The French connection - Extracranial internal carotid artery and cerebral infarction in pediatric sickle cell patients

Sep 30, 2026

Editorial commentary on Bernaudin et al.’s single-center prospective pediatric cohort in sickle cell disease, focusing on extracranial internal carotid artery (eICA) flow and stenosis as determinants of stroke and silent cerebral infarction. Chronic transfusion therapy introduced in 2011 prevented stroke for children with markedly increased eICA flow or eICA stenosis, while hydroxyurea for “conditional” eICA flow avoided eICA stenosis but did not halt silent infarct progression. The report is presented without randomized causality, prompting questions about optimal strategies to prevent SCI progression beyond hydroxyurea.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages2

Reading Time5 min

AccessFree to download

The French connection: pediatric sickle cell and cerebrovascular outcomes

Conditional eICA flow and treatment strategy

Findings on stroke prevention and SCI progression

Limits of evidence and clinical implications

References

EDITORIAL The French connection: extracranial internal carotid artery and cerebral infarction in pediatric sickle cell patients Bart J. Biemond Department of Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands Correspondence: B.J. Biemond [b.j.biemond@amsterdamumc.nl](b.j.biemond@amsterdamumc.nl) [Received:](Received: July 10)[ July 10](Received: July 10), 2025. Accepted: August 11, 2025. Early view: August 21, 2025. [https://doi.org/10.3324/haematol.2025.288350](https://doi.org/10.3324/haematol.2025.288350) ©2026 Ferrata Storti Foundation Published under a CC BY-NC license In their article published in this issue of Haematologica, Bernaudin et al 1 present extensive data on their single center prospective pediatric cohort of sickle cell disease (SCD) patients. These patients have been meticulously followed since 1998, with changing screening and treatment practices over the years, with a special focus on the extracranial part of the carotid internal artery (eICA) in the pathogenesis of stroke and silent cerebral infarction. In previous studies, high flow in the eICA or stenosis had been demonstrated to be an additional risk factor for stroke and silent cerebral infarction (SCI) in patients with normal transcranial Doppler (TCD) and magnetic resonance imaging (MRI) / magnetic resonance angiography (MRA) of the intracranial vasculature.2 Therefore, in this cohort, chronic transfusion therapy was introduced in 2011 to prevent stroke in pediatric SCD patients with these findings. The novelty of this report is the assessment of the impact of “conditional” increased blood flow (blood flow >160 cm/sand < 200 cm/s) in the eICA, which has been routinely analyzed in this center since 2011, a practice which has not been implemented in all centers around the world. Given the increased risk of developing an eICA stenosis with a“conditional” eICA flow, patients with this conditional flow and no stenosis of the eICA were given hydroxyurea (if not already prescribed) in order to prevent further deterioration. In this paper, the authors present their data by comparing the efficacy of this strategy introduced in 2011 in the “new cohort”(defined as pediatric SCD patients born after 2007) in comparison with their initial reported cohort (born before 2007), and the results are very interesting. The first major finding is that chronic transfusion therapy in patients with either an increased eICA flow (>200 cm/s) or stenosis of the eICA successfully prevented stroke in this specific patient group, similar to what has been observed in SCD patients with abnormal TCD or stenosis of the intracranial vessel in severe pediatric SCD patients. The application of TCD, MRI/MRA of the intracranial vessels in combination with Doppler assessment of the extra-cranial carotid artery and MRI/MRA of neck was demonstrated to almost completely prevent stroke in children with severe SCD. This observation confirms that evaluation of the eICA flow and the presence of stenosis in the extracranial artery should be implemented in the routine Doppler and MRI check-ups in severe pediatric SCD patients. Another major finding was that patients with “conditional”flow (160-200 cm/s) in the eICA treated with hydroxyurea did not develop extracranial carotid artery stenoses but appeared to experience continuous progression of silent cerebral infarct (SCI) lesions, resulting in an incidence of SCI at the age of 14 years of 25% . These findings are not based on a randomized controlled trial and causality is, therefore, not proven in this prospective cohort study. However, these observations clearly suggest that the extracranial carotid arteries play an important role in the pathogenesis of stroke, which, in cases of high flow (>200 cm/s) or stenosis in the eICA, can be prevented by chronic transfusion, and SCI progression, which cannot be prevented by hydroxyurea. The question remains: what should be done to prevent progre

FAQs

What was the central focus of Bernaudin et al.’s prospective pediatric cohort?

The study examined how extracranial internal carotid artery (eICA) flow and stenosis relate to stroke and silent cerebral infarction in pediatric sickle cell disease patients, with patients followed since 1998 and screened using TCD, MRI/MRA, and Doppler assessments.

How did chronic transfusion therapy affect stroke risk in the reported patient groups?

Chronic transfusion therapy successfully prevented stroke in children with either increased eICA flow (>200 cm/s) or eICA stenosis, aligning with prior observations in severe pediatric sickle cell disease patients with abnormal TCD or intracranial vessel abnormalities.

What happened to silent cerebral infarction (SCI) in patients with “conditional” eICA flow treated with hydroxyurea?

Patients with conditional eICA flow (160–200 cm/s) treated with hydroxyurea did not develop eICA stenoses, but they showed continuous progression of SCI lesions, with an SCI incidence of 25% by age 14.

Sep 30, 2026

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