Clinical variability in children with dolichoarteriopathies of the internal carotid artery

  • Thomas Foiadelli
  • Rosario Ippolito
  • Riccardo Corbetta
  • Anna Maria Simoncelli
  • Rossella Amariti
  • Amelia Licari
  • Gianluigi Marseglia
  • Salvatore SavastaEmail author
Case Report



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.


Kinking 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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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Pediatric ClinicFondazione IRCCS Policlinico San Matteo, University of PaviaPaviaItaly
  2. 2.Vascular Surgery DepartmentFondazione IRCCS Policlinico San MatteoPaviaItaly
  3. 3.Department of Diagnostic Radiology and Interventional Radiology and NeuroradiologyUniversity of Pavia, Fondazione IRCCS Policlinico San MatteoPaviaItaly

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