Symptoma

Ectopia Lentis

To date, there has been no report of a child with ectopia lentis as the only presentation of an intraocular tumor. We present 2 children with malignant medulloepitheliomas who presented in this fashion. [ncbi.nlm.nih.gov]

  • Dysostosis

    […] common: Marfan syndrome (upward and outward) Homocystinuria (downward and inwards) Weill–Marchesani syndrome Sulfite oxidase deficiency HyperlysinemiaLess common: Ehlers–Danlos syndrome Crouzon disease Refsum syndrome Kniest syndrome Mandibulofacial dysostosis [en.wikipedia.org]

    Other systemic diseases associated with ectopia lentis: Weill-Marchesani syndrome Sulfite oxidase deficiency Hyperlysinemia Ehlers-Danlos syndrome Sturge-Weber syndrome Mandibulofacial dysostosis Wildervanck syndrome Conradi syndrome Pfaundler syndrome [retinavitreous.com]

    Rare Systemic Associations  Ehlers-Danlos syndrome  Crouzon disease  Refsum syndrome  Kniest syndrome  Mandibulofacial dysostosis  Sturge-Weber syndrome 19. [es.slideshare.net]

    […] cases occurs in the inferior or nasal direction. [12] [13] Other systemic diseases associated with ectopia lentis [5] [6] : Weill-Marchesani syndrome Sulfite oxidase deficiency Hyperlysinemia Ehlers-Danlos syndrome Sturge-Weber syndrome Mandibulofacial dysostosis [eyewiki.aao.org]

  • Atrial Septal Defect

    The other cousin had an atrial septal defect, mitral valve prolapse, and only mild myopia. Their intelligence is normal. [ncbi.nlm.nih.gov]

  • Aspiration

    Sodium hyaluronate (Healon GV) enhanced stability of the crystalline lens during aspiration and maintenance of the anterior chamber during passage of the needles through the ciliary sulcus. [ncbi.nlm.nih.gov]

    Vajpayee Intralenticular bimanual irrigation: aspiration for subluxated lens in Marfan's syndrome [33] I. Anteby, M. Isaac, D. BenEzra Hereditary subluxated lenses: visual performances and long-term follow-up after surgery [34] M. Halpert, D. [elsevier.es]

    […] cutting or aspiration. [springerplus.springeropen.com]

    A study of five cases of EL in Saudi Arabia suggested better visual outcomes compared to eyes with acquired EL managed by lens aspiration. [10] In Oman, ten out of twenty eyes with subluxated lens managed by pars plana scleral lensectomy and scleral-fixated [meajo.org]

  • Ear Deformity

    Congenital contractural arachnodactyly (CCA) was described by Beals and Hecht as an autosomal dominant disorder distinct from Marfan syndrome and comprising joint contractures, arachnodactyly, scoliosis, and a distinct "crumpled ear" deformity. [ncbi.nlm.nih.gov]

  • Iridodonesis

    If the eye is not dilated, look for iridodonesis (abnormal tremor of the iris), phacodonesis (abnormal movement of the lens), deep anterior chamber depth or vitreous herniation into the anterior chamber. [mrcophth.com]

    Trembling of the iris (iridodonesis) Trembling of the lens (phacodonesis) Aphakic crescent Mild conjunctival redness Vitreous humour degeneration Prolapse of the vitreous into the anterior chamber Increase or decrease of anterior chamber depth Removal [premilife.com]

    L21) See corectopia ; iridodonesis ; pupillary block. Fig. [medical-dictionary.thefreedictionary.com]

    Megalocornea, With Ectopia Lentis And With Or Without Secondary Glaucoma Synonyms - Classification developmental, eye, genetic Phenotypes Autosomal recessive inheritance ; Deep anterior chamber ; Ectopia lentis ; Glaucoma ; High palate ; Hypermetropia ; Iridodonesis [mousephenotype.org]

    It can be recognized by trembling of the iris (iridodonesis) or lens (phacodonesis) and the presence of an aphakic crescent (an area of the pupil where the lens is absent). [en.wikipedia.org]

  • Diplopia

    Subluxation is one of the causes of monocular diplopia. If the luxation is complete the eye becomes markedly hyperopic and is unable to accommodate. [medical-dictionary.thefreedictionary.com]

    While most clinicians immediately think of neurological problems when diplopia is mentioned, monocular diplopia is always attributed to some type of media issue, such as a cataract, corneal opacity or subluxated lens. [reviewofoptometry.com]

    Patients may also complain of monocular diplopia and are at risk of developing amblyopia, glaucoma, and retinal detachment. Ectopia lentis can be inherited as an isolated condition, usually autosomal dominant (simple ectopia lentis). [visualdx.com]

    Presentation  Common presenting symptoms include the following:  Decreased distance visual acuity (secondary to astigmatism or lenticular myopia)  Poor near vision (loss of accommodative power)  Monocular diplopia  Red painful eye 22. [es.slideshare.net]

Workup  Cardiac evaluation for Marfan syndrome  Serum and urine levels of homocysteine or methionine for homocystinuria  Axial length measurement 32. [es.slideshare.net]

Prophylactic lensectomy has proven to be the most reliable treatment in our experience. [ncbi.nlm.nih.gov]

Treatment and prognosis Complications An anterior dislocation/subluxation carries greater complications than a posterior one. Such complications include: acute glaucoma corneal injury iridal injury Promoted articles (advertising) [radiopaedia.org]

Better prognosis is valued in lens replacement surgery (retained vision and normal intraocular pressure) when it is performed before the onset of secondary glaucoma. [en.wikipedia.org]

Prognosis Depending on the degree of lens dislocation, the age of onset, and its associated secondary complications, most patients do well. [emedicine.com]

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. [ncbi.nlm.nih.gov]

Mortality/Morbidity Ectopia lentis may cause marked visual disturbance, which varies with the degree of lens displacement and the underlying etiologic abnormality. [emedicine.com]

Epidemiology Frequency United States Ectopia lentis is a rare condition. Incidence in the general population is unknown. The most common cause of ectopia lentis is trauma, which accounts for nearly one half of all cases of lens dislocation. [emedicine.com]

Keywords: anterior segment • clinical (human) or epidemiologic studies: pre • linkage analysis 2003, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. [iovs.arvojournals.org]

Pathophysiology Disruption or dysfunction of the zonular fibers of the lens, regardless of cause (trauma or heritable condition), is the underlying pathophysiology of ectopia lentis. [emedicine.com]

Removal of the lens before it completely luxates into the anterior chamber may prevent secondary glaucoma. A nonsurgical alternative involves the use of a miotic to constrict the pupil and prevent the lens from luxating into the anterior chamber. [en.wikipedia.org]

These findings should encourage earlier surgery when indicated to prevent amblyopia and improve visual acuity. [ncbi.nlm.nih.gov]

A nonsurgical alternative involves the use of a miotic to constrict the pupil and prevent the lens from luxating into the anterior chamber. [premilife.com]

In subsequent operations this complication was prevented by instilling miotics prior to surgery. [ijo.in]

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