Sturge-Weber Syndrome Questions & Answers

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Questions & Answers

Overview

What is the hallmark of Sturge-Weber syndrome (SWS)?

What are the neurological signs and symptoms of Sturge-Weber syndrome (SWS)?

Which factors suggest a progressive course of cortical damage in Sturge-Weber syndrome (SWS)?

What are the physical signs of Sturge-Weber syndrome (SWS)?

What the ocular signs and symptoms of Sturge-Weber syndrome (SWS)?

What are the signs and symptoms of infantile glaucoma in Sturge-Weber syndrome (SWS)?

How Sturge-Weber syndrome (SWS) diagnosed?

How is diffuse choroidal hemangioma diagnosed in Sturge-Weber syndrome (SWS)?

How are seizures assessed in Sturge-Weber syndrome (SWS)?

How is Sturge-Weber syndrome (SWS) treated?

How is IOP treated in Sturge-Weber syndrome (SWS)?

How is PWS treated in Sturge-Weber syndrome (SWS)?

Which surgical procedures are used in the treatment of seizures in Sturge-Weber syndrome (SWS)?

Which surgical procedures are used in the treatment of diffuse choroidal hemangiomas in Sturge-Weber syndrome (SWS)?

Which surgical procedures are used in the treatment of glaucoma in Sturge-Weber syndrome (SWS)?

What is Sturge-Weber syndrome (SWS)?

What are the cutaneous manifestations of Sturge-Weber syndrome (SWS)?

What are neurologic manifestations of Sturge-Weber syndrome (SWS)?

What are ophthalmic manifestations of Sturge-Weber syndrome (SWS)?

How is Sturge-Weber syndrome (SWS) classified?

What is included in patient education about Sturge-Weber syndrome (SWS)?

What causes Sturge-Weber syndrome (SWS)?

What causes neurologic dysfunction in Sturge-Weber syndrome (SWS)?

What is the pathophysiology of Sturge-Weber syndrome (SWS)?

What is the role of genetics in the etiology of Sturge-Weber syndrome (SWS)?

What causes glaucoma in Sturge-Weber syndrome (SWS)?

What is the prevalence of Sturge-Weber syndrome (SWS) in the US?

What is the global prevalence of Sturge-Weber syndrome (SWS)?

At what age do the signs and symptoms of Sturge-Weber syndrome (SWS) typically present?

What are neurologic and developmental morbidities in Sturge-Weber syndrome (SWS)?

What is the prognosis of Sturge-Weber syndrome (SWS)?

What are prognostic factors in Sturge-Weber syndrome (SWS)?

What is the prognosis of PWS in Sturge-Weber syndrome (SWS)?

How do seizures affect the prognosis of Sturge-Weber syndrome (SWS)?

How are seizures in Sturge-Weber syndrome (SWS) characterized?

How does status epilepticus affect the prognosis of Sturge-Weber syndrome (SWS)?

What is the prevalence of strokelike episodes in Sturge-Weber syndrome (SWS)?

What is the prevalence of hemiparesis in Sturge-Weber syndrome (SWS)?

What is the prevalence of hemianopsia in Sturge-Weber syndrome (SWS)?

What is the prevalence of developmental delay and intellectual disability in Sturge-Weber syndrome (SWS)?

What is the prevalence of headaches in Sturge-Weber syndrome (SWS)?

What is the prevalence of glaucoma in Sturge-Weber syndrome (SWS)?

Which ocular manifestations of Sturge-Weber syndrome (SWS) may be caused by choroidal hemangioma?

What is the prevalence of ocular manifestations in Sturge-Weber syndrome (SWS)?

What is the prevalence of endocrine disorders in Sturge-Weber syndrome (SWS)?

What are morbidities in Sturge-Weber syndrome (SWS)?

What is the prognosis of Sturge-Weber syndrome (SWS)? In adults

Presentation

Which neurological findings are characteristic of Sturge-Weber syndrome (SWS)?

Which clinical history findings are characteristic of a progressive course of cortical injury in Sturge-Weber syndrome (SWS)?

Which physical findings are characteristic of Sturge-Weber syndrome (SWS)?

How are the cutaneous lesions in Sturge-Weber syndrome (SWS) characterized?

Which ocular findings are characteristic of Sturge-Weber syndrome (SWS)?

What are the ocular changes characteristic of Sturge-Weber syndrome (SWS)?

What causes amblyopia in Sturge-Weber syndrome (SWS)?

Which ocular findings are characteristic of choroidal hemangioma in Sturge-Weber syndrome (SWS)?

Which retinal changes may be present in Sturge-Weber syndrome (SWS)?

How is glaucoma characterized in Sturge-Weber syndrome (SWS)?

DDX

Which syndromes and CNS disorders are included in the differential diagnoses of Sturge-Weber syndrome (SWS)?

How is Klippel-Trenaunay-Weber syndrome differentiated from Sturge-Weber syndrome (SWS)?

How is Beckwith-Wiedemann syndrome differentiated from Sturge-Weber syndrome (SWS)?

Which imaging findings may be helpful in differentiating Sturge-Weber syndrome (SWS) from other conditions?

How is choroidal hemangioma in Sturge-Weber syndrome (SWS) differentiated from choroidal melanoma?

Which conditions are included in the differential diagnoses of a diffuse choroidal detachment in Sturge-Weber syndrome (SWS)?

