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.

1. 20/11/2012

2.  R, 6mth, Mch
 R/O Hamirpur
 Himachal pradesh

3.  Whitish eyes since birth
 Not properly following to light
 Poor response to sounds (Noted subsequently)

4.  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

5.  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

6.  Abdomen- soft, no organomegaly
 CVS - S1S2 normal, no murmur
 Respiratory system - normal
 CNS
 Alert, consolable
 Poor response to sound
 Tone & reflexes normal

7.  5 mth, M
 B/L aphakia
 Impaired hearing

8.  Congenital cataract
 Retinoblastoma
 Retinopathy of prematurity
 Persistent hyperplastic primary vitreous
•EUA
•Dilated pupils
•Direct ophthalmoscopy
•USG of eye and orbit

9.  Post operative case of bilateral congenital cataract with
impaired hearing under evaluation
 Cause of impaired hearing ?
 Rubella
 Retinitis pigmentosa
 Craniofacial dysplasia
 Alports syndrome

10.  Opacity in lens
 Can be:
 Congenital or Acquired
 Unilateral or Bilateral
 Partial or Complete
Congenital: 6/10,000 (10% of childhood blindness)

11. 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

12. Systemic Diseases
 Diabetes mellitus
 Atopic dermatitis
 Neurofibromatosis 2
Ocular Diseases
 Chronic anterior uveitis
 High myopia
 Retinitis pigmentosa
Drugs
•Chlorpromazine
Trauma

13. •Sporadic, no family history
•Ocular anomalies : Persistent foetal vasculature
•Cause identified in only 10%

14.  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

15.  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

16.  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

17.  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

18.  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

19.  Bilateral dense cataracts
 Within six weeks of birth
 1 week apart
 Unilateral dense cataracts
 ASAP (within days)

20.  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

21.  Retrobulbar
hemorrage
 Oculocardiac reflex
 Muscle lacerations
 Corneal / uveal injury
 Vitreous loss
 Nucleous drops into
vitreous cavity
 Hyphaema
 Cystoid macular edema
 Endopthalmitis
 After cataract

22. 6wk 4mth 5mth 6mth
White eyes
Enucleation of lens done
Left eye Right eye
conjunctivitis
Topical antibiotics
Plan

23.  Possible
 Many centers across the globe
 Cataract
 PHPV

24.  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