This document discusses congenital cataracts. It defines congenital cataracts as opacities of the lens present at birth. The signs include opacity of the lens, white pupil reflex, abnormal eye movements, and strabismus. There are different types of congenital cataracts including zonular, polar, nuclear, and posterior lenticonus. The workup involves assessing vision, examining the eyes, and performing additional tests if needed. Treatment options include referral to a pediatrician, treating associated eye diseases, early cataract extraction to prevent amblyopia if vision is obstructed, and treating amblyopia after surgery.

1. Congenital Cataract
Prof. Dr. Hussain Ahmad Khaqan
 MD
 FRCS(Glasgow)
 FCPS(Ophth.)
 FCPS(Vitreo Retina)
 MHPE (KMU)
 CICO(UK)
 CMT(UOL)
 Fellowship in Medical Retina (LMU, Munich)
 Fellowship in Vitreo Retinal Surgery (LMU, Munich)
 Consultant Ophthalmologist & Retinal Surgeon
Professor of Ophthalmology
Lahore General Hospital, Lahore
Ameer Ud Din Medical College, Lahore
Post Graduate Medical Institute, Lahore
Shaukat Khanum Memorial Cancer Hospital & Research Centre ,Lahore

2. SIGNS
• Opacity of the lens at birth
• A white fundus reflex (leukocoria)
• Absent or asymmetric red pupillary reflex
• Abnormal eye movements (nystagmus) in one or both
eyes
• Strabismus

3. Figure: Bilateral cataract with esotropia Figure: Left eye leukocoria

4. TYPES
• Zonular (lamellar): Most common type of congenital
cataract. White opacities that surround the nucleus
with alternating clear and white cortical lamella
resembling an onion skin.
• Polar: Small opacities of the lens capsule and adjacent
cortex on the anterior or posterior pole of the lens.
• Nuclear: Opacity within the embryonic/fetal nucleus.
• Posterior lenticonus: A posterior protrusion, usually
opacified, in the posterior capsule.
• Posterior subcapsular: Opacification of the area
immediately anterior to the posterior capsule.

5. Infantile Cataracts
Cataract Comments
Anterior polar cataract Dominantly inherited, well defined opacities of the anterior capsulate may affect the
vision
Spear cataract Dominantly inherited, polymorphic cataract with needle like clusters of opacities in a
rod shape it is called a fusiform cataract.
Coralliform cataract Dominantly inherited cataract that consists of round and oblong opacities grouped in
the axial region which may not affect vision
Floriform cataract A rare rings shaped bluish white cataract in the axial region
Lamellar cataract A common , bilateral and symmetrical round , gray shell of opacity that surrounds a
clear nucleus.
Cataract centralis pulverulenta Dominantly inherited nonprogressive cataract consisting of fine white powdery dots
within the embryonic or fetal nucleus
Congenital punctate cerulean
cataract
Bilateral , nonprogressive small bluish dots scattered throughout the lens with little
effect on vision
Mittendorf’s Dot A small (about 1 mm diameter) nonprogressive, white condensation occurs on the
posterior pole of the lens capsule

6. Figure: Zonular or Lamellar cataract. Figure: Anterior polar cataract
Figure: Posterior polar cataract Figure: Congenital nuclear cataract

7. Figure: Posterior lenticonus Figure: Posterior subcapsular cataract
Figure: Coralliform cataract Figure: Congenital stellate cataract

8. WORK-UP
• History: Maternal illness or drug ingestion during pregnancy? Systemic or
ocular disease in the infant or child? Radiation exposure or trauma? Family
history of congenital cataracts? Steroid use?
• Visual assessment of each eye individually by using techniques for
nonverbal children (tumbling E’s, pictures, Teller cards, or by following
small toys or a light).
• Ocular examination: Attempt to determine the visual significance of the
cataract by evaluating the size and location of the cataract.
• Cycloplegic refraction.
• B-scan
• Other tests as suggested by the systemic or ocular examination.

9. TREATMENT
1. Referral to a pediatrician to treat any underlying disorder.
2. Treat associated ocular diseases.
3. Cataract extraction, usually within days to weeks of discovery
to prevent irreversible amblyopia, is performed in the following
circumstances:
• Visual axis is obstructed, and the eye’s visual development
is at risk.
• Cataract progression threatens the health of the eye

10. 4. After cataract extraction, treat amblyopia
5. A dilating agent (e.g. , phenylephrine 2.5%, t.i.d. or
cyclopentolate 1% b.i.d.) may be used as a temporizing measure,
allowing peripheral light rays to pass around the lens opacity and
reach the retina. If the cataract is small, and the red reflex is
good around the peripheral lens, this may be the only treatment
needed.
6. Unilateral cataracts that are not large enough to obscure the
visual axis may still result in amblyopia despite not needing
cataract extraction.
TREATMENT