title: 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.
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.
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: W Caleb
authors.initials: WC
authors.lastname: Rutledge
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Omar
authors.initials: O
authors.lastname: Choudhri
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Brian P
authors.initials: BP
authors.lastname: Walcott
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Arnau
authors.initials: A
authors.lastname: Benet
authors.affiliation: Neurology, and.|Pediatrics, University of California, San Francisco, California.
authors.forename: Christine K
authors.initials: CK
authors.lastname: Fox
authors.affiliation: Departments of 1 Neurological Surgery.|Pediatrics, University of California, San Francisco, California.
authors.forename: Nalin
authors.initials: N
authors.lastname: Gupta
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Lawton
mesh_terms: D000199:Actins / Q000235:genetics*; D020771:Anterior Cerebral Artery / Q000601:surgery; D002545:Brain Ischemia / Q000000981:diagnostic imaging / Q000209:etiology / Q000235:genetics; D002539:Cerebral Arterial Diseases / Q000000981:diagnostic imaging / Q000235:genetics* / Q000601:surgery*; D002548:Cerebral Revascularization / Q000009:adverse effects* / Q000379:methods*; D002648:Child; D004423:Ear / Q000098:blood supply; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D009154:Mutation; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000235:genetics; D013699:Temporal Arteries / Q000601:surgery
chemical_list: C541116:ACTA2 protein, human; D000199:Actins
keywords: ACA = anterior cerebral artery; ACTA2; AP = anteroposterior; ECA = external carotid artery; ICA = internal carotid artery; MAP = mean arterial pressure; MCA = middle cerebral artery; MIFA = middle internal frontal artery; PAA = posterior auricular artery; STA = superficial temporal artery; cerebral arteriopathy; cerebrovascular bypass; indirect revascularization; stroke; vascular disorders