Triple and quadruple cervical artery dissections: a systematic review of individual patient data

Valeria Guglielmi,Jonathan M Coutinho,Marcel Arnold,Stéphanie Debette,Paul J Nederkoorn,Alessandro Pezzini,Manja Kloss,David Calvet,René Van Den Berg,Didier Leys,Hakan Sarikaya,Sabrina Schilling,Turgut Tatlisumak,Jeldican Visser

doi:10.1007/s00415-019-09269-1

Abstract

Background and purposeSimultaneous dissection of three or four cervical arteries rarely occurs. As a result, limited information is available on clinical characteristics, underlying causes, treatment, and outcome of these patients.MethodsWe performed a systematic review of individual patient data on triple and quadruple cervical artery dissection (CeAD). We included all cases for whom, at minimum, data on age, sex and affected cervical arteries were available.ResultsOut of 1396 publications identified in the initial search, 52 were included, with data available on 96 patients. Mean age was 42 years and 66% were women. 63% had triple CeAD. The most common manifestations were headache (69%), neck pain (44%), motor deficit (36%), and Horner syndrome (34%). 57% had an ischemic stroke, in the majority of these patients the stroke was confined to the vascular territory of a single artery. 83% were managed medically (antiplatelets or anticoagulants) and 11% underwent endovascular treatment. An underlying disease or triggering event was identified in 71%, most commonly trauma (35%, cervical manipulative therapy in 13%), infection (18%), fibromuscular dysplasia (16%), and hereditary connective tissue disorder (8%). In-hospital mortality was 1%. 80% of patients had a good functional outcome (mRS 0–1) at follow-up. Two recurrences (3%) were reported.ConclusionsTriple or quadruple CeAD mostly affected young women, and underlying disease or triggering event could be identified in more than two-thirds of patients. Less than two-thirds of triple or quadruple CeAD patients suffered ischemic stroke. Most patients were managed medically and the majority had a favorable outcome.

Highlights

Cervical artery dissection (CeAD) accounts for approximately 20% of strokes in patients less than 45 years of age [1]
Triple or quadruple cervical artery dissection (CeAD) was considered simultaneous if the dissections were simultaneously present on baseline imaging of the cervical arteries, or if on baseline imaging an initial dissection was visible and additional dissections were diagnosed within 30 days
While CeAD was complicated by a stroke or TIA in the majority of patients, in-hospital mortality was low and clinical outcome was favorable in 80% of patients

Summary

Introduction

Cervical artery dissection (CeAD) accounts for approximately 20% of strokes in patients less than 45 years of age [1]. Because of the paucity of data on triple and quadruple CeAD, little is known about the clinical manifestations, underlying causes, treatment, and outcome of these patients. The aim of the current study was to perform a systematic review of individual patient data on triple and quadruple CeAD. Methods We performed a systematic review of individual patient data on triple and quadruple cervical artery dissection (CeAD). We included all cases for whom, at minimum, data on age, sex and affected cervical arteries were available. Conclusions Triple or quadruple CeAD mostly affected young women, and underlying disease or triggering event could be identified in more than two-thirds of patients. Most patients were managed medically and the majority had a favorable outcome

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