Yuheng Cui, Yang Liu, Guoliang Shi, Ning Wang, Yueqiao Xu, Xin Qu, Hao Zhao, Meng Qi, Weitao Cheng, Wenjin Chen, Lidan Jiang, Tao Liu, Rongcai Jiang
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.