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Ectopia Lentis


Presentation

  • 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]
  • Ten Pakistani patients presenting with decreased vision and ectopia lentis with concomitant homocystinuria were included in the study. Assessment of systemic and ophthalmic features was performed. All patients presented with visual deterioration.[ncbi.nlm.nih.gov]
  • The findings presented in this paper validate the necessity of FBN1 gene testing in all individuals presenting with isolated EL. As we observed, these individuals are at increased risk of cardiovascular complications.[ncbi.nlm.nih.gov]
  • All patients developed bilateral cataract before the age of 40 years, and two patients presented with intermittent acute intraocular hypertensive crises.[ncbi.nlm.nih.gov]
  • A family from Turkey with congenital ectopia lentis, likely occurring as an autosomal recessive trait, is presented. No systemic disorders such as homocysteinuria, Marfan's or Weill-Marchesani's syndromes were found in any of the patients.[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]
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]
  • 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]
Ectopia Lentis
  • In the absence of trauma, ectopia lentis should evoke suspicion for concomitant hereditary systemic disease or associated ocular disorders. [4, 5] Ectopia lentis. Dislocated traumatic lens (cataract). Ectopia lentis.[emedicine.com]
  • This is the largest family with isolated ectopia lentis reported to date. The results of the present study provide convincing evidence for a correlation of R240C and isolated ectopia lentis.[ncbi.nlm.nih.gov]
  • Ectopia lentis and homocystinuria appear to have a strong correlation in Pakistani population.[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]
  • 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]
  • L21) See corectopia ; iridodonesis ; pupillary block . Fig.[medical-dictionary.thefreedictionary.com]
  • 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.[slideshare.net]

Workup

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

Treatment

  • Prophylactic lensectomy has proven to be the most reliable treatment in our experience.[ncbi.nlm.nih.gov]
  • CONCLUSION: Transscleral intraocular lens fixation with preservation of the anterior vitreous face is a safe and effective technique for treatment of ectopia lentis in patients with Marfan syndrome.[ncbi.nlm.nih.gov]
  • Nevertheless, the described surgical techniques may be considered an effective method of treatment in cases of ectopia lentis et pupillae syndrome.[ncbi.nlm.nih.gov]
  • Visual rehabilitation and treatment of amblyopia are essential for patients within the amblyogenic age group following surgical intervention. Copyright 2015, SLACK Incorporated.[ncbi.nlm.nih.gov]
  • The importance of early diagnosis and treatment is discussed.[ncbi.nlm.nih.gov]

Prognosis

  • 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]

Etiology

  • 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]
  • MeSH terms, Substances MeSH terms ADAMTS Proteins Aphakia, Postcataract/etiology Aphakia, Postcataract/therapy Child, Preschool Codon, Nonsense* Consanguinity DNA Mutational Analysis Ectopia Lentis/genetics* Eyeglasses Functional Laterality Genes, Recessive[ncbi.nlm.nih.gov]

Epidemiology

  • 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]
  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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]

Prevention

  • 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]
  • Recognition of this clinical entity can expedite diagnosis and prevent irreversible loss of vision secondary to glaucoma.[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]
  • Prevention and Treatment If underlying eye conditions, such as cataracts cause ectopia lentis, those conditions should be treated first. Genetically inherited or trauma caused ectopia lentis is most commonly treated with surgery or laser correction.[study.com]

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