Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Beginning in June 1991, we implanted a newly designed, black diaphragm intraocular lens (IOL) into 13 eyes with congenital aniridia after cataract surgery. To the best of our knowledge, this is the first group of patients to receive a black diaphragm IOL. With the first prototype we experienced various difficulties, which prompted four design modifications. The latest IOL with a diaphragm diameter of 10 mm can be guided safely into the ciliary sulcus. IOL implantation improved visual acuity in 9 of 13 eyes; 4 of these exhibited considerably less nystagmus than was observed preoperatively. During the mean follow-up-period of 7.9 months (range, 1-27 months), a slight, persistent intraocular "inflammation" [Tyndall (+)] was observed in all eyes. Glaucoma occurred postoperatively in 4 eyes and was controlled medically in 2 eyes but remained uncontrolled in 2 other eyes that had the condition preoperatively. This pilot study indicates that most patients with congenital aniridia and cataract benefit considerably from the implantation of this newly designed, black diaphragm IOL. However, preexisting glaucoma may herald severe postoperative glaucoma-related problems and should be considered a contraindication until more information has been gained about the long-term tolerance of this IOL.
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PMID:Black diaphragm intraocular lens in congenital aniridia. 780 3

We describe an eye with traumatic cataract and aniridia in which we implanted a frosted-iris intraocular lens following endocapsular cataract extraction. The lens was well tolerated and the cosmetic result acceptable.
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PMID:Frosted-iris intraocular lens for traumatic aniridia with cataract. 789 70

Thirty-eight cases of congenital visual impairment (CVI) were reported in the Inuit population of Greenland over a period of 40 years (1950-1989), corresponding to a frequency of 86 per 100,000 live born children. The two most common causes of blindness were optic atrophy and cerebral visual impairment due to brain disorders of various etiologies. This finding is in accordance with recently published data from the Nordic countries. On the other hand, retinopathy of prematurity and congenital cataract were rare causes of CVI in Greenland. Fifteen out of the 38 cases had unknown etiology. Genetic disorders accounted for 7/38 of the cases. A separate examination of registered cases with probable genetic visual impairment, irrespective of birth year, disclosed 25 patients. New mutations seemed to be the most reasonable explanation for isolated cases of aniridia, lens ectopia, and Down syndrome, while inbreeding was a possible contributory factor in a few autosomal recessive conditions.
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PMID:Congenital and hereditary visual impairment in Greenland. 801 21

A 12-year-old patient presents aniridia, congenital cataract and nystagmus. The zonular cataract at both eyes has evolved at the right eye. Visual acuity at the right eye is very low (hand movement perception). The lens intracapsular removal at the right eye, with the resection of the hyaloid-capsular ligament, has improved the visual function at 1/6 with adequate correction. The specificity of the case resides in his infrequent occurrence, probably because of a genetic mutation causing partial lack of the zonular fibers.
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PMID:[Congenital aniridia and cataract]. 818 8

In cooperation with Morcher GmbH, we developed a black-diaphragm aphakia intraocular lens (IOL) designed to correct congenital and traumatic aniridia. Since June 1991, we have implanted this IOL into five eyes with congenital aniridia combined with cataract, and into eight eyes with traumatic aniridia combined with cataract or aphakia. Positioning the IOL was rather difficult, especially in the traumatic cases with inadequate capsular support, and in combination with penetrating keratoplasty. After up to 15 months' follow up, functional results are encouraging. However, slight persistent intraocular inflammation was observed in all of the eyes, more obviously in the traumatic cases. The effect of the IOL on the corneal endothelium is still being investigated. Cystoid macular edema was observed in one eye, but probably preexisted in this eye, following several earlier surgical procedures. Glaucoma was observed preoperatively in five eyes; postoperatively, in six. After surgery, it was controlled medically in 4 eyes, surgically in 1, and remained uncontrolled in 1.
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PMID:Black-diaphragm intraocular lens for correction of aniridia. 819 24

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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PMID:Membranous cataract in association with aniridia. 829 72

We report our experience with two patients with familial aniridia who underwent combined cataract extraction and penetrating keratoplasty. Persistent epithelial defects developed in all four eyes within 4 months. Combined lateral tarsorrhaphy and application of disposable soft contact lenses restored the integrity of the corneal epithelium, and 21 months after surgery, the grafts were clear and avascular.
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PMID:Lateral tarsorrhaphy and disposable soft contact lenses in aniridia patients after penetrating keratoplasty. 833 98

Aniridia is a rare panocular disorder which primarily involves not only the iris, but also the retina, optic nerve, lens and cornea. Visual acuity deteriorates as a result of nystagmus, glaucoma, cataract, corneal opacities and retinal hypoplasia. Aniridia may appear as an isolated disorder, most often familial with autosomal dominance or sporadically in association with at least 12 syndromes. Both familial isolated and Wilms tumour, bilateral sporadic aniridia, genitourinary abnormalities and mental retardation syndrome-associated aniridia have been traced to a mutation of the PAX6 gene on band 11p13. Since genetic diagnosis of this disorder is already possible, counselling affected families should be preceded by karyotype studies and linkage analysis in familial cases of isolated aniridia. In sporadic cases of isolated aniridia or WAGR syndrome, we suggest that PAX6 mutation analysis be employed.
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PMID:Aniridia: recent achievements in paediatric practice. 852 75

A 30-year-old man with bilateral congenital aniridia presented with a subluxated cataract in the right eye. He wore a soft contact lens for aphakic correction in the fellow eye after lensectomy for a subluxated cataract. We performed pars plana lensectomy and vitrectomy with implantation of a transscleral ciliary sulcus fixated posterior chamber intraocular lens in the right eye. Good postoperative visual acuity was obtained.
J Cataract Refract Surg 1997 May
PMID:Transscleral ciliary sulcus fixation of a posterior chamber lens in an eye with congenital aniridia. 921

Endoscopic laser cycloablation can be effective in treating glaucoma. The Micro probe endoscopic laser permits excellent visualization of the ciliary processes. However, the surgeon can not simultaneously view through the probe and see the probe's location in the eye through the surgical microscope. An iris adhesion with the probe led to total aniridia in the case reported. Having an assistant watch the operative monitor or devising a system that allows the surgeon to watch both monitors could prevent this complication.
J Cataract Refract Surg 1998 Jan
PMID:Traumatic aniridia during endoscopic laser cycloablation. 949 12


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