ECTOPIA LENTIS

 

ECTOPIA LENTIS

 

DEFINITION:

A coloboma of the lens characterized by displacement of the lens.

EPIDEMIOLOGY:

1. Etiology

1. Ocular Changes

  • 1. Trauma
  • 2. Congential
  • 1. Aniridia
  • 2. Congenital Glaucoma
  • 3. Hereditary
    • Simple Ectopia Lentis
    • Ectopia Lentis et Pupilla
  • 3. Others
    • intraocular tumor, megalocornea, uveitis
  • 2. Systemic Causes

  • 1. Marfan Syndrome
  • 2. Homocystinuria-I
  • 3. Sulfite Oxidase Deficiency
  • 4. Weill-Marchesani Syndrome
  • 5. Ehlers-Danlos Syndrome
  • 2. Pathogenesis

    • disruption of the supporting structures (zonular fibres of the ciliary body) results in displacement of the lens
    • two major types of displacement:
      • luxation - complete dislocation of the lens
      • subluxation - partial dislocation of the lens (shifted or tilted)

    CLINICAL FEATURES:

    1. Ocular Manifestations

    • poor visual acuity
      • blurred vision due to myopia, astigmatism, or aphakic hyperopia
      • diplopia
    • iridodenesis
      • tremulousness of the iris (seen best prior to dilatation)
    • phacodenesis
      • tremulousness of the lens (seen best after dilatation)
    • complications
      • glaucoma

    2. Simple Ectopia Lentis

    • autosomal dominant inheritance
    • age of onset: newborn to later in life
    • displacement of the lens up and out

    3. Ectopia Lentis et Pupillae

    • autosomal recessive inheritance
    • bilateral displacement of the lens and pupil (usually temporally) in opposite directions

    4. Marfan Syndrome

    • occurs in 80% of patients with 50% affected by age 5 yrs
    • bilateral displacement of the lens up and out

    5. Homocystinuria-I

    • often present by 5 yrs of age
    • displacement of the lens down and in or into the anterior chamber

    INVESTIGATIONS:

    1. Diagnosis

    1. Slit Lamp

    • black crescent (edge of lens) against a red reflex
    • spherophakia
    • deep anterior chamber

    MANAGEMENT:

    1. Correct Optic Problems

    • glasses or contact lenses

    2. Surgery

    • removal of the lens

     

     

    Pediatric Database - ECTOPIA LENTIS

    Pediatric Organization - Pedbase [at] Gmail.com