collarette to another or from the iris collarette to a focal opacity on the anterior lens surface. They also differed from the stationary congenital hypertrophic pupillary membranes that partially occlude the pupil, originating from multiple sites on the iris collarette, but not attaching directly to the lens. CASE MATERIAL: The present report is an account of 7 additional infants with congenital iris membranes, similar to those reported by Cibis and associates, which caused pupillary distortion and were variably associated with adhesions to the lens, goniodysgenesis, and progressive occlusion or seclusion of the pupil. Six of the 7 patients required surgery to open their pupils for visual purposes or to abort angle closure glaucoma. A remarkable finding was that the lenses in the area of the newly created pupils were clear, allowing an unobstructed view of normal fundi. CONCLUSION: This type of fibrous congenital iris membrane is important to recognize because of its impact on vision and its tendency to progress toward pupillary occlusion. Timely surgical intervention can abort this progressive course and allow vision to be preserved.
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