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 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 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 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
How is Klippel-Trenaunay-Weber syndrome differentiated from Sturge-Weber syndrome (SWS)?
How is Beckwith-Wiedemann syndrome differentiated from Sturge-Weber syndrome (SWS)?
How is choroidal hemangioma in Sturge-Weber syndrome (SWS) differentiated from choroidal melanoma?
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 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 is the role of surgery 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 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)?
Follow-up
Where are patient education resources for Sturge-Weber syndrome (SWS) found?
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A child with Sturge-Weber syndrome with bilateral facial port-wine stain.
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Cranial CT scan showing calcifications.
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MRI image in Sturge-Weber syndrome.
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Single-photon emission computed tomographic scan in Sturge-Weber syndrome.
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A child with Sturge-Weber syndrome that primarily affects the distribution of cranial nerve V2-3, with milder involvement of cranial nerve V1. Secondary glaucoma is evident. Ocular melanocytosis involving the sclera of both eyes is an associated finding. Image courtesy of Dr. Lamia Salah Elewa.
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Close-up view of the left eye, showing the Ahmed valve implanted in the inferotemporal quadrant after multiple failed filtration procedures induced severe superior conjunctival scarring. Intraocular pressure (IOP) was controlled. Image courtesy of Dr. Lamia Salah Elewa.
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T1-weighted, axial magnetic resonance imaging (MRI) scans demonstrate left cerebral hemiatrophy associated with leptomeningeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.
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Ocular ultrasonogram of the posterior segment demonstrating the diffuse choroidal thickening seen in a diffuse choroidal hemangioma with "tomato-catsup fundus." Image courtesy of Dr. Lamia Salah Elewa.
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Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
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Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
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Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
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Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
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B-scan of a choroidal hemangioma showing medium to high internal reflectivity. This is a circumscribed choroidal hemangioma. The patient was not diagnosed with Sturge-Weber Syndrome. Image courtesy of Abdhish R Bhavsar, MD.
Tables
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Approach Considerations
- Pharmacologic Treatment of Seizures
- Pharmacologic Treatment of Glaucoma
- Headaches
- Strokelike Events
- Port-Wine Stain
- Surgical Treatment of Seizures
- Hemispherectomy
- Surgical Treatment of Diffuse Choroidal Hemangiomas
- Glaucoma Surgery
- Correction of Anisometropia and Strabismus
- Consultations
- Long-Term Monitoring
- Show All
- Medication
- Follow-up
- Questions & Answers
- Media Gallery
- Tables
- References