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]

• 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]

• Nightmare

  Agarwal is the author of several books published by SLACK Incorporated, publisher of Ocular Surgery News, including Phaco Nightmares: Conquering Cataract Catastrophes, Bimanual Phaco: Mastering the Phakonit/MICS Technique, Dry Eye: A Practical Guide to [healio.com]

• Long Hands

  The characteristics are : tall, slim built stature ectopia lentis, arachnodactyly with long hands and feet, congenital heart defects, high arched palate, spinal deformities, generalised muscular under-development and mental retardation. [ijo.in]

• Thin Extremities

  Systemic examination revealed a height of 160 cm, with long and thin extremities, a larger arm span than height (arm span / height ratio >1.05) and reduced upper segment / lower segment ratio ( An automated keratometry showed normal corneal curvatures [egms.de]

• 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

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

  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]

  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]

  In the setting of lens dislocation, the patient may have trembling or shimmering of either the lens or iris, phakodenesis or iridodonesis, respectively. [tamingthesru.com]

• 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]

  Symptoms of ectopia lentis include monocular diplopia and visual distortion. Symptoms vary based on the extent of displacement, but complete displacement may result in marked visual blurring. [tamingthesru.com]

  Ocular: blurred vision, monocular diplopia, pain, flashes and floaters. [webeye.ophth.uiowa.edu]

• Focal Neurological Deficit

  Neurologically, eye movements were normal. No focal neurological deficits were evident. Other systemic examination was normal. Uncorrected visual acuity was counting finger close to face bilaterally. [annalsofian.org]

• Generalized Tonic-Clonic Seizure

  Her initial generalized tonic clonic seizures later evolved as brief tonic right upper limb posturing suggestive of mesial frontal originating seizures. Magnetic resonance imaging (MRI) brain revealed BFP [Figure 1] a-c. [annalsofian.org]

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

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

That Kid's Bag: Treatment of Subluxated Crystalline Lens This award-winning video by Mauricio A. [eyewiki.aao.org]

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]

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]

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]

Beta Blockers These medications, taken by mouth, lower blood pressure, which can prevent or slow the enlargement of the aorta. [nyulangone.org]

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