{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,11]],"date-time":"2026-07-11T13:33:31Z","timestamp":1783776811813,"version":"3.55.0"},"reference-count":11,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2016,9]]},"abstract":"<jats:p>Mutations in the smooth muscle\u2013specific isoform of alpha actin (<jats:italic>ACTA2<\/jats:italic>) cause smooth muscle dysfunction in arteries. This rare loss-of-function mutation may cause a diffuse occlusive cerebral arteriopathy, resulting in stroke. While <jats:italic>ACTA2<\/jats:italic> 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 <jats:italic>ACTA2<\/jats:italic> arteriopathy, and the safety and efficacy of these procedures are unknown. In this paper the authors present a symptomatic 6-year-old patient with <jats:italic>ACTA2<\/jats:italic> 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 <jats:italic>ACTA2<\/jats:italic> arteriopathy may be at increased risk for perioperative stroke compared with patients with moyamoya disease.<\/jats:p>","DOI":"10.3171\/2016.3.peds15694","type":"journal-article","created":{"date-parts":[[2016,5,13]],"date-time":"2016-05-13T15:00:34Z","timestamp":1463151634000},"page":"339-343","source":"Crossref","is-referenced-by-count":17,"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"],"prefix":"10.3171","volume":"18","author":[{"given":"W. Caleb","family":"Rutledge","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omar","family":"Choudhri","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian P.","family":"Walcott","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnau","family":"Benet","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine K.","family":"Fox","sequence":"additional","affiliation":[{"name":"Neurology, and"},{"name":"Pediatrics, University of California, San Francisco, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nalin","family":"Gupta","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"},{"name":"Pediatrics, University of California, San Francisco, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael T.","family":"Lawton","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-peds15694","series-title":"J Neurointerv Surg","doi-asserted-by":"publisher","first-page":"e46","DOI":"10.1136\/neurintsurg-2013-010997.rep","article-title":"Cerebral arteriopathy associated with Arg179His ACTA2 mutation","volume":"6","author":"Amans","year":"2014"},{"key":"b2-peds15694","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1016\/j.ajhg.2009.04.007","article-title":"Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease","volume":"84","author":"Guo","year":"2009"},{"key":"b3-peds15694","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"927","DOI":"10.3171\/2009.4.JNS081649","article-title":"Clinical outcome after 450 revascularization procedures for moyamoya disease. 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