Intracranial and extracranial vascular manifestations of patients with a clinical diagnosis of Klippel–Trenaunay syndrome

Tristan N. Covington, Katelyn R. Anderson, Megha M. Tollefson, Julie B. Guerin, Waleed Brinjikji

Research output: Contribution to journalArticlepeer-review


Background and purpose: While numerous reports have demonstrated intracranial CNS anomalies associated with Klippel–Trenaunay syndrome, to our knowledge, there has not been a large consecutive study examining these anomalies. The aim of this study was to determine the spectrum of intracranial neurovascular manifestations in patients with a clinical diagnosis of Klippel–Tranaunay syndrome. Methods: Consecutive patients with a clinical diagnosis of Klippel–Trenaunay syndrome, as defined by the International Society for the Study of Vascular Anomalies, who underwent brain contrast-enhanced CT/computed tomography angiography, MRI/magnetic resonance angiography, or digital subtraction angiography at our institution from 2000 to 2019 were included. Studies were evaluated by a neuroradiologist and a senior radiology resident for the presence of cavernous malformations, developmental venous anomalies, venous sinus developmental abnormalities, craniofacial venous malformations, intraosseous venous malformations, and intracranial/extracranial venous abnormalities. Results: Fifty patients with definite KTS were included. Thirty-four neurovascular anomalies were found in 17 patients (34.0%), including 8 with multiple anomalies. Nine patients had developmental venous anomalies (18.0%), 7 had craniofacial venous malformations (14.0), 6 had venous sinus developmental abnormalities (12.0%), 7 had intraosseous venous malformations (14.0%), and 2 had cavernous malformations (4.0%), and 9 patients had both intracranial venous abnormalities and craniofacial or calvarial findings (13.0%).

Original languageEnglish (US)
Pages (from-to)409-415
Number of pages7
Issue number3
StatePublished - Mar 2021


  • Congenital
  • Dural venous sinus
  • Klippel trenauny
  • Venous malformation

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine


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