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SYMPTOMS
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Typically asymptomatic
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Can lead to a Decrease in vision (degree of vision loss depends on severity and location of the congenital cataract), Glare, Halos, Photophobia, Monocular diplopia
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SIGNS
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Anterior polar cataract: Focal opacity involving the anterior capsule
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Posterior polar cataract: Focal opacity involving the posterior capsule
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Lamellar cataract: Round opacity that involves the cortex
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Fetal nuclear cataract: Focal opacity involving the embryonic nucleus
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Anterior sutural cataract: Opacity involving the Y shaped suture
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Posterior sutural cataract: Opacity involving the inverted Y shaped suture
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Coronary cataract: Radial, club shaped opacities involving the cortex
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Blue dot cataract: Multiple bluish opacities involving the cortex
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Pulverulant cataract: Punctate opacities involving the embryonic nucleus
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Total congenital cataract: Dense white nuclear opacity
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Oil droplet cataract: Nucleus opacity that increases the refractive index and gives an “oil droplet” shape with retroillumination
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Polychromatic cataract (Christmas tree cataract): Reflective, iridescent crystalline deposits in the posterior aspect of the lens
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Congenital cataracts may be associated with a Microcornea, Megalocornea, Iris coloboma, Aniridia
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If congenital cataract is visually significant, it can cause a Sensory nystagmus, Strabismus, Amblyopia, Leukocoria, Absent red reflex
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WORK-UP
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Pupils, EOMs, Cover test, Full eye exam with dilation, OKN drum or preferential looking test (depends on age of the patient), B-scan ultrasound (if cataract is too dense to visualize the back of the eye), Potential acuity testing
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TREATMENT
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No treatment is needed if the congenital cataract is not visually significant
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Refer to a pediatric ophthalmologist for cataract surgery if the congenital cataract is visually significant
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Refer to a pediatrician to evaluate and treat any suspected underlying systemic condition associated with the patient's congenital cataract
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FOLLOW-UP
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See patient back in 12 months in order to monitor congenital cataract (may need to monitor patient more closely if there are other ocular findings associated with the congenital cataract)
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Once the congenital cataract is treated by the pediatric ophthalmologist and the eye is stable, the patient should be seen back in 12 months (may need to monitor patient more closely if there are other ocular findings associated with the congenital cataract)
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ADDITIONAL LAB | TESTS
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If suspecting an associated systemic disease, the following should be ordered: CBC with differential, TORCH titers, VDRL, Red cell galactokinase, Serum calcium and phosphorus, Urine analysis
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ETIOLOGY
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Most common etiology is idiopathic but congenital cataracts can also develop due to due to genetic mutations and systemic diseases
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DIFFERENTIAL DX
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Other causes of leukocoria which include Retinoblastoma, Toxocariasis, Coats disease, Retinopathy of prematurity
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NOTES
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Coronary cataracts can be associated with Down syndrome
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Total congenital cataracts are associated with TORCH syndromes with the most common being rubella
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Oil droplet cataracts are associated with galactosemia
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Polychromatic cataracts are associated with myotonic dystrophy
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Congenital cataracts can also be associated with Alport syndrome, Fabry disease, and Turner syndrome
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