Sturge-Weber Syndrome (SWS) Print E-mail

What is it?

Birthmark IrelandSturge-Weber Syndrome is a congenital, non-familial disorder with no known cause. It is characterized by a facial birthmark and neurological abnormalities. Other symptoms associated with Sturge-Weber can include eye, endocrine and organ irregularities, as well as developmental disabilities. Each child with Sturge-Weber Syndrome is different and exhibits the characterizing findings to varying degrees.

What are the statistics?

  • SWS has no clear genetic pattern, and two affected individuals almost never arise in the same family. The syndrome presents in all races and with equal frequency in both sexes.
  • In a patient with a facial port wine stain, the overall risk of having SWS is only about 8% to 15%. The risk of having SWS increases to 25% when half of the face, including the ophthalmic division of the trigeminal nerve is involved and rises to 33% when both sides of the face, including the ophthalmic division of the trigeminal nerve are involved.
  • Epileptic s eizures occur in 72% to 80% of SWS patients with brain lesions on one side and in 93% of patients with lesions on both sides.
  • Seizures can begin anytime from birth to adulthood, but 75% of those with seizures begin having them during the first year of infancy, 86% by age 2, and 95% before age 5.
  • Glaucoma occurs in 30% to 71% of patients.

Investigations and Treatment.

Seizures - There are many types of seizures, and children with SWS may present with more than one type. The seizure threshold may be lowered by cutting teeth, immunizations, illness, etc. Your doctor will give you more information on types of seizures and how to recognise and manage them. Anti-epileptic medications may be perscribed.

Eye - All newborn babies with a port wine mark affecting the eyelids should see an eye specialist in the first few weeks of life. Children with port wine mark involving lids may be at risk of glaucoma and require regular eye checks. Glaucoma is raised pressure within the eye, which must be treated.

Brain - An MRI scan may be done to confirm SWS.

Birthmark IrelandLaser treatment- It may lighten the colour and decrease the size of port wine stains and thereby give significant improvement, but completely removing it is not usually possible at this time. But the earlier a child begins laser treatments, the more effective they will be, and laser treatment can start at a very young age. The number of treatments needed or the degree of clearing depends on the stain itself, and varies with each child. It is done under a light general anaesthetic as the laser stings when being used. It has been likened to the snapping of an elastic band on the skin. Calpol and/or baby neurofen are given post treatment for soreness. An aloe vera gel is used for a few days post treatment to cool the area, and a good moisturiser applied daily, or more often as required. There are strict rules regarding the use of sunscreen too to protect the area, and all this will be explained by the dermatologist and his team.

Further information.

Further information can be obtained from the following website:

 

 

 

 
Birthmark Ireland