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Sturge-Weber Syndrome Follow-up

  • Author: Masanori Takeoka, MD; Chief Editor: Amy Kao, MD  more...
 
Updated: May 27, 2015
 

Patient Education

The Sturge-Weber Foundation provides patients with a mentor undergoing similar procedures or operations.

 
Contributor Information and Disclosures
Author

Masanori Takeoka, MD Assistant Professor, Department of Neurology, Harvard Medical School; Staff Physician, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Boston Children's Hospital

Masanori Takeoka, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Medical Association, Child Neurology Society, Massachusetts Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

James J Riviello, Jr, MD George Peterkin Endowed Chair in Pediatrics, Professor of Pediatrics, Section of Neurology and Developmental Neuroscience, Professor of Neurology, Peter Kellaway Section of Neurophysiology, Baylor College of Medicine; Chief of Neurophysiology, Director of the Epilepsy and Neurophysiology Program, Texas Children's Hospital

James J Riviello, Jr, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Partner received royalty from Up To Date for section editor.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Acknowledgements

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, and Child Neurology Society

Disclosure: Nothing to disclose.

Gerhard W Cibis, MD Clinical Professor, Director of Pediatric Ophthalmology Service, Department of Ophthalmology, University of Kansas School of Medicine

Gerhard W Cibis, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, and American Ophthalmological Society

Disclosure: Nothing to disclose.

Monte A Del Monte, MD Skillman Professor of Pediatric Ophthalmology, Professor of Ophthalmology, Pediatrics and Communicable Diseases, Director of Pediatric Ophthalmology and Strabismus, W K Kellogg Eye Center, University of Michigan Medical School

Monte A Del Monte, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Association for Research in Vision and Ophthalmology, International Society for Genetic Eye Diseases and Retinoblastoma, Pan-American Association of Ophthalmology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Maya Eibschitz-Tsimhoni, MD Assistant Professor of Ophthalmology, Pediatric Ophthalmology and Adult Strabismus, Department of Ophthalmology and Visual Sciences, Kellogg Eye Center, University of Michigan Medical Center

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Pan-American Association of Ophthalmology, Sigma Xi, and Southern Medical Association

Disclosure: Nothing to disclose.

Hampton Roy Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Michael Taravella, MD Director of Cornea and Refractive Surgery, Rocky Mountain Lions Eye Institute; Professor, Department of Ophthalmology, University of Colorado School of Medicine

Michael Taravella, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, and Eye Bank Association of America

Disclosure: AMO/VISX None Consulting

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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A child with Sturge-Weber syndrome with bilateral facial port-wine stain.
Cranial CT scan showing calcifications.
MRI image in Sturge-Weber syndrome.
Single-photon emission computed tomographic scan in Sturge-Weber syndrome.
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.
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.
T1-weighted, axial magnetic resonance imaging (MRI) scans demonstrate left cerebral hemiatrophy associated with leptomeningeal angiomatosis. Image courtesy of Dr. Lamia Salah Elewa.
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.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Choroidal hemangioma. Image courtesy of Thomas M. Aaberg, Jr, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
Circumscribed hemangioma. Image courtesy of F. Ryan Prall, MD.
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.
Table 1. Clinical Manifestations of Sturge-Weber Syndrome
Clinical ManifestationIncidence Rate
Risk of SWS with facial PWS8%
SWS without facial nevus13%
Bilateral cerebral involvement15%
Seizures72-93%
Hemiparesis25-56%
Hemianopia44%
Headaches44-62%
Developmental delay and mental retardation50-75%
Glaucoma30-71%
Choroidal hemangioma40%
Table 2. Developmental Morbidity Associated with Seizures in Adults with SWS
 With Seizures (%) Without Seizures (%)
Developmental delay450
Emotional/behavioral problems8558
Need for special education710
Employability4678
Table 3. Summary of Work-up Findings in Sturge-Weber Syndrome
ProcedureFindings
CSF analysisElevated protein
Skull radiographyTram-track calcifications
AngiographyLack of superficial cortical veins



Non-filling dural sinuses



Abnormal, tortuous vessels



CT scanningCalcifications, tram-track calcifications



Cortical atrophy



Abnormal draining veins



Enlarged choroid plexus



Blood-brain barrier breakdown (during seizures)



Contrast enhancement



MRIGadolinium enhancement of leptomeningeal angiomas (LAs)



Enlarged choroid plexus



Sinovenous occlusion



Cortical atrophy



Accelerated myelination



SPECT scanningHyperperfusion, early



Hypoperfusion, late



PET scanningHypometabolism
Electroencephalography (EEG)Reduced background activity



Polymorphic delta activity



Epileptiform features



Table 4. Seizure Control in Sturge-Weber Syndrome
Study Complete Partial Refractory/No Control
Gilly et al[95] NA*NA37%
Sujanski and Conradi[42]



(adults)



27%49%24%
Sujanski and Conradi[25, 42] (all ages) 50%39%11%
Pascual-Castroviejo et al[39] 47%12%28%
Oakes[38] 10%NA83%
Sassower et al[87] NANA43%
Arzimanoglou and Aicardi[94] NANA39%
Erba and Cavazzuti[40] 50%NANA
Toronto[90, 96] NANA32%
*NA = not available
Table 5. Surgical Results of Hemispherectomy and Limited Resection from 3 Centers
Center Hemispherectomy Seizure Free Limited resection Seizure Free Improved
Toronto12111182
Paris551578
Boston98630
Total2624321810
24 of 26 patients with hemispherectomy - Seizure free



28 of 32 patients with limited resection - Seizure free or improved



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