eMedicine Specialties > Radiology > Pediatrics

Sturge-Weber Syndrome: Follow-up

Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Coauthor(s): Ian Turnbull, MD, Lecturer, Department of Radiology, University of Manchester; Consulting Neuroradiologist, Hope Hospital, Salford, Manchester and North Manchester Hospital; Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute; Riyadh Al-Okaili, MBBS, Interventional/Therapeutic and Diagnostic Neuro-Radiologist, King Abdulaziz Medical City
Contributor Information and Disclosures

Updated: Feb 26, 2008

Intervention

Cerebral lobectomy may be considered in some patients. In cases with severe drug-refractory focal epilepsy caused by SWS, promising results have been achieved with hemispherectomy.31

Devlin and associates described the clinical course and outcomes in 33 children who underwent hemispherectomy and concluded that the optimal timing of surgery with respect to age at presentation and the influence of the underlying pathology are only slowly emerging. At surgery, their patients were aged 0.33-17 years.32

Vining and associates from John-Hopkins hospital reported their experience with 58 children after hemispherectomy. They concluded that early surgery relieves the burden of constant seizures and allows the child to return to a more-normal life.33 Kossoff et al concluded that the patient's age at surgery does not have an adverse effect on either seizure or cognitive outcomes.34

Medicolegal Pitfalls

  • SWS is a clinical diagnosis in children presenting with developmental delay and hemiparesis, which eventually develop in most patients if medical and surgical treatment are unsuccessful.
  • The radiologic diagnosis is usually made with a high index of clinical suspicion. In diagnosing SWS, the clinician must have supporting imaging findings that have been correctly interpreted.
  • Early surgery relieves the patient from the burden of constant seizures and allows the child to return to a more-normal life.

See also the Medscape topic Medical Malpractice and Legal Issues.

 
Acknowledgments

The authors and editors would like to thank Ramesh Chandra Raja, MBBCH, for his contributions to this article.



More on Sturge-Weber Syndrome

Overview: Sturge-Weber Syndrome
Imaging: Sturge-Weber Syndrome
Follow-up: Sturge-Weber Syndrome
Multimedia: Sturge-Weber Syndrome
References
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References

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Further Reading

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Keywords

SWS, Sturge-Weber-Dimitri syndrome, encephalotrigeminal angiomatosis, trigeminal angiomatosis, meningofacial angiomatosis, encephalofacial angiomatosis, Dimitri's hemangiomatosis, Jahnke's syndrome (variant without glaucoma), Kulisher's syndrome, Krabbe's II syndrome, Lawford's syndrome (variant with glaucoma and without increased ocular pressure), meningocutaneous syndrome, neurooculocutaneous syndrome, Parkes Weber's phacomatosis, vascular encephalotrigeminal Weber-Dimitri syndrome

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Contributor Information and Disclosures

Author

Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP is a member of the following medical societies: American Institute of Ultrasound in Medicine, Radiological Society of North America, Royal College of Physicians, Royal College of Physicians and Surgeons of the United States, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.

Coauthor(s)

Ian Turnbull, MD, Lecturer, Department of Radiology, University of Manchester; Consulting Neuroradiologist, Hope Hospital, Salford, Manchester and North Manchester Hospital
Disclosure: Nothing to disclose.

Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.

Riyadh Al-Okaili, MBBS, Interventional/Therapeutic and Diagnostic Neuro-Radiologist, King Abdulaziz Medical City
Riyadh Al-Okaili, MBBS is a member of the following medical societies: American College of Radiology
Disclosure: Nothing to disclose.

Medical Editor

Charles M Glasier, MD, Professor, Departments of Radiology and Pediatrics, University of Arkansas for Medical Sciences; Chief, Magnetic Resonance Imaging, Vice-Chief, Pediatric Radiology, Arkansas Children's Hospital
Charles M Glasier, MD is a member of the following medical societies: American College of Radiology, American Society of Neuroradiology, Radiological Society of North America, and Society for Pediatric Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Marta Hernanz-Schulman, MD, FAAP, Professor, Radiology, Radiological Sciences, and Pediatrics, Director, Department of Pediatric Radiology, Radiologist-in-Chief, Director, Department of Diagnostic Imaging, Vanderbilt University Medical Center, Vanderbilt Children's Hospital
Marta Hernanz-Schulman, MD, FAAP is a member of the following medical societies: American Institute of Ultrasound in Medicine and American Roentgen Ray Society
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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