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Objectives This study aimed to identify the etiology and the direction of dislocation of the natural crystalline lens or intraocular lens (IOL) in IOL intrascleral fixation surgery and to determine the change in intraocular pressure (IOP) after surgery. Methods We retrospectively investigated the diagnosis, direction of lens and IOL dislocation, and IOP before and after surgery (preoperatively and one day, one week, and one month postoperatively) in 236 eyes from 228 patients who underwent IOL intrascleral fixation at Chiba University Hospital between February 2015 and September 2020. Results IOL intrascleral fixation was performed in 48 (20.

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The zonular fibres are formed primarily of fibrillin-1, a large extracellular matrix (ECM) glycoprotein, and also contain other constituents such as LTBP-2, ADAMTSL6, MFAP-2 and EMILIN-1, amongst others. They are critical for sight, holding the crystalline lens in place and being necessary for accommodation. Zonulopathies refer to conditions in which there is a lack or disruption of zonular support to the lens and may clinically be manifested as ectopia lens (EL)-defined as subluxation of the lens outside of the pupillary plane or frank displacement (dislocation) into the vitreous or anterior segment.

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: We report the successful surgical treatment of a case of spontaneous complete anterior crystalline lens luxation in a patient affected by retinitis pigmentosa (RP), associated with elevated intraocular pressure and pupillary block. Additionally, we review the current literature regarding the association between ectopia lentis and RP. : A 44-year-old female RP patient presented to our emergency department reporting severe ocular pain in her left eye (LE) and sickness.

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To our knowledge, this is the first report of a patient with both genetically confirmed Kabuki and Marfan syndrome demonstrating a perifoveal macular degeneration in one eye. Progressive loss of the outer retinal layers was captured and demonstrated with spectral-domain optical coherence tomography imaging. Fundus autofluorescence imaging revealed perifoveal hypoautofluorescence.

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Genotype Associated With Visual Prognosis in Patients With Congenital Ectopia Lentis Following Lens Surgery: A Prospective Cohort Study.

Am J Ophthalmol

December 2024

From the Eye Institute and Department of Ophthalmology (W.N.J., Z.X.C., Y.L.W., X.S., X.Y.C., T.H.C., Y.S., Y.L., L.H.S., Q.Y.H., and Y.X.J.), Eye & ENT Hospital, Fudan University, Shanghai China; NHC Key Laboratory of Myopia (Fudan University), Key Laboratory of Myopia (W.N.J., Z.X.C., Y.L.W., X.S., X.Y.C., T.H.C., Y.S., Y.L., L.H.S., Q.Y.H., and Y.X.J.), Chinese Academy of Medical Sciences, Shanghai, China; Shanghai Key Laboratory of Visual Impairment and Restoration (W.N.J., Z.X.C., Y.L.W., X.S., X.Y.C., T.H.C., Y.S., Y.L., L.H.S., Q.Y.H., and Y.X.J.), Shanghai, China. Electronic address:

Purpose: To investigate the relationship between visual prognosis and genotype in patients undergoing lens surgery for congenital ectopia lentis (EL).

Design: Prospective clinical cohort study.

Methods: Patients with congenital EL who underwent lens removal and intraocular lens implantation received panel-based next-generation sequencing.

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