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Detailed information of ECTOPIA LENTIS
ECTOPIA LENTIS
DEFINITION:
A coloboma of the lens characterized by displacement of the lens.
EPIDEMIOLOGY:
- 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
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
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Pediatric Database - ECTOPIA LENTIS
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