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Published on: June 3, 2021
Transcranial Doppler ultrasound in children with Sturge-Weber syndrome
Lori C Jordan1, Robert J Wityk, Michael M Dowling
1Department of Neurology, The Johns Hopkins University School of Medicine, USA.
Insights
Transcranial Doppler ultrasound reveals reduced cerebral blood flow velocity and increased pulsatility in children with Sturge-Weber syndrome. These findings suggest potential venous stasis and hypoperfusion, warranting further investigation.
Area of Science:
- Pediatric Neurology
- Vascular Neurology
- Neuroimaging
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder associated with neurological deficits.
- Stroke risk is elevated in pediatric SWS, necessitating effective monitoring strategies.
- Transcranial Doppler (TCD) ultrasound is a noninvasive tool for assessing cerebral blood flow.
Purpose of the Study:
- To evaluate the utility of Transcranial Doppler (TCD) ultrasound in assessing cerebral hemodynamics in children with Sturge-Weber syndrome.
- To compare blood flow velocities and pulsatility indexes between affected and unaffected sides of the brain in pediatric SWS patients.
Main Methods:
- Eight children diagnosed with Sturge-Weber syndrome underwent Transcranial Doppler (TCD) ultrasound.
- Velocities and pulsatility indexes were measured in major cerebral arteries (MCA, PCA) using the Stroke Prevention Trial in Sickle Cell Anemia methodology.
- Data from affected and unaffected cerebral hemispheres were compared, and correlations with MRI findings were analyzed.
Main Results:
- All subjects exhibited reduced blood flow velocity on the affected side, with a mean Middle Cerebral Artery (MCA) velocity difference of 20%.
- An increased pulsatility index was observed on the affected side, with a mean MCA pulsatility index difference of 34%.
- Reduced Posterior Cerebral Artery (PCA) velocity was noted in 6 subjects, and side-to-side velocity differences correlated with MRI asymmetry.
Conclusions:
- The observed pattern of decreased arterial flow velocity and increased pulsatility index in pediatric SWS suggests a high-resistance cerebral circulation.
- This hemodynamic pattern may indicate venous stasis and contribute to chronic hypoperfusion in brain tissue.
- Further research utilizing Transcranial Doppler ultrasound is recommended for children with Sturge-Weber syndrome to better understand and manage cerebrovascular complications.
Abstract:
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 rho = 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.
