This presentation is orginaly uploaded to http://kpkmedicalcolleges.tk by Dr.Suleman

1. Lens & Cataract
Congenital Defects of Lens
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Congenital Aphakia
Lenticonus and Lentiglobus
Lens Coloboma
Mittendorf’s Dot (Hyaloid Corpuscle)
Epicapsular Star
Micro-sphero Phakia

2. Congenital Defects of Lens
 Congenital Aphakia
 Very rare
 Associated other eye malformations ±
 Primary: Lens plate fails to form
 Secondary:
 More common than primary aphakia
 It occurs when developing lens is
spontaneously absorbed

3. Congenital Defects of Lens
 Lenticonus and Lentiglobus
 Lenticonus
 UL
 Axial in location
 Localized cone-shaped deformation of
anterior / posterior lens surface
 Posterior more common
 Anterior (Alport syndrome) and often BL.

4. Congenital Defects of Lens
 Lenticonus and Lentiglobus
 Lentiglobus
 Shperical localized lens surface deformation
 Posterior> anterior
 May be associated with lens opacities
 Retinoscopy – distorted/Myopic reflex (center)
 Retroillumination – Deformation visible in red
reflex as “Oil droplet” (cf. “Oil droplet” of
galactosemia)

5. Congenital Defects of Lens
 Lens Coloboma
 An anomaly of lens shape
 Primary: wedge shaped defect in lens
periphery (isolated anomaly)
 Secondary: flattening/indentation of lens
periphery due to lack of ciliary body or zonular
development

6. Congenital Defects of Lens
 Lens Coloboma
 Location:
 Inferiorly
 May be associated with uveal colobomas
 Adjascent lens opacities ±
 Cortical/capsular thickening
 Zonular attachments in the region of coloboma
usually show some defects

7. Congenital Defects of Lens
 Mittendorf’s Dot (Hyaloid corpuscle)
 Common anomaly observed in many healthy eyes
 Remnant of posterior vascular capsule of tunica
vaculosa lentis
 Small, dense white spot inferior nasal to posterior
pole of lens
 Sometimes associated with a fibrous tail projecting
into the vitreous body

8. Congenital Defects of Lens
 Epicapsular Star
 Remnant of tunica vasculosa lentis
(common)
 Star shaped brown/golden flecks on the
central anterior capsule
 Often called chicken tracks

9. Congenital Defects of Lens
 Micro-sphero-phakia
 Lens spherical and small in diameter (Eye highly
myopic)
 Entire lens visible through dilated pupil
 Isolated anomaly/associated with other anomalies
like:
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Marfan syndrome
Peter’s anomaly
Alport syndrome
Weill-Marchesani syndrome
Congenital rubella
Lowe syndrome

10. Congenital Defects of Lens
 Micro-sphero-phakia
 Lens may block pupil (ACG):
 Miotics aggrevate
 Cycloplegics relieve an attack of ACG
 Treatment:
 Laser iridotomy