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 Savasta

Research output: Contribution to journalArticlepeer-review


Introduction: 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). Discussion: 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.

Original languageEnglish
JournalChild's Nervous System
Publication statusAccepted/In press - Jan 1 2019


  • Coiling
  • Ehlers-Danlos syndrome
  • Hyperlaxity
  • Kinking

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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