Congenital cataract
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Commonest cause of leucocoria in infants
- leucocoria = white pupil reflex due to light scatter; loss of normal red reflex​
- although congenital cataract is the commonest cause of leucocoria, retinoblastoma is the most important differential for leucocoria to rule out
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May be associated with ocular or systemic disorders, including:​
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Down’s syndrome
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Rubella
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Toxoplasmosis
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Cytomegalovirus
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Herpes simplex
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Roughly half of bilateral congenital cataracts are hereditary
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Other causes include toxins, drugs and radiation
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Impact on vision is variable
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Present at birth
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Test is direct ophthalmoscopy
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Affects 1 in 4000 live births
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High risk of irreversible amblyopia if not treated early
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can be severe and treatment may be challenging
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If leucocoria detected on ophthalmology, requires urgent referral to ophthalmology to investigate cause
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if found to have congenital cataract, will require surgery
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surgery involves removal of cataractous lens​
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child will require contact lens following surgery to correct refractive error of aphakic eye (one without a lens)
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risks of surgery include glaucoma
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References
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​Denniston, A. K. O. and Murray, P. I. (eds) (2018) Oxford handbook of ophthalmology. 4th edn. London, England: Oxford University Press (Oxford Medical Handbooks). doi: 10.1093/med/9780198804550.001.0001.
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James, B., Bron, A. J. and Parulekar, M. V. (2016) Lecture Notes Ophthalmology. 12th edn. Nashville, TN: John Wiley & Sons (Lecture Notes)