Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

Waleed Brinjikji, Vivek N. Iyer, Giuseppe Lanzino, Kent R. Thielen, Christopher P. Wood

Research output: Contribution to journalArticle

2 Scopus citations

Abstract

BACKGROUND AND PURPOSE: Brain capillary vascular malformations (CVMs) are known to occur with relatively high frequency in hereditary hemorrhagic telangiectasia (HHT) patients. These lesions are thought to have a benign natural history but this has not been systematically studied. The purpose of our study was to examine the natural history of CVMs in a consecutive series of HHT patients.

MATERIALS AND METHODS: Consecutive patients with untreated CVMs receiving serial imaging were included. Baseline data including demographics, HHT gene mutations, and Curacao diagnostic criteria were collected. The primary outcome was rupture on follow-up. A secondary outcome was new focal neurological deficit or seizure related to the lesion.

RESULTS: 22 patients with 42 CVMs were included. Mean age was 45.9±16.9 years. 18 patients (81.8%) were women and 4 (18.2%) were men. 19 patients (86.4%) had definite HHT and 3 patients (13.6%) had probable HHT. Mean follow-up was 4.6±3.7 years. There were a total of 100.2 patient years of follow-up and 222.5 lesion years. No lesions ruptured on follow-up and no patient had focal neurological deficits or seizures related to the lesions.

CONCLUSIONS: Our study found that CVMs in HHT patients have a benign natural history as no patients had hemorrhage or other symptoms related to these lesions. These findings should be confirmed in additional multicenter longitudinal studies.

Original languageEnglish (US)
Pages (from-to)26-28
Number of pages3
JournalJournal of NeuroInterventional Surgery
Volume9
Issue number1
DOIs
StatePublished - Jan 1 2017

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Keywords

  • Arteriovenous Malformation
  • Genetic
  • Hemorrhage

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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