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      Pediatric cataract: challenges and future directions

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          Abstract

          Cataract is a significant cause of visual disability in the pediatric population worldwide and can significantly impact the neurobiological development of a child. Early diagnosis and prompt surgical intervention is critical to prevent irreversible amblyopia. Thorough ocular evaluation, including the onset, duration, and morphology of a cataract, is essential to determine the timing for surgical intervention. Detailed assessment of the general health of the child, preferably in conjunction with a pediatrician, is helpful to rule out any associated systemic condition. Although pediatric cataracts have a diverse etiology, with the majority being idiopathic, genetic counseling and molecular testing should be undertaken with the help of a genetic counselor and/or geneticist in cases of hereditary cataracts. Advancement in surgical techniques and methods of optical rehabilitation has substantially improved the functional and anatomic outcomes of pediatric cataract surgeries in recent years. However, the phenomenon of refractive growth and the process of emmetropization have continued to puzzle pediatric ophthalmologists and highlight the need for future prospective studies. Posterior capsule opacification and secondary glaucoma are still the major postoperative complications necessitating long-term surveillance in children undergoing cataract surgery early in life. Successful management of pediatric cataracts depends on individualized care and experienced teamwork. We reviewed the etiology, preoperative evaluation including biometry, choice of intraocular lens, surgical techniques, and recent developments in the field of childhood cataract.

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          Most cited references139

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          Congenital cataracts and their molecular genetics.

          Cataract can be defined as any opacity of the crystalline lens. Congenital cataract is particularly serious because it has the potential for inhibiting visual development, resulting in permanent blindness. Inherited cataracts represent a major contribution to congenital cataracts, especially in developed countries. While cataract represents a common end stage of mutations in a potentially large number of genes acting through varied mechanisms in practice most inherited cataracts have been associated with a subgroup of genes encoding proteins of particular importance for the maintenance of lens transparency and homeostasis. The increasing availability of more detailed information about these proteins and their functions and is making it possible to understand the pathophysiology of cataracts and the biology of the lens in general.
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            Refractive development of the human eye.

            A complete refractive investigation was performed on 148 normal eyes of 79 patients ranging from premature newborns to 36-year-old adults. Cycloplegic refraction, keratometry, and axial length measurements were performed. From these data, we then calculated the refractive power of the lens. The change with respect to age in these measurements was subjected to cross-sectional analysis. The full-term newborn eye had a mean axial length of 16.8 mm, a mean keratometric power of 51.2 diopters (spherical equivalent), and a mean lens power of 34.4 D. The adult values for these measurements were 23.6 mm, 43.5 D, and 18.8 D, respectively. This information concerning the expected change with age in the refractive components should aid in the refractive management of pediatric patients requiring cataract extraction.
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              Ocular manifestations of Fabry's disease: data from the Fabry Outcome Survey.

              Fabry's disease is an X-linked lysosomal storage disorder characterised by deficient activity of the enzyme alpha-galactosidase A. To study eye abnormalities in patients with Fabry's disease in the Fabry Outcome Survey (FOS). This is the largest study of ocular manifestations in patients with Fabry's disease. In all, 173 of the 688 patients enrolled in FOS underwent a detailed ophthalmic examination, with a special focus on abnormalities of the cornea, lens, conjunctival and retinal vessels. Cornea verticillata was reported in 76.9% of females and 73.1% of males; vessel tortuosity was observed in 21.9% of females and in 48.7% of males. Fabry cataract was recorded in 9.8% of females and in 23.1% of males. Cornea verticillata was therefore the most frequently reported ophthalmic abnormality in Fabry's disease in both hemizygotic males and heterozygotic females. Tortuous conjunctival and retinal vessels and Fabry cataract were more frequently found in males than in females. Vessel tortuosity was more frequently observed in patients with a higher severity score and greater impairment of renal and cardiac function, suggesting that it may be associated with a more severe disease. The youngest patient with ocular changes was 3 years old. The prevalence of tortuous vessels in males increased with age. The presence of cornea verticillata is a useful aid in the diagnosis of Fabry's disease, as it is often present at the time of diagnosis. Vessel tortuosity may have some predictive value for systemic involvement.
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                Author and article information

                Journal
                Clin Ophthalmol
                Clin Ophthalmol
                Clinical Ophthalmology
                Clinical Ophthalmology (Auckland, N.Z.)
                Dove Medical Press
                1177-5467
                1177-5483
                2015
                07 January 2015
                : 9
                : 77-90
                Affiliations
                [1 ]Pediatric Ophthalmology, Strabismus, and Adult Motility, Children’s Hospital of Pittsburgh of University of Pittsburgh Medical Center, (UPMC), Pittsburgh, PA, USA
                [2 ]University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
                Author notes
                Correspondence: Ken K Nischal, Pediatric Ophthalmology, Strabismus, and Adult Motility, Children’s Hospital of Pittsburgh of University of Pittsburgh Medical Center, (UPMC) 4401 Penn Avenue – Suite 5000, Pittsburgh, PA 15224, USA, Tel +1 412 692 5918, Fax +1 412 692 3203, Email nischalkk@ 123456upmc.edu
                Article
                opth-9-077
                10.2147/OPTH.S59009
                4293928
                25609909
                6c3c3fc7-6cfa-462c-8325-891a7f46a6c2
                © 2015 Medsinge and Nischal. This work is published by Dove Medical Press Limited, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License

                The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

                History
                Categories
                Review

                Ophthalmology & Optometry
                children,pediatric cataract,infantile cataract,aphakia,pseudophakia
                Ophthalmology & Optometry
                children, pediatric cataract, infantile cataract, aphakia, pseudophakia

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