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Struge-Weber Syndrome Parts of the visual system affected Sturge-Weber Syndrome is a congenital and progressive condition. Increased pressure within the eye (glaucoma) is a condition which can be present at birth or develop later due to SturgeWeber Syndrome. The incidence of glaucoma in patients with Sturge-Weber is approximately 70% and 40% for choroidal lesions. The glaucoma is usually restricted to the eye which has the stain involvement. Enlarging of the eye (buphthalmos) can also occur in the eye which has been affected by the stain. Comprehensive description Sturge-Weber Syndrome is a neurological disorder indicated at birth by seizures accompanied by a large port-wine stain birthmark on the forehead and upper eyelid of one side of the face. The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the trigeminal nerve just beneath the surface of the face. Sturge-Weber syndrome is also accompanied by the loss of nerve cells and calcification of tissue in the cerebral cortex of the brain on the same side of the body as the birthmark. Neurological symptoms include seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark and vary in severity. There may be muscle weakness on the same side. Some children will have developmental delays and mental retardation; most will have glaucoma (increased pressure within the eye) at birth or developing later. The increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket (buphthalmos). Sturge-Weber syndrome rarely affects other body organs. The most apparent sign of SWS is a birthmark or port wine stain (PWS) on the face. The PWS is due to an overabundance of capillaries just beneath the surface of the involved areas. When the port wine stain covers the eye and forehead region of the face, SWS should be considered. Much variation in the size of the stain has been reported and may be limited to one side of the face or may involve both sides. The stain, varying from light pink to deep purple, is due to an overabundance of capillaries just beneath the surface of the involved skin. In persons with dark pigmentation, the stain may be difficult to recognize. In rare instances, there is an absence of a Port Wine Stain. Effects of the condition on the visual system Most children will have glaucoma (increased pressure within the eye) at birth or developing lateras a result of Sturge-Weber Syndrome. The increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket (buphthalmos). Common treatments Treatment for Sturge-Weber syndrome is symptomatic. Laser treatment may be used to lighten or remove the birthmark. Anticonvulsant medications may be used to control seizures. Surgery may be performed on more serious cases of glaucoma. Physical therapy should be considered for infants and children with muscle weakness. Educational therapy is often prescribed for those with mental retardation or developmental delays. Doctors recommend yearly monitoring for glaucoma. There is no known prevention for Sturge-Weber Syndrome. Anticipated functional implications of the condition Infants diagnosed with Sturge-Weber Syndrome must be followed closely for other medical issues, including vision problems, epilepsy, and developmental delays. Most cases of Sturge-Weber are not lifethreatening. The patient's quality of life depends on how well the symptoms (such as seizures) can be prevented or treated. Patients will need to visit an ophthalmologist at least once a year to treat glaucoma. They also will need to see a neurologist to treat seizures and other neurologic symptoms. Complications Abnormal blood vessel growth in the skull Continued growth of the port-wine stain Developmental delays Emotional and behavioral problems Glaucoma, which may lead to blindness Paralysis Seizures References: National Institute of Neurological Disorders and Stroke. (n.d.). Retrieved from http://www.ninds.nih.gov/disorders/stur ge_weber/sturge_weber.htm The Sturge-Weber Foundation. (n.d.). Retrieved from http://www.sturgeweber.org/about-sws/conditions/12sturge-weber-syndrome.html Seattle Children’s Hospital. (Copyright 19952011). Retrieved from http://www.seattlechildrens.org/medical -conditions/common-childhoodconditions/sturge-weber/ The Hunter Nelson Sturge-Weber Center. (Copyright 2007). Retrieved from http://sturgeweber.kennedykrieger.org/ U.S. National Library of Medicine. (2011, July 1). Retrieved from http://www.nlm.nih.gov/medlineplus/en cy/article/001426.htm Developed by Jerry Mullins