Tanaka Reiko, Fujita Yasuyuki, Ishibashi Hiasa Kana, Yumoto Yasuo, Hidaka Nobuhiro, Fukushima Kotaro, Wake Norio
Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
Case Rep Obstet Gynecol. 2011;2011:723467. doi: 10.1155/2011/723467. Epub 2011 Oct 27.
Klippel-Trenaunay syndrome (KTS) is a rare congenital disease, and extensive cutaneous hemangiomas and abnormal venous vessels are characteristic. In our case, to manage her pregnancy with KTS, whole-body MRA was performed before delivery. A 29-year-old woman was referred at 28 weeks because of prominent vulvovaginal varicosities due to KTS. At 35 weeks, hypertrophy and multiple venous varicosities of her leg as well as massive vulvovaginal varicosities became prominent with a normal coagulation profile. Systematic MRAs revealed hemangiomas and varicosities in the right leg, the lower abdomen, and the pubic region, while no obvious AVM was detected around the bronchial tube and spine. We decided to deliver her baby by cesarean section at 37 weeks under general anesthesia, and a healthy baby was delivered. No blood transfusion was required. Prophylaxis against thrombosis was performed after the operation. She was discharged with her baby. Her vulvovaginal varicosities shrunk considerably one month later.
克-特综合征（KTS）是一种罕见的先天性疾病，其特征为广泛的皮肤血管瘤和异常静脉血管。在我们的病例中，为了处理这位患有KTS的患者的妊娠情况，在分娩前进行了全身磁共振血管造影（MRA）。一名29岁女性在孕28周时因KTS导致的明显外阴阴道静脉曲张前来就诊。孕35周时，她腿部的肥大和多处静脉曲张以及大量外阴阴道静脉曲张变得更加明显，凝血指标正常。系统性MRA显示右腿、下腹部和耻骨区域有血管瘤和静脉曲张，而在支气管和脊柱周围未检测到明显的动静脉畸形（AVM）。我们决定在孕37周时在全身麻醉下为她进行剖宫产，产下一名健康婴儿。无需输血。术后进行了血栓预防。她和宝宝一起出院了。一个月后，她的外阴阴道静脉曲张明显缩小。
