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

Abstract

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.

Figure 1

Prominent hypertrophy and multiple venous varicosities of the patient's right leg at 35 weeks of gestation (a). (R: right, L: left). The massive vulvovaginal varicosities present at 35 weeks of gestation (b) shrunk one month later (c).

Figure 2

Results of MRA performed at 35 weeks of gestation. (a) Many hemangiomas and varicosities in the right leg can be seen. (b) Abnormal vessels near the anterior superior iliac spine, (c) abnormal varicosities in the right lower abdomen and upper pubic region, and (d) hemangioma near the inferior epigastric vein are also visible.