Extracranial internal carotid arterial disease in children with sickle cell anemia

Abstract

BackgroundSickle cell anemia is one of the commonest causes of stroke in children. It is usually, but not always, associated with intracranial vasculopathy. We have assessed the value of ultrasound screening for extracranial internal carotid artery disease. Design and MethodsUsing Doppler ultrasound scanning, we assessed peak systolic blood velocity, tortuosity and stenosis in the extracranial internal carotid arteries of 236 children with sickle cell anemia. Seventeen of the children had previously had a stroke. All measurements were performed as part of routine clinical care. ResultsThe median extracranial internal carotid artery velocity was 148cm/s (5 th centile 84, 95 th centile 236). Higher velocities were significantly correlated with younger age, higher white blood cell counts and higher rates of hemolysis. Fourteen (5.9%) had tortuous extracranial internal carotid arteries and 13 (5.4%) had stenosis or occlusion. None of the children with tortuous vessels but 8 of those with stenosis had previously had a stroke; the presence of stenosis was strongly associated with overt clinical stroke (OR 35.9, 95% C.I. 9.77-132, P<0.001). In 6 children, extracranial stenosis was part of extensive intracranial vasculopathy, but in 2 there was no evidence of intracranial disease. Stenosis seemed to be more common in older children. ConclusionsExtracranial internal carotid artery stenosis is strongly associated with stroke in children with sickle cell anemia, and may explain some cases of stroke without overt intracranial vasculopathy. Doppler ultrasound scanning of extracranial internal carotid arteries is non-invasive and fairly quick to perform and may identify children at increased risk of stroke who would otherwise be missed. The value of extracranial internal carotid artery scanning should be studied prospectively.Key words: sickle cell anemia, internal carotid artery, Doppler ultrasound scanning.Citation: Deane CR, Goss D, Bartram J, Pohl KRE, Height SE, Sibtain N, Jarosz J, Thein SL, and Rees DC. Extracranial internal carotid arterial disease in children with sickle cell anemia. Haematologica 2010;95(8):1287-1292. doi:10.3324/haematol.2010 This is an open-access paper. Extracranial internal carotid arterial disease in children with sickle cell anemia

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

“…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.…”

“…Second, despite the apparently predominant involvement of the Circle of Willis in SCA cerebral vasculopathy, the extracranial portion of the internal carotid artery (eICA) can be the site of stenosis and/or occlusion and is also responsible for overt strokes and SCI. [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.…”

“…[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.…”

Cervical carotid artery disease in sickle cell anemia: clinical and radiological features

“…11 In another Doppler study, Deane et al identified 13 of 236 children with cICA stenosis or occlusion, and showed that children with these lesions had a 36-fold increased risk of stroke. 12 In both of these studies, the investigators recommended that routine cervical carotid Doppler screening should be offered in addition to TCD to children with SCD. In another study, Calviere et al reported the case of a 19-year-old patient presenting with acute stroke due to cICA occlusion.…”

“…[3][4][5][8][9][10] Recently, cervical arterial lesions have been reported in children with SCD undergoing cervical Doppler scanning. 11,12 After observing 3 patients presenting acutely with stroke and cICA occlusion, we decided to include cervical MRA (cMRA) in our routine neuroimaging protocol. We present 10 patients with cICA occlusion or stenosis and highlight clinical implications.…”