Which conditions are included in the differential diagnoses of Sturge-Weber syndrome (SWS)?

What are the differential diagnoses for Sturge-Weber Syndrome?

Workup

What are the initial steps in the diagnosis of Sturge-Weber syndrome (SWS)?

Which clinical procedures are used to confirm the diagnosis of Sturge-Weber syndrome (SWS)?

What is the role of a free thyroxine assay in the workup of Sturge-Weber syndrome (SWS)?

What is the role of skull radiography in the workup of Sturge-Weber syndrome (SWS)?

What is the role of angiography in the workup of Sturge-Weber syndrome (SWS)?

What is the role of CT scanning in the workup of Sturge-Weber syndrome (SWS)?

What is the role of MRI in the workup of Sturge-Weber syndrome (SWS)?

What is the role of MRI with gadolinium enhancement in the workup of Sturge-Weber syndrome (SWS)?

What is the role of MR spectroscopy and diffusion-weighted MRI in the workup of Sturge-Weber syndrome (SWS)?

What is the role of BOLD MR venography in the workup of Sturge-Weber syndrome (SWS)?

What is the role of MRI with DTI in the workup of Sturge-Weber syndrome (SWS)?

Which MRI findings are characteristic of Sturge-Weber syndrome (SWS)?

What is the role of SPECT scanning in the workup of Sturge-Weber syndrome (SWS)?

What is the role of PET scanning in the workup of Sturge-Weber syndrome (SWS)?

What is the role of ultrasonography in the workup of Sturge-Weber syndrome (SWS)?

What is the role of xenon inhalation in the workup of Sturge-Weber syndrome (SWS)?

When are multiple imaging modalities indicated in the workup of Sturge-Weber syndrome (SWS)?

What is the role of EEG in the workup of Sturge-Weber syndrome (SWS)?

Which histologic findings are characteristic of Sturge-Weber syndrome (SWS)?

Treatment

What is included in medical care of Sturge-Weber syndrome (SWS)?

When is surgery indicated in the treatment of Sturge-Weber syndrome (SWS)?

How are seizures in Sturge-Weber syndrome (SWS) treated?

What is the role of medications in the treatment of glaucoma in Sturge-Weber syndrome (SWS)?

How are recurrent headaches in Sturge-Weber syndrome (SWS) treated?

How are strokelike events in Sturge-Weber syndrome (SWS) treated?

How are PWS in Sturge-Weber syndrome (SWS) treated?

What is the role of surgery in the treatment of seizures in Sturge-Weber syndrome (SWS)?

What is the efficacy of epilepsy surgery in the treatment of Sturge-Weber syndrome (SWS)?

When is a focal cortical resection indicated in the treatment of Sturge-Weber syndrome (SWS)?

What is the role of hemispherectomy in the treatment of Sturge-Weber syndrome (SWS)?

What is the efficacy of hemispherectomy in the treatment of Sturge-Weber syndrome (SWS)?

What are the Sturge-Weber Foundation recommendations for the surgical treatment of Sturge-Weber syndrome (SWS)?

What is the role of surgery in the treatment of diffuse choroidal hemangiomas in Sturge-Weber syndrome (SWS)?

What is the role of laser photocoagulation in the treatment of diffuse choroidal hemangiomas in Sturge-Weber syndrome (SWS)?

What is the role of radiation therapy in the treatment of diffuse choroidal hemangiomas in Sturge-Weber syndrome (SWS)?

What is the role of drainage in the treatment of diffuse choroidal hemangiomas in Sturge-Weber syndrome (SWS)?

What is the role of surgery in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What is the role of goniotomy and trabeculotomy in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What is the role of filtration surgery in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What is the role of cyclocryotherapy in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What is the role of Seton devices in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What is the role of laser surgery in the treatment of Sturge-Weber syndrome (SWS) glaucoma?

What are the possible complications of glaucoma surgery for Sturge-Weber syndrome (SWS)?

How is choroidal hemorrhage and effusion treated following Sturge-Weber syndrome (SWS) glaucoma surgery?

What causes choroidal and retinal detachment from Sturge-Weber syndrome (SWS) glaucoma surgery and how are they treated?

How are complications of Sturge-Weber syndrome (SWS) glaucoma surgery prevented?

How are anisometropia and strabismus corrected in the treatment of Sturge-Weber syndrome (SWS)?

Which specialist consultations are beneficial to patients with Sturge-Weber syndrome (SWS)?

What is included in the long-term monitoring of Sturge-Weber syndrome (SWS)?

Medications

Which medications are used in the treatment of Sturge-Weber syndrome (SWS)?

Which medications in the drug class Antineoplastic Agents are used in the treatment of Sturge-Weber Syndrome?

Which medications in the drug class Corticosteroids, Ophthalmic are used in the treatment of Sturge-Weber Syndrome?

Which medications in the drug class Antiglaucoma, Prostaglandin Agonists are used in the treatment of Sturge-Weber Syndrome?

Which medications in the drug class Antiglaucoma, Carbonic Anhydrase Inhibitors are used in the treatment of Sturge-Weber Syndrome?

Which medications in the drug class Beta-Blockers, Nonselective are used in the treatment of Sturge-Weber Syndrome?

Which medications in the drug class Anticonvulsants are used in the treatment of Sturge-Weber Syndrome?

Follow-up

Where are patient education resources for Sturge-Weber syndrome (SWS) found?