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]

Conclusion Ectopia lentis is classified as syndromic when it is present with syndromes. This condition affects the positioning of the lens. [icliniq.com]

Methods: Case 1- Fifty year old woman presented with sudden painless diminution of vision both eye since 1 day with history of blunt trauma by wood-log. [djo.org.in]

Clinical description Patients with IEL are found to have dislocation of the lens, which may present at any age, but may be present from birth. Dislocation of the lens can be very mild leading to late diagnosis. [orpha.net]

• Prognathism

  The score calculated on the basis of revised Ghent criteria’s systemic score was =7 (wrist and thumb sign, hind-foot deformity, reduced elbow extension and facial features including malar hypoplasia, dolichocephaly, prognathism, high-arched palate) which [djo.org.in]

• Blonde Hair

  It is often characterized by mental handicap, osteoporosis, chest abnormalities, atherosclerosis, and increased incidence of thrombosis.[15] The other features include blue iris, coarse blonde hair, marfanoid habitus, and malar flush. [statpearls.com]

• Dolichocephaly

  The score calculated on the basis of revised Ghent criteria’s systemic score was =7 (wrist and thumb sign, hind-foot deformity, reduced elbow extension and facial features including malar hypoplasia, dolichocephaly, prognathism, high-arched palate) which [djo.org.in]

• Iridodonesis

  Slitlamp biomicroscopy revealed that the corneas and anterior chambers were normal but iridodonesis associated with ectopia lentis was observed in both eyes. [jamanetwork.com]

  Other findings include congenital abnormalities of the iris, spherophakia, enlarged iris processes leading to abnormal iridocorneal angle, iridodonesis, lens coloboma, refractive errors (hyperopia, myopia, astigmatism), and early-onset cataract. [orpha.net]

  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]

• 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]

  Ocular: blurred vision, monocular diplopia, pain, flashes and floaters. [eyerounds.org]

  Double vision (diplopia). Dizziness. Cataract (clouding of the lens). Tearing of the lining of the eye. Glaucoma (a group of eye disorders causing vision loss by affecting the optic nerve). Retinal detachment can occur. [icliniq.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]

• Retinal Damage

  Additionally, ectopic lentis can cause major complications within the eye, including significant refractive shifts, pupillary block glaucoma, retinal damage, and blindness. [retinavitreous.com]

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

Treatment of the resulting aphakia may be with contact lenses. [orpha.net]

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

Treatment In some cases in which the dislocation is minimal and there are no significant visual changes, so no treatment is necessary. If the vision is affected, surgery will be necessary. [bceye.com]

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

Prognosis Visual prognosis depends on the degree of lens dislocation, the age of onset, age of treatment and complications. The documents contained in this web site are presented for information purposes only. [orpha.net]

[…] complete luxation the lens assumes a dependent position in the vitreous demonstrates free mobility with eye movements Associated findings include: traumatic cataract increased echogenicity of the lens is suggestive vitreous hemorrhage Treatment and prognosis [radiopaedia.org]

Prognosis Most patients do well and at least 85% have 20/40 or better visual acuity after treatment. [bceye.com]

They concluded that both are viable options to safeguard vision in pediatric aphakia cases secondary to ectopia lentis.[39] Prognosis The patient's prognosis depends on the presenting visual acuity, the extent of ocular and systemic involvement, degree [statpearls.com]

The commonest atraumatic etiologies are Marfan syndrome and homocystinuria. [radiopaedia.org]

As ectopia lentis may be the first sign of a more serious systemic disease, it is important to determine the etiology for appropriate patient management. [eyewiki.aao.org]

Summary Epidemiology The prevalence of IEL is not known. About 90 cases have been reported to date, primarily in Europeans. [orpha.net]

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.medscape.com]

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.medscape.com]

When caused by a mutation in the ADAMTSL4 gene, IEL is inherited in an autosomal recessive manner.[11236][11240] The primary goal of treatment is preventing amblyopia (lazy eye) through early correction of astigmatism. [rarediseases.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]

There should be multifaceted approach to manage such cases and prevent sight-threatening complication. [djo.org.in]