Ectopia Lentis

A subluxated lens in the superior nasal
direction. The patient has Marfan's 

Arachnodactyly (long fingers)
in a Marfan's patient

High arch palate in a Marfan's patient.

The pupil of the affected eye(s) is usually dilated for the examination. Describe the direction in which the lens is 
subluxated, this can help with the diagnosis. If the eye is not dilated, look for iridodonesis (abnormal tremor of the 
iris), phacodonesis (abnormal movement of the lens), deep anterior chamber depth or vitreous herniation into the 
anterior chamber. 

In the examination:

  • look for signs of Marfan's syndrome (superior nasal subluxation of the lens, arachnodactyly, high arch 

  • palate and arm span longer than height)
  • rarely the patient has homocystinuria (downward subluxation oft he lens, same features as Marfan's syndrome 

  • but patient tends to be mentally subnormal and may have fair hair) or Weill-Marchesani's syndrome (mental 
    retardation, short stature, stubby fingers)
  • look for pseudoexfoliation syndrome 
  • look for signs of trauma (which is the most common cause of subluxated lens but does not feature as commonly 

  • as Marfan's syndrome in the clinical examination)


1. What are the other causes of lens subluxation?

2. What are the complications of a subluxated lens?

3. What are the risks of performing general anaesthesia on patient with Marfan's syndrome?

4. What is the main risk of performing general anaesthesia in patient with homocystinuria?


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