Disorders Of Lens

DISORDERS OF LENS


DISORDERS OF LENS

Ectopic lentis

  • Defined as displacement or malposition of the crystalline lens of the eye.
  • The lens is considered dislocated (luxated) when it lies completely outside the lens patellar fossa.
  • The lens is described as subluxated when it is partially displaced but contained within the lens space.

Causes are:

  1. Homocystinuria
  2. Marfan syndrome
  3. Weil-Marchesani syndrome
  4. Ehler Danlos syndrome
  5. Consecutive/ spontaneous
  6. Hyperlysinemia
  7. Sulphite oxidase deficiency
  8. Stickler syndrome
  9. Trauma
DISEASE  TYPICAL LENS DISLOCATION
Marfan syndrome Superotemporal
Homocystinuria Inferonasal
Weil-Marchesani syndrome Forward
  • Clinical features includes unicular diplopia, with irregular depth of anterior chamber.
  • Slit lamp examination evaluates lens position & identifies phacodonesis or cataract.
  • Slit lamp (oblique illumination) typically shows the edge of the lens as shining (bright) golden crescent.
  • Coaxial illumination (Direct ophthalmoscopy) shows the edge of subluxated lens as a dark crescent line.
  • On retinoscopy the aphakic area is hypermetropic while the phakic area is generally shows index myopia.

Fundus shows two discs:

  1. One large through the phakic area
  2. one small through the aphakic area

Congenital abnormalities

1. Coloboma of the lens:

  • A notch is seen in the inferior quadrant of the equator.
  • Occurs due to defective developmen of the suspensory ligament in that part.

2. Anterior lenticonus:

  • Cone-shaped anterior axial bulge.
  • May occur in Alport’s syndrome.

3. Posterior lenticonus:

  • Posterior axial bulge.
  • May occur in Lowe’s syndrome

4. Micro-spherophakia:

  • A small spherical lens may occur as an isolated finding or as a feature of Weip Marchessano’s or Marfan’s syndrome.

5. Microphakia is a small lens which occur in Lowe’s syndrome.

6. Lentiglobus: Generalised hemi-spherical deformity.

Exam Important

  • Ectopia lentis is associated with Marfan’s syndrome.

Causes of Ectopia lentis are:

  1. Homocystinuria
  2. Marfan syndrome
  3. Weil-Marchesani syndrome
  4. Ehler Danlos syndrome
  5. Sulphite oxidase deficiency
  • Posterior lenticonus is seen in Lowe’s syndrome.
  • Anterior lenticonus associated with Alport’s syndrome.
  • Anterior Lenticonus is more common in males.
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