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Spinal Neurovascular Malformations in Klippel-Trenaunay Syndrome: A Single Center Study.

Authors
  • Larson, Anthony1
  • Covington, Tristan1
  • Anderson, Katelyn2
  • Tollefson, Megha2
  • Lanzino, Giuseppe1, 3
  • Brinjikji, Waleed1, 3
  • 1 Department of Radiology, Mayo Clinic, Rochester, Minnesota.
  • 2 Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
  • 3 Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota.
Type
Published Article
Journal
Neurosurgery
Publication Date
Feb 16, 2021
Volume
88
Issue
3
Pages
515–522
Identifiers
DOI: 10.1093/neuros/nyaa457
PMID: 33073841
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

A number of studies have demonstrated spinal anomalies associated with Klippel-Trenaunay syndrome (KTS). To date, there are no large consecutive series examining the prevalence and subtype distribution of spinal neurovascular malformations in patients with KTS. To report the spectrum and incidence of spinal neurovascular manifestations in the KTS population. This was a cross-sectional study. Consecutive patients with definite KTS as defined by International Society for the Study of Vascular Anomalies criteria who underwent spinal neuroimaging at our institution were included. All studies were evaluated by a staff neuroradiologist and a senior radiology resident for the presence of developmental venous anomalies, cavernous malformations (CMs), and arteriovenous shunts (AVS). A total of 116 patients with definite KTS who underwent spinal neuroimaging were included. A total of 23 neurovascular anomalies were found in 19 patients (16.4%), including 4 patients with multiple anomalies. These included 5 patients with spinal cord CMs (4.3%), 14 patients with a paraspinal or epidural venous malformation (12.1%), and 4 patients with an AVS (3.4%). Of the AVS, 3 were epidural arteriovenous fistulas, 1 of which likely formed de novo in an epidural venous malformation. One was a conus medullaris arteriovenous malformation. Our study cohort of 116 KTS patients demonstrated a wide spectrum of spinal neurovascular anomalies with a relatively high prevalence. Potential phenotypic descriptions of KTS should include the possibility for spinal neurovascular anomalies. Copyright © 2020 by the Congress of Neurological Surgeons.

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