Published online June 1, 2006
PEDIATRICS Vol. 117 No. 6 June 2006, pp. 2119-2125 (doi:10.1542/10.1542/peds.2005-1815)
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrowRequest Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (5)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Evans, A. L.
Right arrow Articles by Griffiths, P. D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Evans, A. L.
Right arrow Articles by Griffiths, P. D.
Related Collections
Right arrow Radiology
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Cerebral Perfusion Abnormalities in Children With Sturge-Weber Syndrome Shown by Dynamic Contrast Bolus Magnetic Resonance Perfusion Imaging

Amlyn L. Evans, FRCRa, Elysa Widjaja, FRCRb, Daniel J. A. Connolly, FRCRa,c and Paul D. Griffiths, PhDb,c

a Department of Radiology, Royal Hallamshire Hospital, Sheffield, United Kingdom
b Unit of Academic Radiology, University of Sheffield, Sheffield, United Kingdom
c Department of Radiology, Sheffield Children's Hospital, Sheffield, United Kingdom

OBJECTIVE. Sturge-Weber syndrome is characterized by leptomeningeal angiomatosis and a facial naevus that is usually unilateral. Magnetic resonance imaging is the cornerstone of confirming the disease and judging the extent of the abnormalities. It has been shown, however, that brain perfusion abnormalities on nuclear medicine imaging often are more extensive than the abnormal leptomeningeal enhancement on magnetic resonance. In this article, we assess the utility of magnetic resonance perfusion in demonstrating perfusion abnormalities in pediatric cases of Sturge-Weber syndrome.

METHODS. Magnetic resonance perfusion studies were performed on 7 consecutive children who presented to our department with clinically suspected Sturge-Weber syndrome. The extent of time to peak abnormality on dynamic gadolinium bolus magnetic resonance perfusion imaging was compared with the extent of leptomeningeal enhancement and the presence of venous abnormalities.

RESULTS. Good magnetic resonance perfusion data were obtained in all 7 cases. Perfusion abnormalities were closely anatomically related to meningeal enhancement on postcontrast T1-weighted imaging. However, perfusion abnormalities were found consistently in the vicinity of developmental venous anomalies that were present in 4 of 7 cases. In 1 child, there was a perfusion deficit in the cerebellar lobe contralateral to the leptomeningeal angiomatosis, consistent with crossed cerebellar diaschisis.

CONCLUSIONS. Magnetic resonance perfusion is a sensitive indicator of perfusion abnormalities in Sturge-Weber syndrome and can be performed easily at the same time as the diagnostic scan. Magnetic resonance perfusion imaging therefore is useful in the assessment of this disease. This approach has the extra advantage of correlating the perfusion abnormalities with the high-resolution imaging that is provided from magnetic resonance imaging.

Key Words: cerebral blood flow • phakomatoses • magnetic resonance

Abbreviations: SWS—Sturge-Weber syndrome • FLAIR—fluid-attenuated inversion recovery • SPECT—single photon emission computed tomography • MR—magnetic resonance • FmMTT—first mean transit time

Accepted Nov 10, 2005.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?


This article has been cited by other articles:


Home page
 Am. J. Neuroradiol.Home page
M.E. Adams, S.E. Aylett, W. Squier, and W. Chong
A Spectrum of Unusual Neuroimaging Findings in Patients with Suspected Sturge-Weber Syndrome
AJNR Am. J. Neuroradiol., February 1, 2009; 30(2): 276 - 281.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
L. C. Jordan, R. J. Wityk, M. M. Dowling, M. R. DeJong, and A. M. Comi
Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome
J Child Neurol, February 1, 2008; 23(2): 137 - 143.
[Abstract] [PDF]