{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T04:46:35Z","timestamp":1785732395733,"version":"3.56.0"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T00:00:00Z","timestamp":1785715200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>CLOVES syndrome is driven by somatic activating PIK3CA mutations that constitutively activate the PI3K-AKT-mTOR pathway, resulting in segmental overgrowth and complex vascular malformations. Emerging evidence suggests these vascular lesions are biologically active and may pre-dispose to localized intravascular coagulation and thrombosis. We report a 21-year-old man with genetically confirmed CLOVES syndrome who developed concurrent consumptive coagulopathy and hypercoagulability. Post-operatively, he experienced both hemorrhagic and thromboembolic complications, culminating in fatal cardiac arrest. This case highlights a fragile perioperative hemostatic balance in CLOVES syndrome, underscoring the need for careful risk stratification and multidisciplinary management.<\/jats:p>","DOI":"10.3389\/fmed.2026.1883591","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T04:27:49Z","timestamp":1785731269000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: When benign overgrowth turns fatal in CLOVES syndrome: underestimated perioperative risk"],"prefix":"10.3389","volume":"13","author":[{"given":"Yuheng","family":"Cui","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yang","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Tianjin Medical University General Hospital","place":["Tianjin, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoliang","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ning","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yueqiao","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xin","family":"Qu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hao","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meng","family":"Qi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weitao","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenjin","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lidan","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tao","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rongcai","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Xuanwu Hospital, Capital Medical University","place":["China"]},{"name":"Department of Neurosurgery, Tianjin Medical University General Hospital","place":["Tianjin, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,8,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","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 A."},{"key":"B2","doi-asserted-by":"publisher","first-page":"2508","DOI":"10.1016\/j.jhsa.2013.08.120","article-title":"CLOVES syndrome","volume":"38","author":"Bloom","year":"2013","journal-title":"J Hand Surg Am."},{"key":"B3","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1097\/MCD.0b013e328317a716","volume":"18","author":"Alomari","year":"2009","journal-title":"Characterization of a distinct syndrome that associates complex truncal overgrowth, vascular, and acral anomalies: a descriptive study of 18 cases of CLOVES syndrome. 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