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Blood, 1 June 2001, Vol. 97, No. 11, pp. 3401-3404
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Correlation of abnormal intracranial vessel velocity,
measured by transcranial Doppler ultrasonography, with abnormal
conjunctival vessel velocity, measured by computer-assisted intravital
microscopy, in sickle cell disease
Anthony T. W. Cheung,
Paul Harmatz,
Ted Wun,
Peter C. Y. Chen,
Edward C. Larkin,
Robert J. Adams, and
Elliott P. Vichinsky
From the Departments of Medical Pathology and Internal
Medicine, University of California Davis School of Medicine, Davis, CA;
Departments of Gastroenterology and Hematology/Oncology, Children's
Hospital, Oakland, CA; Department of Bioengineering, University of
California San Diego, La Jolla, CA; and Department of Neurology,
Medical College of Georgia, Augusta, GA.
The Stroke Prevention Trial has confirmed that utilization of
transcranial Doppler ultrasonography (TCD), which examines blood flow
in large intracranial vessels, can identify children with sickle cell
disease (SCD) who are at high risk of developing a premature stroke. It
is not known to what extent the vasculopathy in SCD involves small
vessels and whether the abnormalities, if present, correlate with
large-vessel vasculopathy. Eighteen children with SCD were examined
with TCD to determine middle cerebral artery (MCA) velocity and
computer-assisted intravital microscopy (CAIM) to determine bulbar
conjunctival vessel velocity during the same visit for vasculopathy
correlation. High MCA velocity ( 200 cm/sec) was found by TCD in 4 patients who also showed abnormal conjunctival velocity (< 0.2 mm/sec
or intermittent trickle flow) by CAIM. Three patients had conditional
( 170 cm/sec and < 200 cm/sec) MCA velocity: 2 showed abnormal
(trickle) and 1 showed normal conjunctival velocity (1.9 mm/sec). One
patient with unmeasurable MCA velocity had abnormal (trickle)
conjunctival velocity. Of the remaining 10 patients who had normal MCA
velocity, 2 showed abnormal (0.05 mm/sec and 0.1 mm/sec) and 8 showed
normal conjunctival velocities (1.1-2.4 mm/sec). The MCA velocities
correlated significantly with bulbar conjunctival flow velocities
(P  .008, Fisher exact test). A correlation exists
between MCA (large-vessel) and conjunctival (small-vessel) flow
velocities. CAIM is a noninvasive quantitative technique that might
contribute to the identification of SCD patients at high risk of
stroke. Small-vessel vasculopathy might be an important pathological
indicator and should be further explored in a large-scale study.
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