Indirect and direct revascularization of ACTA2 cerebral arteriopathy: Feasibility of the superficial temporal artery to anterior cerebral artery bypass with posterior auricular artery interposition graft: Case report

Document Type

Article

Abstract

Mutations in the smooth muscle-specific isoform of alpha actin (ACTA2) cause smooth muscle dysfunction in arteries. This rare loss-of-function mutation may cause a diffuse occlusive cerebral arteriopathy, resulting in stroke. While ACTA2 arteriopathy is often described as moyamoya-like, it has a distinct phenotype characterized by dilation of the proximal internal carotid artery (ICA) and occlusion of the terminal ICA and proximal middle cerebral artery. Intracranial arteries have an abnormally straight course, often with small aneurysms. There is limited experience with revascularization procedures for ACTA2 arteriopathy, and the safety and efficacy of these procedures are unknown. In this paper the authors present a symptomatic 6-year-old patient with ACTA2 cerebral arteriopathy who underwent both indirect revascularization and direct cerebrovascular bypass. Postoperatively, the patient suffered an ischemic infarct in a neighboring vascular territory. While direct cerebrovascular bypass is technically feasible, patients with ACTA2 arteriopathy may be at increased risk for perioperative stroke compared with patients with moyamoya disease.

Publication Date

9-1-2016

Publication Title

Journal of Neurosurgery: Pediatrics

ISSN

19330707

E-ISSN

19330715

Volume

18

Issue

3

First Page

339

Last Page

343

PubMed ID

27176728

Digital Object Identifier (DOI)

10.3171/2016.3.PEDS15694

This document is currently not available here.

Share

COinS