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Successful Management of Pregnancy Complicated by Klippel-Trenaunay Syndrome Using MR Angiography-Based Evaluation

机译:基于MR血管造影的评估成功管理合并Klippel-Trenaunay综合征的妊娠

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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.
机译:Klippel-Trenaunay综合征(KTS)是一种罕见的先天性疾病,其特征是广泛的皮肤血管瘤和静脉血管异常。在我们的病例中,为了控制她的KTS妊娠,在分娩前进行了全身MRA。一名28岁的妇女因KTS引起的明显的阴道阴道静脉曲张而在28周时被转诊。在第35周时,她的腿肥大和多处静脉曲张以及大量外阴阴道曲张变得很突出,并且凝血功能正常。系统性MRA显示右腿,小腹和耻骨区的血管瘤和静脉曲张,而在支气管和脊柱周围未发现明显的AVM。我们决定在全麻下第37周通过剖宫产术分娩婴儿,并分娩出健康的婴儿。无需输血。术后预防血栓形成。她带着孩子出院了。一个月后,她的外阴静脉曲张明显缩小。

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