{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:06:36Z","timestamp":1753877196601,"version":"3.41.2"},"reference-count":7,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2021,4,19]],"date-time":"2021-04-19T00:00:00Z","timestamp":1618790400000},"content-version":"vor","delay-in-days":18,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>CLOVES syndrome is a condition characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies. The association of cervical arteriovenous fistula with CLOVES syndrome is extremely rare. To the best of our knowledge, only two cases were reported in the literature. We hereby report an additional case and review the literature on this condition. A 12-year-old girl, known to have CLOVES syndrome, presented to the emergency department with a 2-week history of bilateral, progressive lower extremity and left arm weakness. Radiological imaging demonstrated a C3\u2013C6 left extradural lesion, containing multiple enhancing vessels, causing marked compression on the spinal cord. Urgent spinal decompression was performed, with C3\u2013C6 instrumentation, followed by a two-stage embolization of the fistula. Cervical arteriovenous fistula can be rarely identified in patients with CLOVES syndrome. High index of suspicion is required to promptly evaluate patients with CLOVES syndrome presenting with new-onset neurological deficits.<\/jats:p>","DOI":"10.1093\/jscr\/rjab122","type":"journal-article","created":{"date-parts":[[2021,3,13]],"date-time":"2021-03-13T04:13:24Z","timestamp":1615608804000},"source":"Crossref","is-referenced-by-count":1,"title":["CLOVES syndrome and cervical arteriovenous fistula: a unique association managed by combined microsurgical and endovascular therapy"],"prefix":"10.1093","volume":"2021","author":[{"given":"Alhanouf","family":"Alhazzab","sequence":"first","affiliation":[{"name":"College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Alkhaibary","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center, Riyadh, Saudi Arabia"},{"name":"Division of Neurosurgery, Department of Surgery, King Abdulaziz Medical City, Ministry of National Guard \u2013 Health Affairs, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sami","family":"Khairy","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center, Riyadh, Saudi Arabia"},{"name":"Division of Neurosurgery, Department of Surgery, King Abdulaziz Medical City, Ministry of National Guard \u2013 Health Affairs, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wael","family":"Alshaya","sequence":"additional","affiliation":[{"name":"College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center, Riyadh, Saudi Arabia"},{"name":"Division of Pediatric Neurosurgery, Department of Pediatric Surgery, King Abdullah Specialist Children's Hospital, King Abdulaziz Medical City, Ministry of National Guard \u2013 Health Affairs, Riyadh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,4,19]]},"reference":[{"key":"2021070811401701800_ref1","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1097\/ANC.0000000000000342","article-title":"An extremely rare disorder of somatic mosaicism: CLOVES syndrome","volume":"16","author":"Anderson","year":"2016","journal-title":"Adv Neonatal Care"},{"key":"2021070811401701800_ref2","doi-asserted-by":"crossref","first-page":"1108","DOI":"10.1016\/j.ajhg.2012.05.006","article-title":"Somatic mosaic activating mutations in PIK3CA cause CLOVES syndrome","volume":"90","author":"Kurek","year":"2012","journal-title":"Am J Hum Genet"},{"key":"2021070811401701800_ref3","doi-asserted-by":"crossref","first-page":"2944","DOI":"10.1002\/ajmg.a.32023","article-title":"Newly delineated syndrome of congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) in seven patients","volume":"143","author":"Sapp","year":"2007","journal-title":"Am J Med Genet Part A"},{"key":"2021070811401701800_ref4","doi-asserted-by":"crossref","first-page":"2688","DOI":"10.1002\/ajmg.a.32515","article-title":"Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome: CNS malformations and seizures may be a component of this disorder","volume":"146","author":"Gucev","year":"2008","journal-title":"Am J Med Genet Part A"},{"key":"2021070811401701800_ref5","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1016\/j.jocn.2016.09.001","article-title":"Combined endovascular and microsurgical treatment of a complex spinal arteriovenous fistula associated with CLOVES syndrome in an adult patient","volume":"34","author":"Boroumand","year":"2016  1","journal-title":"J Clin Neurosci"},{"key":"2021070811401701800_ref6","doi-asserted-by":"crossref","first-page":"10730","DOI":"10.1073\/pnas.162349799","article-title":"Adipose tissue mass can be regulated through the vasculature","volume":"99","author":"Rupnick","year":"2002","journal-title":"Proc Natl Acad Sci U S A [Internet]"},{"key":"2021070811401701800_ref7","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1097\/BPO.0000000000000351","article-title":"Surgical treatment of spinal deformity in patients with CLOVES syndrome: a report of 4 cases","volume":"35","author":"Hedequist","year":"2015","journal-title":"J Pediatr Orthop [Internet]"}],"container-title":["Journal of Surgical Case 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