
Treatment Treatment for Sturge-Weber syndrome is symptomatic. Laser treatment may be used to lighten or remove the birthmark. Anticonvulsant
Anticonvulsant
Anticonvulsants are a diverse group of pharmacological agents used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder and borderline personality disorder, since many seem to act as mood stabilizers, and for the treatment of neuropathic pain. Anticonvulsants suppress the excessive rapid firing of neurons during seizures. Antic…
Full Answer
How is Sturge Weber syndrome (SWS) treated?
Sturge-Weber syndrome (also called encephalofacial or encephalotrigeminal angiomatosis) is a rare neurocutaneous syndrome characterized by facial port-wine stains in the trigeminal nerve distribution area. An innovative treatment based on surgical and laser techniques of a patient affected with Sturge-Weber syndrome is reported.
What is Sturge Weber syndrome of the eye?
What are the complications of Sturge-Weber syndrome?
What is the efficacy of hemispherectomy in the treatment of Sturge-Weber syndrome?
In patients with Sturge-Weber syndrome, brain involvement typically presents in infancy with seizures, strokes, and stroke-like episodes, and a range of neurologic impairments. Standard treatment includes laser therapy for the birthmark, control of glaucoma through eyedrops or surgery, and the use of anticonvulsants.

How does laser treatment work on port-wine stain?
“The VBeam pulsed dye laser specifically targets the red blood cells and vessels in the port-wine stain,” Dr. Olasz Harken said. “The laser's energy, the photon, is preferentially absorbed in the vessels and is converted to heat, which in turn destroys the vessels.Feb 4, 2020
Do port-wine stains come back after laser?
"Although pulsed-dye laser treatment of port-wine stains is still the best and most effective method currently available, patients should be aware of the fact that the effect of this treatment may not last forever and that the port-wine stain may return -- in part -- at long-term follow-up," said Dr.Mar 23, 2008
How does pulsed dye laser work?
The pulsed dye laser works by targeting the tiny blood vessels beneath the skin's surface which, over time (or due to scarring or birth marks, for instance), can become large or clustered, creating the appearance of redness. The laser delivers a beam of concentrated pure yellow light to the targeted blood vessels.Dec 18, 2018
How many laser treatments does it take to remove port-wine stains?
Multiple treatments are the norm, and most patients require eight to ten treatments or more for optimal results.Jan 12, 2015
Can port-wine birthmarks be removed?
Port-wine stains won't go away on their own, but they can be treated. Laser therapies can make many port-wine stains much less noticeable by shrinking the blood vessels in the birthmark and fading it.
Can port-wine stains become cancerous?
Nonmelanoma skin cancer is known to develop in port-wine stains, most commonly basal cell carcinoma. The range of skin cancer types known to arise in these malformations can be expanded to include melanoma in situ. It is important to routinely examine these vascular proliferations for new lesions.
How long does it take to heal from pulsed dye laser?
After Your Pulsed-Dye Laser Skin Therapy Procedure Your esthetician can provide you with gels or cremes to aid in your recovery, but most symptoms will resolve within 1-3 days, at which point most patients are able to resume normal activities, including wearing makeup.
How painful is pulsed dye laser?
The pulses of the laser might feel similar to having a rubber band snapped against the skin. This feeling is more startling than painful. The number of pulses in a treatment depends on the size of the birthmark and varies from patient to patient.
How long does it take to see results from pulsed dye laser?
You'll begin to see results about 4 to 8 weeks after your VBeam treatment. Most patients achieve 25-75% improvement with each treatment.Feb 9, 2021
Do port-wine stains get bigger?
Some port wine stains are small, others can be quite large. Port wine stains can be found anywhere on the body, but most often appear on the face, neck, arms, legs and scalp. They will grow as the child grows (not enlarge by themselves) and become darker in adulthood.
How many treatments does it take to get rid of a port-wine stain?
The pulsed dye laser, the standard therapy for port-wine stains since 1986, can achieve 50% to 75% lightening within 2 to 3 treatments.Sep 1, 1995
Are port-wine stains permanent?
A port-wine stain is a permanent birthmark present from birth. It starts out pinkish or reddish and turns darker as the child grows. Most often, a port-wine stain appears on the face, but it can affect other areas of the body.
