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Cataracts in Paediatric patients
(With acknowledgements to the Online Journal of Ophthalmology:
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Introduction Opacity in lens Can be: Visually significant or not
Stable or Progressive Congenital or Acquired Unilateral or Bilateral Partial or Complete Congenital: incidence 6/10 000 10% of childhood blindness
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Classification : Acquired cataracts
Systemic diseases : Diabetes mellitus : Myotonic dystrophy : Atopic dermatitis : Neurofibromatosis 2 Ocular diseases : Chronic anterior uveitis : High myopia : Fundus dystrophies eg Retinitis pigmentosa Drugs : Corticosteroids : Chlorpromazine Trauma : Blunt : Sharp
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Congenital cataracts: Bilateral
Genetic Mutation : Autosomal Dominant Metabolic : Galactosaemia : Lowe : Hypoparathyroidism : Fabry Infective : TORCH organisms Chromosomal : Trisomy 21 (Down) : Trisomy 18 (Edward) : Trisomy 13 (Patau) Skeletal : Hallerman-Streiff : Nance-Horan Ocular anomalies : Aniridia : Anterior segment dysgenesis syndrome Idiopathic : in 50%
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Congenital cataracts: Unilateral
Sporadic, no family history Ocular anomalies : Persistent foetal vasculature Cause identified in only 10%
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Morphology : Examples
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Evaluation Screen newborns with red reflex test History : Family
Maternal infections Examination: systemic diseases or syndromes Workup: Bilateral cases without known hereditary basis TORCH screen s-glucose s-calcium, phosphate Urine: reducing substances (galactosaemia) amino acids ( Lowe syndrome) haematuria (Alport syndrome)
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Ocular examination Formal estimate of vision not possible in neonate
Special tests: Preferential looking test, visually evoked potentials Density and position of cataract Morphology Associated ocular pathology Indicators of severe visual impairment : No fixation Nystagmus Strabismus
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The visually significant cataract
In central visual axis, bigger than 3mm Posterior cataract No clear zones in between Retinal details not visible with direct ophthalmoscope Nystagmus or strabismus present Poor central fixation after 8 weeks
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Treatment Surgery: Cataract extraction and intraocular lens
implantation for visually significant cataract By 6 weeks of age Bilateral cases: 1 week apart Non visually significant cases : careful observation, possible pupillary dilation
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Considerations regarding surgery
Intraocular lens : Power of lens – Myopic shift of the growing eye Surgical technique Postoperative intraocular inflammation (uveitis) Glaucoma and retinal detachment may develop
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Pseudophakic eye
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Postoperative considerations
Clear vision for distance and near Intraocular lens: regular refraction Spectacles Contact lenses Treatment of amblyopia : Occlusion therapy
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Conclusion: Congenital cataracts
Correct management essential to prevent permanent visual loss Team effort ophthalmologist, paediatrician, geneticist,family Early detection within the first month of life is very important Knowledge of systemic conditions associated with cataract
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