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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
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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