Sickle cell anemia: Intracranial stenosis and silent cerebral infarcts in children
with low risk of stroke
Arkuszewski, M [ 1,2 ]; Krejza, J [ 3,4 ]; Chen, R [ 3 ]; Ichord, R [ 5,6 ]; Kwiatkowski, JL [ 7 ];
Bilello, M [ 1 ]; Zimmerman, R [ 8 ]; Ohene-Frempong, K [ 7 ]; Melhem, ER [ 3 ]
ADVANCES IN MEDICAL SCIENCES
Volume: 59, Issue: 1, Pages: 108-113, DOI: 10.1016/j.advms.2013.09.001, Published:
MAR 2014
Publisher MEDICAL UNIV BIALYSTOK, UL KILINSKIEGO 1, BIALYSTOK, 15-089,
POLAND
ISSN: 1896-1126
eISSN: 1898-4002
Abstract
Purpose: Children with sickle cell anemia (SCA), who have mean blood flow velocities
<170 cm/s in the terminal internal carotid (tICA) or middle cerebral (MCA) arteries on
transcranial Doppler ultrasonography (TCD), are considered to be at low risk of stroke.
The prevalence of intracranial stenosis, which raises the risk of stroke, is not known in
these children. Here, we estimated the prevalence of stenosis and explored its
association with silent cerebral infarcts determined based on Magnetic Resonance (MR)
scans.
Patients/methods: We studied prospectively a cohort of 67 children with SCA without
prior clinically overt stroke or TIA (median age 8.8 years; range limits 2.3-13.1 years; 33
females) and with TCD mean velocity <170 cm/s. They underwent MR imaging of the
brain and MR angiography of intracranial arteries.
Results: In 7 children (10.5%, 95% CI: 4.9-20.3%) we found 10 stenoses, including 4 with
isolated left tICA stenosis and 3 with multiple stenoses. We found silent infarcts in 26
children (37.7%, 95% CI: 27.2-49.5%). The median number of infarcts in an affected child
was 2 (range limits: 1-9), median volume of infarcts was 171 mm(3) (range limits: 7-1060
mm(3)), and median infarct volume in relation to total brain volume was 0.020% (range
limits: 0.001-0.101%). The number and volume of infarcts were significantly higher in
children with arterial stenosis (both p = 0.023).
Conclusions: The prevalence of intracranial arterial stenosis in children with SCA
classified as at low risk of stroke by TCD mean velocity <170 cm/s is high. Children with
stenosis are at higher risk of brain parenchymal injury as they have more silent cerebral
infarcts. (C) 2014 Medical University of Bialystok. Published by Elsevier Urban & Partner
Sp. z o.o. All rights reserved.
Keywords
Author Keywords:Sickle cell disease; Stroke risk; Children; Transcranial Doppler; Silent
cerebral infarct; Intracranial stenosis
KeyWords Plus:TRANSCRANIAL DOPPLER ULTRASONOGRAPHY;
MAGNETIC-RESONANCE ANGIOGRAPHY; CENTRAL-NERVOUS-SYSTEM;
CONVENTIONAL ANGIOGRAPHY; CEREBROVASCULAR-DISEASE; PREVENTION
TRIAL; IMAGING FINDINGS; FLOW-VELOCITY; BRAIN; MRI
Author Information
Reprint Address: Krejza, J E-mail Addresses:jkrejza@me.com
Univ Maryland, Med Ctr, Dept Diagnost Radiol & Nucl Med, 22 S Greene St, Baltimore,
MD 21201 USA.
Organization-Enhanced Name(s)
University System of Maryland
University of Maryland Baltimore
Addresses:
[ 1 ] Univ Penn, Dept Radiol, Div Neuroradiol, Philadelphia, PA 19104 USA
Organization-Enhanced Name(s)
University of Pennsylvania
[ 2 ] Med Univ Silesia, Dept Neurol, Katowice, Poland
Organization-Enhanced Name(s)
Medical University Silesia
[ 3 ] Univ Maryland, Med Ctr, Dept Diagnost Radiol & Nucl Med, Baltimore, MD 21201
USA
Organization-Enhanced Name(s)
University System of Maryland
University of Maryland Baltimore
[ 4 ] King Khalid Univ, Abha, Saudi Arabia
Organization-Enhanced Name(s)
King Khalid University
[ 5 ] Childrens Hosp Philadelphia, Dept Neurol, Philadelphia, PA 19104 USA
Organization-Enhanced Name(s)
Childrens Hospital of Philadelphia
University of Pennsylvania
[ 6 ] Childrens Hosp Philadelphia, Dept Pediat, Philadelphia, PA 19104 USA
Organization-Enhanced Name(s)
Childrens Hospital of Philadelphia
University of Pennsylvania
[ 7 ] Childrens Hosp Philadelphia, Dept Hematol, Philadelphia, PA 19104 USA
Organization-Enhanced Name(s)
Childrens Hospital of Philadelphia
University of Pennsylvania
[ 8 ] Childrens Hosp Philadelphia, Dept Radiol, Philadelphia, PA 19104 USA
Organization-Enhanced Name(s)
Childrens Hospital of Philadelphia
University of Pennsylvania