approach and management of congenital cataract
DESCRIPTION
This to teach about approach and adequate management of the congenital cataract. This presentation highlights the common casues of the congenital cataract. Early treatment prevents amblyopia. Delayed causes loss of vision.TRANSCRIPT
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20/11/2012
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R, 6mth, Mch
R/O Hamirpur
Himachal pradesh
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Whitish eyes since birth
Not properly following to light
Poor response to sounds (Noted subsequently)
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Delivered vaginally at term at home, cried after
birth
Antenatal period not supervised but uneventful
Peri – postnatal period uneventful
Breastfed exclusively
Immunization started at ~6 wk of age
No significant family history
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Vitals stable
Lt – 64 cm (~50th centile)
Wt - 5.5 kg ( <3rd centile)
OFC - 42cm ( > 15th centile - < 50th centile) Eyes
B/L aphakia No proper fixation Follows light Nystagmus b/l
No obvious dysmorphism
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Abdomen- soft, no organomegaly
CVS - S1S2 normal, no murmur Respiratory system - normal
CNS
Alert, consolable
Poor response to sound
Tone & reflexes normal
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5 mth, M
B/L aphakia
Impaired hearing
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Congenital cataract
Retinoblastoma
Retinopathy of prematurity
Persistent hyperplastic primary vitreous
•EUA•Dilated pupils•Direct ophthalmoscopy•USG of eye and orbit
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Post operative case of bilateral congenital cataract with
impaired hearing under evaluation
Cause of impaired hearing ?
Rubella
Retinitis pigmentosa
Craniofacial dysplasia
Alports syndrome
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Opacity in lens
Can be:
Congenital or Acquired
Unilateral or Bilateral
Partial or Complete
Congenital: 6/10,000 (10% of childhood blindness)
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Maternal Factors Malnutrition Infection
TORCHS (50% cases with rubella has cataract at birth)
Measles Varicella Zoster Influenza
Drugs Corticosteroids, Thalidomide
Radiation
Fetal / Infantile Factors Fetal malnutrition Deficient oxygenation Metabolic disorder
Galactosemia Neonatal hypoglycemia
Associated with other congenital anomalies Lowe’s syndrome Myotonic dystrophy
Birth trauma
Hereditary / Genetic: ~1/3rd of cases
Idiopathic: ~50% of cases
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Systemic Diseases
Diabetes mellitus Atopic dermatitis Neurofibromatosis 2
Ocular Diseases
Chronic anterior uveitis High myopia Retinitis pigmentosa
Drugs
•Chlorpromazine
Trauma
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•Sporadic, no family history
•Ocular anomalies : Persistent foetal vasculature
•Cause identified in only 10%
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Disturbances in normal growth of lens
Opacity of lens at various locations
White pupillary reflex
Impaired refraction of lens
Focusing of image elsewhere than retina
Amblyopia
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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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Screening of newborns with red reflex test
History
Family
Maternal infections
Maternal drugs
Maternal radiation exposure
Examination of newborn / infant / child
Associated systemic diseases or syndromes
TORCHS screen
Index case
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Metabolic
Blood glucose (Hyperglycemia)
Serum calcium, phosphate, X-ray skull (Hypoparathyroidism)
Urine- reducing substances (Galactosaemia)
Haematuria (Alport syndrome)
Urine chromatography for amino acids ( Lowe syndrome)
Detail ophthalmologic evaluation
EUA, ophthalmoscopy with dilated pupil, USG
Index case hearing
assessment planned
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Visually significant cataract
Cataract extraction and
IOL implantation
Non visually significant cases
Careful observation,
Possible pupillary dilation
Index case:Cataract extracted B/L Spectacles – prescribed
IOL implantation planned after 2 yrs of
age
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Bilateral dense cataracts
Within six weeks of birth
1 week apart
Unilateral dense cataracts
ASAP (within days)
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In 1st yr axial length increases very fast
It causes myopic shift of growing eye
Goal
Undercorrect the refraction
20% in neonate and 10% in toddlers
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Retrobulbar
hemorrage
Oculocardiac reflex
Muscle lacerations
Corneal / uveal injury
Vitreous loss
Nucleous drops into
vitreous cavity
Hyphaema
Cystoid macular edema
Endopthalmitis
After cataract
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6wk 4mth 5mth 6mth
White eyesEnucleation of lens done
Left eye Right eye
conjunctivitis
Topical antibiotics
Plan
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Possible
Many centers across the globe
Cataract
PHPV
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Early recognition and detection – most important
Correct management is essential to prevent permanent
visual loss
Evaluation and treatment should go side by side
Knowledge of common associated systemic conditions
Team effort is essential (ophthalmologist, pediatrician,
geneticist)
Counseling of parents and family members