Cases reported "Lens Diseases"

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1/20. 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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2/20. Bilateral anterior lenticonus: Scheimpflug imaging system documentation and ultrastructural confirmation of Alport syndrome in the lens capsule.

    BACKGROUND: Alport syndrome is a combination of proteinuria, hematuria, and neurosensory high-frequency deafness. Bilateral anterior lenticonus may be a late sign. diagnosis relies on characteristic electron microscopy changes of glomerular basement membranes in renal biopsy specimens. PATIENT: A 38-year-old man was seen for progressive visual acuity loss (20/400 OU; best-corrected visual acuity, 20/60 OD and 20/50 OS). Findings from slitlamp examination included bilateral anterior lenticonus and central posterior subcapsular cataract, documented using a modified Scheimpflug imaging system. Retinal pathology was not present. On detailed questioning, a history of microhematuria and proteinuria since childhood and progressive high-frequency deafness for years were discovered. The family history was negative for nephropathies, deafness, or eye diseases. cataract extraction rehabilitated the patient's vision. RESULTS: Electron microscopy of a fragile capsulorhexis specimen showed typical thinned basal lamina with basement membrane disruptions. CONCLUSIONS: Anterior lenticonus is a rare bilateral progressive developmental anomaly. More than 90% of cases are associated with Alport syndrome. For diagnosis of Alport syndrome, the presence of 3 of 4 criteria is required: family history positive for Alport syndrome, progressive intra-auricular deafness, characteristic eye anomalies, and positive findings from glomerular ultrastructural examination. We believe that ultrastructural proof of anterior lenticonus may also be provided in the lens capsule. Arch Ophthalmol. 2000;118:895-897
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3/20. True exfoliation of the lens capsule following uveitis.

    PURPOSE: To report a rare case of true exfoliation of the lens capsule following panuveitis, with slit-lamp photographs before and following the development of the true exfoliation. methods: Case report. review of the history and clinical features of a 70-year-old woman who developed true exfoliation during the treatment for panuveitis. RESULTS: After 7 months of treatment for bilateral panuveitis with topical steroid and tropicamide, the patient developed a thin, transparent membrane arising on the lens surface in the right eye. A diagnosis of true exfoliation of the lens capsule was made. CONCLUSIONS: True exfoliation of the lens capsule following uveitis may occur due to the weakening of the anterior capsule by metabolic disorder caused by uveitis and precipitated by repeated mydriasis.
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4/20. 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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ranking = 2.25
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5/20. Cornea with Peters' anomaly: perturbed differentiation of corneal cells and abnormal extracellular matrix in the corneal stroma.

    PURPOSE: We examined histopathologically the anterior ocular segment including the cornea and lens of an eye which had been enucleated in a patient with Peters' anomaly because of untreatable corneal perforation. Special effort was made to differentiate the corneal stromal and endothelial cells, and the stromal extracellular matrix.methods: light microscopy, with hematoxylin and eosin staining, and transmission electron microscopy were employed.RESULTS: Corneal endothelial cells and Descemet's membrane were not detected in the central cornea, where there were immature cells with a fibroblastic configuration. The inner surface of the peripheral cornea was covered with cells containing pigment granules in the cytoplasm. Cell density in the central corneal stroma was relatively high. The diameter of the stromal collagen fibrils was not uniform. A mature collagen fibril-free area was also seen in the central corneal stroma.CONCLUSIONS: Differentiation of neural crest-derived cells in corneal stroma and endothelium might have been perturbed in the cornea of this patient with Peters' anomaly, inducing the defect in the corneal endothelium and the qualitative and quantitative abnormalities of the extracellular matrix.
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keywords = membrane
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6/20. Ultrasound biomicroscopy of capsular delamination (true exfoliation) of the crystalline lens.

    An 86-year-old woman came to our emergency ward, and the impression was acute-angle-closure glaucoma attack with corneal edema of the left eye. Incidentally, we found a diaphanous membrane in the anterior chamber, and it was confirmed to be capsular delamination (true exfoliation) of the crystalline lens using ultrasound biomicroscopy. Capsular delamination of the lens can easily be missed because it is transparent and very thin. It is more difficult to find this membrane if the cornea is not sufficiently clear. Ultrasound biomicroscopy at 50 MHz produces good resolution of the fine diaphanous capsular split membrane. Ultrasound biomicroscopy is an effective method for investigating these difficult cases.
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ranking = 0.75
keywords = membrane
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7/20. Congenital pupillary-iris-lens membrane with goniodysgenesis.

    BACKGROUND: A unilateral congenital pupil-iris-lens membrane with goniodysgenesis syndrome, not benign tunica vasculosa lentis, was first described by Cibis et al. One of three cases developed angle closure. Robb described catastrophic vision loss from angle closure in one of his seven cases. methods: We did a retrospective review of previously unreported cases of pupil-iris-lens membrane with goniodysgenesis seen in our practices. RESULTS: We report the clinical spectrum of a further nine cases, three of which needed surgery for angle closure, two of which needed surgery for clearing the visual axis. CONCLUSION: Congenital pupil-iris-lens membrane with goniodysgenesis is a unilateral membrane clearly differentiated from benign persistent tunica vasculosa lentis tissue. The membrane represents ectopic iris on the lens with abnormal iris stroma and chamber angle from aberrant induction, migration, or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. Surgery may be needed to open the visual axis even when glaucoma is not present and may prevent angle closure.
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ranking = 2.5
keywords = membrane
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8/20. Idiopathic cyclitic retrolental membrane in children.

    BACKGROUND: Cyclitic retrolental membranes (CRM) in children are usually associated with chronic uveitis or genetic syndromes. We report two rare cases of idiopathic CRM. patients AND methods: Two girls aged 9 and 13 years with visual acuities (VA) of 0.05 underwent lensectomy and anterior vitrectomy with dissection of the central part of the retrolental membrane and intraocular lens (IOL) implantation. RESULTS: The clinical evolution was excellent for the 9 year old girl who recovered 1.0 VA after 2 months. Histological examination revealed a fibroelastic tissue of unknown origin without inflammatory components. The 13 year old girl showed VA of 0.6 within 1 month. However, a recurrent CRM developed with retinal detachment and proliferative vitreoretinopathy (PVR). vitrectomy, complete excision of the CRM and 360 degrees retinotomy with silicon oil tamponade attached the retina with limited visual recovery. histology showed fibrovascular tissue with inflammatory components infiltrating the CRM. CONCLUSIONS: Idiopathic CRM in children are rare and can be composed of different histological tissues with very different clinical outcomes.
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ranking = 1.5
keywords = membrane
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9/20. Pupillary-iris-lens membrane with goniodysgenesis: a case report.

    We describe a rare case of pupillary-iris-lens membrane with goniodysgenesis, a unilateral neurocristopathy. The membrane represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. This subject needed treatment for amblyopia.
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ranking = 1.75
keywords = membrane
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10/20. Complications of hypermature cataract: spontaneous absorption of lens material and phacolytic glaucoma-associated retinal perivasculitis.

    Uncommon complications of hypermature cataract in two aged patients are described. One patients, a 58-year-old woman, with unilateral hypermature cataract secondary to Fuchs' heterochromic cyclitis developed spontaneous absorption of the lens material, followed by its dislocation into the anterior chamber. The other patient, a 63-year-old woman, showed phacolytic glaucoma associated with extensive retinal perivasculitis which concurred with leakage of hypermature cataractous lens material and subsided with its complete absorption. Histopathology of the membraneous cataract revealed degenerative changes of the capsule and extinction of the epithelial cells and cortical fibers.
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ranking = 0.25
keywords = membrane
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