This document defines congenital cataract and provides information on its etiology, epidemiology, morphology, diagnosis, differential diagnosis, management, complications, and prognosis. Congenital cataract is a cloudiness of the lens present at birth. It can be bilateral or unilateral, with various morphologies. Diagnosis involves examining the eye and ruling out other conditions. Management typically involves early surgical removal of dense cataracts to prevent amblyopia. Post-operative care and visual rehabilitation is important to optimize outcomes.

2. Overview:
1. Definition
2. Etiology
3. Epidemiology
4. Morphology
5. Diagnosis
6. Differential Diagnosis
7. Management
8. Complication
9. Prognosis

3. Definition:
• A congenital opacity of the
crystalline lens. Cloudiness in the
lens of the eye that is present
at, or develops shortly after
birth. Congenital cataracts are also
the most frequent cause of
leukocoria (white pupil) in
children.
(Friedman, NJ & Kaiser, PK, 2009)

4. Etiology:
BILATERAL
• Idiopathic (60%)
• Hereditary (30%)
• Intrauterine infection
• Associated with ocular disorders
• Tumor
• Metabolic
• Maternal drug ingestion/
malnutrition
• Trauma
UNILATERAL
• Idiopathic (80%)
• Intrauterine infection
• Ocular abnormalities (10%)
• Trauma (9%)
(Friedman, NJ & Kaiser, PK, 2009)

5. Epidemiology:
• The incidence is about 1 in 2000, and
approximately one-third of congenital cataracts
are familial, one-third are associated with a
syndrome, and one-third are isolated.
(Friedman, NJ & Kaiser, PK, 2009)

6. Morphology:
• The morphology of cataract is important for several reasons: it can give a clue to the age of
onset, to the visual prognosis, it may suggest heritability, and it may give a clue to the aetiology.
(Joseph, E., 2006)
Punctate (blue-dot)
cataract
Anterior polar
cataract
-pyramidal
-reduplicated
Posterior polar
cataract
(Embryonal) nuclear
cataract
Coronary cataract Coralliform/Floriform
cataract
Zonular (Lamellar)
cataract
Total cataract
-soft -membranous
(Basak, SK., 2007)

7. Morphology:
(Basak, SK., 2007)

8. Punctate (blue dot) cataract:
• Most common type
• Appears as multiple, tiny blue dots
scattered all over the lens, especially
in the cortex by oblique illumination
with the slit lamp.
• Bluish color is due to the effects of
dispersion of light, in the same way
that the sky appears blue.
• Visual acuity is not affected
(Basak, SK., 2007)

9. Anterior Polar Cataract
• It is sharply demarcated opacity at the
anterior lens capsule, & usually the size of a
pin’s head.
• It may project forwards into the anterior
chamber like a pyramid (pyramidal
cataract); or subsequently, a sub-capsular
cataract may develop, just behind it with a
clear zone in between (reduplicated
cataract)
• Due to delayed formation of the anterior
chamber, during development of the lens.
• These opacities are stationary and rarely
interfere with vision.
(Basak, SK., 2007)

10. Zonular (Lamellar) Cataract
• The commonest developmental
cataract presenting with visual
impairment.
• May be congenital/ occur 1st year of
life.
• Usually bilateral & hereditary
(autosomal dominant), without any
ocular anomaly.
• Ass. with hypovitaminosis vit D or
hypocalcemia & maternal
malnutrition.
(Basak, SK., 2007)

11. Zonular (Lamellar) Cataract
• Consist of concentric, sharply
demarcated zones(lamellae) of
opacities surrounding a core, that is
clear, & enveloped by the clear
cortex externally.
• May be linear opacities, like spokes
of a wheel (Called riders) that
extends outwards towards the
equator pathognomonic.
• Child present with photophobia
(due to light scattering)
• Surgery when VA less than 6/18
(Basak, SK., 2007)

12. Diagnosis:
• History:
• 1. Duration
• 2. Family history of congenital cataracts
• 3. Visual status: Ambulation in familiar & unfamiliar surroundings.
• 4. Behavioural pattern & school performance.
• Birth history:
• 1. History & Degree of consanguinity
• 2. History of maternal infection during 1st trimester
• 3. Gestational age & birth weight
• 4. Birth trauma
• 5. Supplemental O2 therapy in perinatal period.
• 6. Developmental milestone
(Joseph, E., 2006) & (The Royal College of Ophthalmologists, 2010)