How to treat Sturge Weber syndrome?
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. Persons with drug-resistant seizures may be treated by surgical removal of epileptic brain tissue.
What is the name of the condition that causes the loss of nerve cells and calcification of underlying tissue in the
Sturge-Weber syndrome is also accompanied by abnormal blood vessels on the brain surface and the loss of nerve cells and calcification of underlying tissue in the cerebral cortex of the brain on the same side of the brain as the birthmark.
Why does my eyeball bulge out?
Some children will have developmental delays and cognitive impairment; 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).
What is SWS surgery?
Surgical procedures include focal cortical resection, hemispherectomy, corpus callosotomy, and vagal nerve stimulation (VNS). [ 4] . SWS is considered one of the catastrophic epilepsies, which, according to Holmes, result in poor seizure control and developmental outcome if not controlled early.
Why is cryotherapy used for glaucoma?
Cryotherapy and penetrating diathermy are of limited use because of the posterior location of the tumor. Most ophthalmologists consider surgical therapy to be the mainstay of glaucoma therapy in patients with Sturge-Weber syndrome, [ 92] with antiglaucoma medications primarily useful as treatment adjuncts.
Is a trabeculectomy successful for glaucoma?
With glaucoma onset in the older age group, when the outflow angle appears clinically normal, glaucoma filtration surgery, either full thi ckness or partial thickness (trabeculectomy), is more likely to be successful, because it bypasses any component of the glaucoma possibly caused by elevated episcleral venous pressure.
What is SWS in the eye?
Key Points. SWS is a rare, sporadic disorder involving vasculature in the facial skin, CNS, and eye. The chief ocular manifestation is glaucoma, in which developmental anomaly of the anterior chamber angle and elevated episcleral venous pressure are underlying mechanisms.
What is the treatment for PWS?
The current management of PWS is treatment with a pulsed dye laser. Usually, early intervention and multiple treatments are required to lighten the skin discoloration and enhance cosmetic outcomes. EXTERNAL FINDINGS.
What are the vascular abnormalities of the eyelid, orbit, conjunctiva, episcler
Vascular abnormalities of the eyelid, orbit, conjunctiva, episclera, ciliary body, retina, and choroid may occur. Dense episcleral venous plexus and ampulliform dilatations of the conjunctival vessels are usually observed on anterior segment examination. Choroidal hemangiomas occur in up to 50 percent of patients with SWS and may be localized or diffuse. They may cause vision loss from choroidal thickening or retinal detachment. Iris heterochromia, with hyperpigmentation on the affected side of the face, may be noted. Glaucoma is the principal, therapeutically challenging ocular abnormality of SWS.
What percentage of SWS patients have glaucoma?
Glaucoma occurs in 30 to 70 percent of individuals with SWS. 3 The incidence of glaucoma increases with PWS involvement of the ipsilateral eyelid and with more severe vascular anomalies of the episclera and conjunctiva.
What is the SWS?
Sturge-Weber syndrome (SWS) belongs to a group of disorders known as phakomatoses. These disorders are characterized by hamartomas, which are congenital tumors arising from tissue that is normally found at the involved site. Unlike other phakomatoses, SWS has no hereditary pattern and is caused by a somatic mutation in the GNAQ gene. 1 The incidence of this condition is approximately 1 per 50,000 live births, with no reported racial or gender predilection. The classic triad of SWS consists of facial cutaneous venous dilation, often called port-wine stain (PWS), leptomeningeal capillary-venous malformation, and ocular abnormalities. This review discusses the clinical features of SWS with an emphasis on secondary glaucoma, the most common ocular manifestation of this disorder.
What causes SWS?
Unlike other phakomatoses, SWS has no hereditary pattern and is caused by a somatic mutation in the GNAQ gene. 1 The incidence of this condition is approximately 1 per 50,000 live births, with no reported racial or gender predilection.
Where is PWS on the face?
PWS is typically present on the forehead and upper eyelid, along the ophthalmic and maxillary divisions of the trigeminal nerve. Although usually unilateral and apparent at birth, the lesion may extend to both sides of the face and extremities. In newborns, PWS is flat with a light pink appearance.
