Extracranial carotid arteriopathy in stroke-free children with sickle cell anemia: detection by submandibular Doppler sonography

Abstract

Adding extracranial internal carotid artery evaluation via the submandibular window to transcranial Doppler sonograpy allowed us to detect 10.3% patients at risk for extracranial internal carotid arteriopathy. Further studies are needed to evaluate the prognosis of these anomalies.

Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia

“…Gorman et al, 21 using TCD assessment via a submandibular approach and neck MRA, identified 3 strokefree patients with SCA with eICA stenosis and SCI. We recently reported 24 that eICA velocities of 160 cm/s or higher are highly predictive of eICA stenosis, which were more frequently observed in tortuous arteries than in straight arteries, suggesting a causal mechanism similar to that involved in the carotid siphon stenosis. Turbulent flow secondary to increased cardiac and cerebral blood flow resulting from chronic anemia and decreased oxygen supply to the brain may induce shear stress gradients and subsequent endothelial dysfunction, especially in bended segments, leading to narrowing of the arterial lumen.…”

“…32 The relationship between cerebrovascular events and extracranial ICA stenosis had previously been suspected. [20][21][22][23][24] An eICA bulbar occlusion with a contralateral postbulbar eICA stenosis was reported in a 19-year-old man who suffered a stroke. 20 Telfer et al 23 reported 10 patients with eICA occlusion or stenosis among 67 patients with SCA assessed with MRI/MRA and neck MRA because of history of stroke or abnormal velocities.…”

“…Moreover, since June 2011, all patients with SCA had eICA assessment via a submandibular approach with the same low-frequency (2 Mhz) TCDI transducer, placed below the angle of the jaw and aimed cephalad. 24 Patients underwent cerebral MRI/MRA every 2 years after age 5 years to avoid the need for sedation or after 2 transfusions for those requiring a transfusion program. MRI/MRA protocol included fluid-attenuated inversion recovery, T1, T2, diffusion-weighted sequences and 3-dimensional time-of-flight angiography of the Circle of Willis, and the same expert (S.V.)…”

“…[19][20][21][22][23] Contrary to intracranial stenosis in the Circle of Willis, detectable by TCD via a temporal window and by intracranial magnetic resonance angiography (MRA), assessing eICA requires using a new submandibular approach 21 and cervical MRA. [19][20][21][22][23][24] We recently reported in 2 large cohorts of stroke-free SCA children that eICA velocities of 160 cm/s or higher were highly predictive of eICA stenosis. 24,25 We hypothesized that in addition to low baseline hemoglobin level, the rate of AAE and the presence of eICA stenosis could be additional risk factors for SCI.…”

“…[19][20][21][22][23][24] We recently reported in 2 large cohorts of stroke-free SCA children that eICA velocities of 160 cm/s or higher were highly predictive of eICA stenosis. 24,25 We hypothesized that in addition to low baseline hemoglobin level, the rate of AAE and the presence of eICA stenosis could be additional risk factors for SCI. Thus, to determine the association between eICA stenosis and SCI, we chose to study this issue in a longitudinal cohort, the Créteil Newborn Cohort, as patients are systematically assessed by MRI/MRA.…”