What is the main ocular manifestation of Sturge-Weber syndrome?

What is the main ocular manifestation of Sturge-Weber syndrome?

Ocular manifestations of SWS include conjunctival, episcleral, and choroidal hemangiomas. Choroidal hemangiomas may be present in up to 71% of patients with SWS and may be circumscribed or diffuse. SWS is usually associated with the diffuse variant that often presents in patients with late-onset glaucoma.

Is Sturge-Weber syndrome life threatening?

Sturge-Weber syndrome is a congenital disorder that affects the skin, the neurological system, and sometimes the eyes. There is no cure, but it is not fatal. Other names include encephalotrigeminal angiomatosis, encephalofacial angiomatosis, or Sturge-Weber-Dimitri syndrome.

Is Sturge-Weber syndrome life expectancy?

Sturge Weber syndrome life expectancy The long-term outlook varies depending on the severity of symptoms, and how well seizures and glaucoma can be controlled or prevented. More severe seizures at an early age are associated with an increased chance for developmental and intellectual disability 7).

Is Sturge-Weber syndrome a disability?

The seizures usually involve only one side of the brain (focal seizures), during which the port-wine birthmark may darken and individuals may lose consciousness. People with Sturge-Weber syndrome have varying levels of cognitive function, from normal intelligence to intellectual disability.

How is Sturge-Weber syndrome treated?

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.

Can Sturge-Weber syndrome be cured?

Sturge-Weber is a lifelong condition that can’t be cured. However, treatment of symptoms can help prevent complications and improve your child’s quality of life. Seizures: In many cases, seizures can be controlled with anti-seizure medications.

Is Sturge-Weber syndrome curable?

What does Sturge mean?

Thor’s follower
Sturge is a Middle Ages surname of Norse-Viking origins, meaning son of Turgis or Thurgis, Turgeus etc., which meant “Thor’s follower”.

Is Sturge-Weber a neurological condition?

Sturge-Weber syndrome is a neurological disorder indicated at birth by a port-wine stain birthmark on the forehead and upper eyelid of one side of the face.

What is Sturge–Weber syndrome (STW)?

Sturge–Weber syndrome has been included in the group of phakomatoses that is characterized by hamartomas involving the brain, skin, and eyes.

What causes glaucoma and Sturge-Weber syndrome?

Glaucoma is an eye disease often caused by increased pressure in the eye. This can cause vision impairment, sensitivity to light, and eye pain. What Causes Sturge-Weber Syndrome? Though SWS is present at birth, it isn’t an inherited condition. Instead, it’s the result of a random mutation in the GNAQ gene.

What are the symptoms of Sturge-Weber syndrome?

What Are the Symptoms of Sturge-Weber Syndrome? The most apparent indication of SWS is a port-wine stain, or red and discolored skin on one side of the face. The discoloration is due to dilated blood vessels in the face that make the skin appear reddened.

What are the treatment options for choroidal hemangioma in Sturge-Weber syndrome?

Photodynamic therapy of choroidal hemangioma in Sturge-Weber syndrome, with a review of treatments for diffuse and circumscribed choroidal hemangiomas. Surv Ophthalmol.

  • September 21, 2022