Cases reported "Aniridia"

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1/25. cataract surgery combined with implantation of an artificial iris.

    We describe 6 patients who presented with cataract or aphakia and absent or nonfunctional irides. The etiologies included congenital aniridia, traumatic iris loss, and chronic mydriasis secondary to recurrent herpetic uveitis. In 5 eyes, a prosthetic iris was successfully implanted in combination with small incision cataract surgery. In 2 eyes, a single-piece iris diaphragm and optical lens was implanted. Artificial irides offer a safe alternative for patients who previously had no viable options for iris reconstruction.
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ranking = 1
keywords = iris
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2/25. Black iris-diaphragm intraocular lens for aniridia and aphakia.

    We present the first reported use in the united states of a black iris-diaphragm intraocular lens (IOL) for the treatment of traumatic aniridia and aphakic bullous keratopathy. The patient presented to a university-based practice with contact-lens-intolerant aniridia and aphakia with painful bullous keratopathy from a failed corneal graft. He was treated with combined penetrating keratoplasty and transscleral fixation of an aniridia IOL. The patient's preoperative symptoms of debilitating glare and photophobia resolved substantially after surgery, despite mild postoperative inflammation that resolved. The symptoms associated with aniridia can be successfully treated with a black iris-diaphragm IOL; however, chronic low-grade inflammation has been reported with its use in some cases.
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ranking = 0.75
keywords = iris
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3/25. Mystery of the vanishing iris.

    We present the first reported case of traumatic aniridia as an isolated injury after blunt trauma to an eye that had phacoemulsification through a corneal tunnel. This case provides insight into the properties of the self-sealing corneal tunnel and possible advantages that it may confer on the eye.
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ranking = 0.5
keywords = iris
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4/25. Bilateral cataract surgery combined with implantation of a brown diaphragm intraocular lens after trabeculectomy for congenital aniridia.

    A 17-year-old male patient was referred for poorly controlled glaucoma on maximal medication, congenital aniridia, cataract, nystagmus, and hypoplasia of the macula. A bilateral filtering procedure was performed to control the glaucoma. Three months later, a slow motion phacoemulsification and implantation of a brown diaphragm intraocular lens (IOL) was attempted. Despite the presence of nystagmus and hypoplasia of the macula, the visual acuity improved from 20/300 to 20/100 in the right eye and from 20/400 to 20/150 in the left eye. Both aniridia IOLs were well centered, the anterior segment was quiet with normal intraocular pressure without medication, and all of the patient's glare symptoms disappeared. A single-piece iris diaphragm and optical lens offer a safe alternative for patients who previously had no viable options for iris reconstruction. The most serious postoperative problem, glaucoma, should be addressed before the cataract and lens implantation is performed to avoid a possible acceleration of the glaucoma progression by the large aniridia IOL.
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ranking = 0.25
keywords = iris
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5/25. Management of post-traumatic aniridia with retinal detachment.

    PURPOSE: To reconstruct the anatomic and functional impairment in patients with post-traumatic aniridia, aphakia, and retinal detachment. methods: Four patients with unilateral aniridia and aphakia as well as retinal detachment as results of severe eye injuries underwent scleral buckling, vitrectomy, membrane peeling, endolaser photocoagulation, silicone oil or gas temponade, combined with iris diaphragm-IOL implantation. RESULTS: All four patients achieved successfully anatomic and functional reconstruction after surgery. During five to 22 months postoperative follow-up, all retinas remained attached. The final visual acuity increased from finger counting to 0.1-0.3. CONCLUSIONS: The combination of vitreoretinal surgery and iris diaphrgm-IOL implantation is an effective method for post-traumatic aniridia, aphakia and traumatic retinal detachment. It could ameliorate photophobia and improve the biocular vision. Furthermore, artificial iris diaphragm implantation could prevent silicone oil-endothelia contact and salvage silicone keratopathy.
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ranking = 0.375
keywords = iris
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6/25. Management of traumatic aniridia and aphakia with an iris reconstruction implant.

    We describe the clinical features and management of a 36-year-old man with aniridia and aphakia following blunt ocular trauma. Examination showed partial aniridia and aphakia. We discuss the various options available in the management of this patient and describe the surgical technique involved in the implantation of an iris reconstruction implant.
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ranking = 0.625
keywords = iris
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7/25. The human PAX6 gene is mutated in two patients with aniridia.

    aniridia is an inherited ocular disorder of variable expressivity characterized by iris hypoplasia. A candidate aniridia gene, AN, which is the human homologue of the mouse Pax-6 gene, has recently been isolated by positional cloning from the WAGR region of 11p13. Here we describe mutations in this gene in two cases of sporadic aniridia, one detected at the dna level and one at the rna level, both of which are predicted to affect protein function. Mutations in Pax-6 have been described previously in Small eye, the proposed mouse model for aniridia. We present new phenotypic evidence for the validity of this mouse model.
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ranking = 0.125
keywords = iris
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8/25. Wilms tumour associated with unilateral dysplasia of the iris.

    We report a case of Wilms tumour associated with unilateral partial aniridia. Although Wilms tumour is known to be associated with bilateral aniridia, there is only one previously reported case associated with dysplasia of the iris.
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ranking = 0.625
keywords = iris
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9/25. Artificial iris diaphragm and silicone oil surgery.

    In order to avoid contact between silicone oil and the cornea and subsequent painful corneal dystrophy in aniridial eyes, an artificial iris diaphragm was constructed. It consists of polymethylacrylat (PMMA) and simulates the situation of the iris with a central pupillary opening and inferior iridectomy. To date, these diaphragms have been implanted in 11 cases of the severest ocular trauma with accompanying aniridia and proliferative vitreoretinopathy. In the presence of sufficient residual secretion of the ciliary body (9 cases), the diaphragm assumes the function of normal iris and prevents the silicone oil from coming into contact with the corneal endothelium. The transparent diaphragm ensures a view through to the fundus. In the early postoperative period, there was, as anticipated, a fibrinous reaction in the area of the anterior segment.
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ranking = 0.875
keywords = iris
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10/25. aniridia and Wilm's tumor.

    aniridia (absence of iris) is a congenital, bilateral, uncommon panocular disorder. Whereas the occurrence of aniridia in the general population is 1:50000, it is present in about 1 in 70 patients with Wilm's tumor. This aniridia is sporadic and Wilm's tumor in these cases presents at an unusually early age. aniridia was present in two cases out of 60 cases of Wilm's tumor operated at PGIMS, Rohtak. Both cases presented below two years of age. The recognition of a child with sporadic aniridia should alert to the increased risk of development of Wilm's tumor.
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ranking = 0.125
keywords = iris
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