• Jian Zhang, Kana Wang, and Jie Mei.
• Department of Gynecology and Obstetrics, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital.
• Medicine (Baltimore). 2019 Dec 1; 98 (50): e18378e18378.

IntroductionThe Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder. The obstetric course of women with KTS varies. Complications include bleeding, disseminated intravascular coagulation (DIC), thromboembolic events, etc. PATIENT CONCERNS:: Here, we report a case of late puerperal hemorrhage of a Chinese puerpera with KTS. The repeating severe hemorrhage, the DIC, and the Kasabach-Merrit syndrome made the treatment more difficult.DiagnosisKTS is a mixed malformation with a vascular component that is characterized by abnormal development of veins, capillaries, and lymphatics. Our patient was first diagnosed with KTS at the last trimester of pregnancy.InterventionsMassive infusion of blood products, two laparotomies, as well as bilateral internal iliac artery embolization was carried out.OutcomesAlthough the patient survived from the life-threatening hemorrhage, she lost her uterus forever.ConclusionAn interdisciplinary cooperation of obstetrician, anesthesiologist, vascular surgeon, and intensive care physician is highly recommended. Prophylactic anticoagulation is generally advised in the gestational and postpartum period.

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