Zhang Jian, Wang Kana, Mei Jie
Department of Gynecology and Obstetrics, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital.
Department of Gynecology and Obstetrics, West China Second University Hospital and Sichuan University, Chengdu, Sichuan, China.
Medicine (Baltimore). 2019 Dec;98(50):e18378. doi: 10.1097/MD.0000000000018378.
The 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.
KTS 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.
Massive infusion of blood products, two laparotomies, as well as bilateral internal iliac artery embolization was carried out.
Although the patient survived from the life-threatening hemorrhage, she lost her uterus forever.
An 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.
克-特综合征（KTS）是一种罕见的先天性疾病。患有KTS的女性的产科病程各不相同。并发症包括出血、弥散性血管内凝血（DIC）、血栓栓塞事件等。
在此，我们报告一例患有KTS的中国产妇晚期产后出血的病例。反复的严重出血、DIC和卡萨巴赫-梅里特综合征使治疗更加困难。
KTS是一种伴有血管成分的混合畸形，其特征是静脉、毛细血管和淋巴管发育异常。我们的患者在妊娠晚期首次被诊断为KTS。
进行了大量输血、两次剖腹手术以及双侧髂内动脉栓塞。
尽管患者从危及生命的出血中存活下来，但她永远失去了子宫。
强烈建议产科医生、麻醉师、血管外科医生和重症监护医生进行多学科合作。一般建议在妊娠期和产后进行预防性抗凝。