13. Diagnosis:
• Ocular examination:
• 1. Measurement of visual acuity
• (An up to date refraction should be available as part of the optometrist’s report)
• 2. Pupil examination
• 3. External eye examination including lids and lashes.
• 4. Measurement of intraocular pressure
• 5. Slit lamp examination
• -associated congenital anomalies of iris & lens.
• -type of cataract
• -iridodenesis/ phacodonesis
• 6. Dilated examination of the cataract and fundus
• 7. Biometry if the child is old enough to cooperate. If not this may need to be
done under anaesthesia
(Joseph, E., 2006) & (The Royal College of Ophthalmologists, 2010)

14. Differential Diagnosis:
 -retinoblastoma
 -toxoplasmosis
 -toxocariasis
 -retinal detachment
 -retinitis of prematurity
 - persistent hyperplastic primary vitreous (PHPV)
 -Coat’s disease
 -coloboma
 -mylinated nerve fibers
 -retinal dysplasia
 -Norrie’s disease
 -incontinentia pigmenti
 -retinoschisis
 -cyclitic membrane
(Friedman, NJ & Kaiser, PK, 2009)

15. Management:
• Indications for surgery:
-They are interfering with a person’s quality of life
-There is a reasonable prospect that surgery will lead to a significant improvement in vision.
-All dense cataract( unilateral/bilateral) & partial cataract with vision less than 6/18.
• Timing for surgery
-The critical period for development of fixation reflex is between 6 weeks & 6 months of age.
So, best time for surgery is before this period to prevent stimulus-deprivation amblyopia.
-In dense monocular cataract, the surgery should be done as early as possible, may be on the
next day after birth.
(Basak, SK., 2007)

16. Surgical Techniques:
• Options available for children are:
-Lensectomy
-Lens aspiration with IOL
-Lens aspiration with Primary Posterior Capsulotomy (PPC) and IOL
-Lens aspiration with PPC, Anterior Vitrectomy (AV) and IOL
-Lens aspiration with PPC, AV, IOL and Posterior capsular optic capture
(PCOC)
(Basak, SK., 2007)

17. Visual Rehabilitation:
• IOL Implantation
-Implanted with an IOL during surgery and the criteria of IOL implantation depend on the child’s age and
whether the cataract is unilateral or bilateral. It is perfectly safe and acceptable to perform primary
implantation in a child older than one year. A foldable acrylic hydrophobic IOL is the most biocompatible IOL.
• Contact Lens
-If no IOL is implanted, contact lenses are given as early as possible to prevent stimulus deprivation amblyopia.
Frequent retinoscopy should be performed to decide the power of CL and an overcorrection of +2 to +3D is
mandatory. Silicon lenses or soft hydrogels are well tolerated.
• Spectacles
-In some children with bilateral aphakia spectacles are better tolerated than contact lenses. In addition a
secondary strabismus may be manipulated by prismatic effect of spectacles. . Bifocal glasses should be
prescribed when the child is about to start school.
(Joseph, E., 2006)

18. Post-Operative Complications:
• -Inflammation
• - Deposition of pigment on lens
• - Iris capture/ Lens decentration
• - Retinal Detachment
• - Glaucoma
(The Royal College of Ophthalmologists, 2010)

19. Prognosis:
• The visual outcome depends on:
-Unilateral /bilateral involvement
-Density of cataract during presentation
-Degree of visual impairment
(Basak, SK., 2007)

20. REFERENCES:
• Basak, SK., Essentials of Ophthalmology 4th edition, 2007. Current Books Int, India, 235-240.
• Bashour, M., et al., http://emedicine.medscape.com/article/1210837-clinical, 07 August 2007.
Congenital Cataract. [Assessed on 08-06-2013]
• Friedman, NJ & Kaiser, PK, Essentials of Ophthalmology, 2009. Saunders Elsevier, India, 92-95.
• Joseph, E., Management of Congenital Cataract, Kerala Journal of Ophthalmology, 2006; 18(3):
224-230.
• Taylor, D., Congenital cataract: the history, the nature and the practice, Journal of Eye, 1998, 12:
9-36.
• The Royal College of Ophthalmologists, Cataract Surgery Guidelines: Paediatric
Ophthalmology, September 2010: 25-31.