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Cataracts in Paediatric patients. (With acknowledgements to the Online Journal of Ophthalmology: www.onjoph.com). Introduction. Opacity in lens Can be: Visually significant or not Stable or Progressive Congenital or Acquired
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Cataracts in Paediatric patients (With acknowledgements to the Online Journal of Ophthalmology: www.onjoph.com)
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
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
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%
Congenital cataracts: Unilateral • Sporadic, no family history • Ocular anomalies : Persistent foetal vasculature • Cause identified in only 10%
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)
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
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
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
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
Postoperative considerations • Clear vision for distance and near • Intraocular lens: regular refraction • Spectacles • Contact lenses • Treatment of amblyopia : Occlusion therapy
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