Clinical variability in children with dolichoarteriopathies of the internal carotid artery
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Dolichoarteriopathies of the internal carotid artery (DICA) are frequent non-atheromatous anatomical changes in the general population. The etiology of DICA is still controversial: several hypotheses have been suggested, including an anomaly of embryological development, or a degenerative loss of elasticity of the vessel wall. DICA have been related to a wide spectrum of clinical presentations in adults, varying from asymptomatic forms to acute cerebrovascular events. However, to date, only a few pediatric cases have been reported.
Methods and results
We report seven patients with DICA, 6 males and 1 female, aged 3 to 13 years, presenting with variable clinical symptoms. Different imaging techniques, including color Doppler ultrasound and magnetic resonance angiography, were used to show loops and/or kinking of the ICA. Three of these patients received a diagnosis of Ehlers-Danlos syndrome (EDS).
This study highlights the clinical variability in pediatric patients with DICA. We emphasize the need for close clinical management of pediatric DICA. Finally, considering the long-term prognostic implications of EDS, we recommend specific testing in children with DICA and suspicious clinical signs of this pathology.
KeywordsKinking Coiling Hyperlaxity Ehlers-Danlos syndrome
The Authors wish to kindly thank Dr Laurene Marguerite Kelly for providing a comprehensive revision of the English grammar for this manuscript.
Compliance with ethical standards
Conflict of interest
None of the authors have any conflict of interest to disclose. We confirm that we have read the Journal’s position on issues concerning ethical publication and affirm that this report is compliant with those guidelines.
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