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Transcranial Doppler Ultrasound in Children with Sturge-Weber Syndrome

Department of Neurology, The Johns Hopkins University School of Medicine

Robert J. Wityk, MD

Department of Neurology, The Johns Hopkins University School of Medicine

Michael M. Dowling, MD, PhD

Department of Pediatric Neurology, University of Texas Southwestern Medical Center, Dallas Texas

M. Robert DeJong, RDMS, RDCS, RVT

Department of Radiology, The Johns Hopkins Hospital

Anne M. Comi, MD

Department of Neurology, The Johns Hopkins University School of Medicine, Department of Neurology and Developmental Medicine, Kennedy Krieger InstituteBaltimore, Maryland, comi{at}kennedykrieger.org

Transcranial Doppler ultrasound is a noninvasive vascular assessment technique proved useful in the management of pediatric disorders predisposed to stroke and may have similar utility for Sturge-Weber syndrome. Eight children with Sturge-Weber syndrome had velocities measured in the major cerebral arteries via the Stroke Prevention Trial in Sickle Cell Anemia methodology. Velocities and pulsatility indexes were compared between the unaffected and affected sides. All subjects had reduced velocity on the affected side; the mean middle cerebral artery percentage difference was 20% (95% CI, 15%-25%). Pulsatility index was increased on the affected side; mean middle cerebral artery pulsatility index percentage difference, 34% (95% CI, 15%-53%). Six subjects also had reduced posterior cerebral artery velocity on the affected side. Side-to-side differences in middle cerebral artery and posterior cerebral artery velocities correlated with severity of MRI asymmetry (Spearman = 0.88, P = .02). Decreased arterial flow velocity and increased pulsatility index in the middle cerebral artery and posterior cerebral artery suggests a high resistance pattern that may reflect venous stasis and may contribute to chronic hypoperfusion of brain tissue. Further study of Transcranial Doppler in children with Sturge-Weber syndrome is indicated.

Key Words: transcranial Doppler ultrasound • Sturge-Weber syndrome • vascular

This version was published on February 1, 2008

Journal of Child Neurology, Vol. 23, No. 2, 137-143 (2008)
DOI: 10.1177/0883073807307079


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