1/8. retinal degeneration associated with ectopia lentis.Two brothers had retinal degeneration, lens subluxation, and myopia since early life. There was no evidence of marfan syndrome, homocystinuria, or other systemic disease. They had nystagmus, myopia, inferior dislocation of the lens, and posterior subcapsular opacities in both eyes. Fundus examination showed attenuated retinal vessels, macular atrophy with occasional pigment accumulation as clumps, and perivascular sleeves. electroretinography revealed decreased photopic and scotopic responses. The visual fields were constricted. We believe this to be the first report of retinal degeneration with bilateral lens subluxation in a family. It appears to be inherited in an autosomal recessive fashion.- - - - - - - - - - ranking = 1keywords = myopia (Clic here for more details about this article) |
2/8. Craniosynostosis, ectopia lentis, and congenital heart defects: further delineation of an autosomal dominant syndrome with incomplete penetrance.The association of craniosynostosis with ectopia lentis is extremely rare. This was recently reported in monozygotic twin sisters, supporting a genetic etiology for this syndromic association. We report on female first cousins once removed who were born with unilateral coronal synostosis. One cousin also had peripheral pulmonic branch stenosis at birth and was later found to have ectopia lentis and severe myopia. The other cousin had an atrial septal defect, mitral valve prolapse, and only mild myopia. Their intelligence is normal. The inheritance is likely autosomal dominant with variable expression and incomplete penetrance and further defines this syndrome to include congenital heart defects. These findings will have important implications for genetic counseling.- - - - - - - - - - ranking = 1keywords = myopia (Clic here for more details about this article) |
3/8. weill-marchesani syndrome and secondary glaucoma associated with ectopia lentis.weill-marchesani syndrome (WMS) is a rare systemic connective tissue disorder with the systemic features of short stature, short and stubby hands and feet and stiff joints, especially in the hands. Occasionally, it is associated with heart defects and mental retardation. The main ocular features of WMS are microspherophakia (small and spherical crystalline lens), ectopia lentis (a displaced or malpositioned lens), severe myopia and glaucoma. Rare findings include asymmetric axial lengths associated with presenile vitreous liquefaction. A 14-year-old patient with WMS, who developed a secondary glaucoma and suffered visual loss from the ocular features of WMS, is described. The clinical findings and its successful management are also reported.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
4/8. Anterior axial lens subluxation, progressive myopia, and angle-closure glaucoma: recognition and treatment of atypical presentation of ectopia lentis.PURPOSE: To describe the clinical features of an atypical presentation of ectopia lentis consisting of primary anterior axial lens subluxation in childhood, associated progressive myopia, and complicating angle-closure glaucoma; to facilitate early diagnosis of this triad; and to report the results of lensectomy for treatment. methods: A retrospective case review of eight patients. Clinical course, visual acuity, gonioscopy, axial length, refraction, fundus examination, associated systemic features, and results of treatment are provided. RESULTS: This subgroup of patients with ectopia lentis is at risk for progressive, synechial, angle-closure glaucoma. Identification of a clinical triad consisting of rapidly increasing myopia, normal axial length, and progressive axial subluxation leads to earlier diagnosis. Prophylactic lensectomy provides a safe and sight-saving treatment that arrests and, at times, reverses the progression of angle-closure glaucoma. CONCLUSIONS: ectopia lentis with anterior axial subluxation and progressive myopia can be a sight-threatening condition when significant forward mobilization of the lens results in synechial angle-closure glaucoma. Recognition of this clinical entity can expedite diagnosis and prevent irreversible loss of vision secondary to glaucoma. Because the primary mechanism of angle closure is angle crowding, peripheral iridotomy does little to control the rise in intraocular pressure. Prophylactic lensectomy has proven to be the most reliable treatment in our experience.- - - - - - - - - - ranking = 3.5keywords = myopia (Clic here for more details about this article) |
5/8. Clinical manifestations of ectopia lentis et pupillae in 16 patients.The largest known series of patients with ectopia lentis et pupillae (ELeP) was clinically evaluated. Sixteen patients from eight families were characterized with respect to ocular anomalies. Variability between eyes of the same patient and among different patients was marked. In addition to the well-known but inconstant displacement of the lens and pupil, other characteristics of this protean syndrome included severe axial myopia (and associated fundus abnormalities), poor vision, retinal detachment (RD), enlarged corneal diameters, cataract, abnormal iris transillumination, poor pupillary dilatation, persistent pupillary membranes, iridohyaloid adhesions, and prominent iris processes in the anterior chamber angle.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
6/8. Aphakic correction in ectopia lentis.ectopia lentis may cause a reduction in visual acuity, which varies with the type and degree of dislocation and the presence of other ocular abnormalities. retinoscopy may reveal a significant refractive error, usually myopia and astigmatism. Occasionally, an accurate refraction may be difficult because of tilting or dislocation of the lens. If aphakic refraction improves visual acuity, an aphakic correction should be considered.- - - - - - - - - - ranking = 0.5keywords = myopia (Clic here for more details about this article) |
7/8. Dominantly inherited blepharoptosis, high myopia, and ectopia lentis.A previously undescribed syndrome of bilateral blepharoptosis, ectopia lentis, and high myopia apparently was dominantly inherited and originated from a spontaneous genetic mutation. The syndrome seems to be caused by a decrease in the tensile strength of the levator aponeurosis, the zonules, and the sclera.- - - - - - - - - - ranking = 2.5keywords = myopia (Clic here for more details about this article) |
8/8. ectopia lentis, chorioretinal dystrophy and myopia. A new autosomal recessive syndrome.Four of five siblings born of a consanguineous marriage demonstrated abnormalities of the lens (ectopia lentis and dense cataracts) and retina (generalized tapetoretinal dystrophy and total retinal detachment) associated with myopia. This association in an autosomal recessive pedigree has not been previously noted.- - - - - - - - - - ranking = 2.5keywords = myopia (Clic here for more details about this article) |