Back to Journals » Neuropsychiatric Disease and Treatment » Volume 16

Profiles of Vertebral Artery Dissection with Congenital Craniovertebral Junction Malformation: Four New Cases and a Literature Review

Authors Xue S, Yang Y, Li P, Liu P, Du X, Ma X

Received 21 May 2020

Accepted for publication 3 August 2020

Published 22 October 2020 Volume 2020:16 Pages 2429—2447


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Dr Yuping Ning

Sufang Xue,1,* Yi Yang,1,* Pengyu Li,2 Ping Liu,3 Xiangying Du,2 Xin Ma1

1Neurology Department of Xuanwu Hospital, Capital Medical University, Beijing, People’s Republic of China; 2Radiology Department of Xuanwu Hospital, Capital Medical University, Beijing, People’s Republic of China; 3Neurology Department of Hejian People’s Hospital, Cangzhou, Hebei, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Xin Ma
Neurology Department of Xuanwu Hospital, Capital Medical University, Beijing, People’s Republic of China
, Tel +86-135-0139-0691

Objective: Vertebral artery dissection (VAD) combined with congenital craniovertebral junction malformation (CVJM) is rare. This study aimed to analyze the etiology, clinical and imaging features, treatment, and prognosis of VAD with CVJM.
Methods: Four new cases of VAD with congenital CVJM and 28 similar cases found in the literature were included. Detailed clinical data from all cases were retrospectively analyzed.
Results: A total of 32 patients (28 men, four women; mean age 19.01± 12.53 years) were included. Seventeen of 32 cases (53.1%) had had multiple ischemic episodes. The most common neurological symptoms were limb numbness/weakness (20/32), ataxia (15/32), and dizziness/vertigo (12/32). In sum, 31 of 32 cases had multiple infarcts scattered throughout the posterior circulation area on cranial computed tomography or resonance imaging. Dissection had occurred in the V3 segment of the VA in 29/31 cases (93.5%). The most common congenital CVJMs were atlantoaxial dislocation and atlantoaxial subluxation (found in 20/32 cases [62.5%]), while 27/32 cases (84.3%) had multiple combined abnormalities. Seven of eleven cases (63.6%) with initial antiplatelet treatment and one of eleven (9.1%) with initial anticoagulation treatment experienced stoke recurrence. Fusion or vertebral fixation was performed in 16 patients and aneurysm resection in one patient. There was no reported recurrence after surgery in 13 patients with follow-up data.
Conclusion: Underlying CVJM is a rare but overlooked etiology in VAD, and is prone to induce recurrent ischemic stroke. Patients with VAD, especially that localized in the V3 segment, should be examined for CVJM. Timely assessment is critical for determining the specific cause and to provide targeted intervention.

Keywords: vertebral artery dissection, craniovertebral junction malformation, clinical and imaging features, treatment, prognosis

Creative Commons License This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at and incorporate the Creative Commons Attribution - Non Commercial (unported, v3.0) License. By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms.

Download Article [PDF]  View Full Text [HTML][Machine readable]