Sturge-Weber's syndrome
 

Right sided port-wine stain of the face and ipsilateral  tissue hypertrophy. Left-sided port-wine stain with conjunctival  involvement. The patient also has left glaucoma.

The face of this patient has a port-wine in the distribution of the first and second divison of the trigeminal nerve.  The ipsilateral eye may be red (due to the presence of haemangioma of the conjunctiva; the iris may be involved  causing heterochromic iridis). The affected side of the face may also show hypertrophy. Further examination: examine the anterior segment for haemangioma and the presence of trabeculectomy posterior segment for glaucomatous disc and choroidal haemangioma (this is diffuse and may cause exudative  retinal detachment).

Question:

1. What is the inheritance of Sturge-Weber's syndrome?

Answer
Unlike other phakomatosis which has a hereditary tendency, Sturge-Weber's syndrome is a sporadic condition.

2. What is responsible for the port-wine stain and can it be removed?

Answer
There are caused by abnormal ectatic vessels in the dermis. Removal can be achieved with photothermolysis using dye laser.

 

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