Cases reported "Aniridia"

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1/6. Treatment of traumatic cyclodialysis with vitrectomy, cryotherapy, and gas endotamponade.

    An aphakic patient with severe chronic hypotony had an alternative treatment of a traumatic cyclodialysis cleft: a 3-port pars plana vitrectomy, cryotherapy of the cleft, and fluid-gas exchange with subsequent supine positioning. The therapeutic principle was mechanical apposition of the detached ciliary muscle to the scleral spur by the gas bubble and scar induction by cryotherapy. intraocular pressure increased to within normal ranges, and visual acuity improved over a 15 month follow-up.
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2/6. 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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3/6. Favorable outcome using a black diaphragm intraocular lens for traumatic aniridia with total iridectomy.

    A 25-year-old man had a penetrating injury to the left eye. Ocular examination revealed a full-thickness corneal laceration, total aniridia, anterior capsule rupture, and microscopic hyphema. Ten days after the immediate primary-repair surgery, aspiration of the lens secondary to traumatic cataract was performed. Four months later, a black diaphragm intraocular lens was implanted. Postoperatively, the best corrected visual acuity was 20/20 in both eyes. Multifocal spectacles were prescribed to provide the near and far vision required for the patient's job. Normal intraocular pressure and acceptable photophobia were noted during the 18-month follow-up.
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4/6. The results of glaucoma surgery in aniridia.

    aniridia is an uncommon disorder that may be associated with glaucoma that is usually refractory to conventional medical and surgical therapy. In this study, we report our experience with the surgical management of glaucoma in aniridia in 17 eyes of 10 patients. A total of 45 surgical procedures were performed. The mean preoperative intraocular pressure was 38 mm Hg. The intraocular pressure was ultimately controlled successfully in 11 eyes (intraocular pressure range, 8 to 20 mm Hg) with a mean of 2.8 surgical procedures required in successful cases. trabeculectomy controlled the intraocular pressure in one eye, but it was successful in only 9% of cases in which it was performed. Cyclocryotherapy was successful in five eyes (25% of cases in which it was performed), although complications of phthisis bulbi and progressive cataract occurred. We detail our experience with the Molteno implant, which was successful in five eyes (83% of cases in which it was performed). We have found this type of drainage device to be effective in controlling intraocular pressure in aniridic eyes when previous attempts at control have failed.
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5/6. Membranous cataract in association with aniridia.

    A case of aniridia associated with a membranous cataract and glaucoma in a 52-year-old woman was reported. Bilateral ocular findings included marked hypoplasia of the iris, goniodysgenesis, corneal opacification with superficial vascularization, macular hypoplasia and glaucoma. A membranous cataract was found in the right eye. The intraocular pressure was abnormally high. Since trabeculectomy on the right eye was ineffective, a seton procedure was carried out. During the follow-up period, the membranous cataract in the right eye spontaneously moved from its original position and floated in the vitreous, maintaining its shape.
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6/6. Selective defect of baroreflex blood pressure buffering with intact cardioinhibition in a woman with familial aniridia.

    We report a symptomatic failure of the baroreceptor blood pressure (BP) buffering mechanism in a woman with familial aniridia. Her baseline BP oscillated at 0.1 Hz, the frequency of Mayer waves, with increased amplitude on standing without orthostatic hypotension. Although sudomotor function was normal, cutaneous thermoregulatory function and BP response to Valsalva's maneuver were abnormal. The defective BP buffer mechanism suggests Mayer waves could be a sympathetic mediated cardiovascular resonance. Baroreceptor cardioinhibition was intact. We presume that the lesion is in the rostral aspect of the dorsal medulla oblongata.
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