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Key Features

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  • May be unilateral or bilateral

  • May exist as isolated defects

  • May be accompanied by other ocular disorders or systemic disease

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Clinical Findings

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  • Leukocoria, poor fixation, and strabismus or nystagmus may be the presenting complaints

  • Absence of a red reflex in the newborn may be due to a cataract, which requires an urgent referral to an ophthalmologist

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Diagnosis

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  • Laboratory investigation for infectious, genetic and metabolic causes of bilateral congenital cataracts is often indicated

  • Such investigation would include cultures or serologic tests for

    • Toxoplasmosis

    • Rubella

    • Cytomegalovirus

    • Herpes simplex virus

    • Syphilis

    • Inborn metabolic errors, such as galactosemia or Lowe syndrome

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Treatment

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  • Early diagnosis and treatment are necessary to prevent deprivation amblyopia in children younger than 9 years because they are visually immature

  • Cataracts that are visually significant require removal

  • Visually significant cataracts in infants are usually removed prior to 6 weeks of age to prevent deprivation amblyopia

  • Rehabilitation of the vision will require the correction of refractive errors and amblyopia treatment

  • Contact lenses, glasses, and artificial intraocular lenses are used to correct refractive errors after cataract extraction

  • Concomitant treatment of associated amblyopia, glaucoma, and the underlying systemic disease as indicated

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