A case report of a pregnant woman with Klippel-Trenaunay-Weber syndrome

OKU Koki, TANIMURA Kenji, TAIRAKU Sinya, MAESAWA  Yoko, MORIZANE Mayumi, MORIMOTO Noriyuki, DEGUCHI Masashi, YAMADA Hideto

doi:10.11437/sanpunosinpo.68.93

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disorder characterized by extensive cutaneous vascular malformation, venous varicosities, hemangiomas, and hypertrophy of soft or bony tissue. We herein report a case of a pregnant woman complicated with KTWS. A 23-year-old woman (gravida 1, para 0) underwent anticoagulation therapy with low-dose aspirin and unfractionated heparin from the first trimester because of the presence of hemangiomas. We assessed the location of the hemangiomas by pelvic and spinal magnetic resonance imaging (MRI) at gestational age (GA) of 27 and 35 weeks. A cesarean delivery with spinal anesthesia was supposed to be done based on the initial assessment of hemangiomas at GA of 27 weeks. However, MRI at GA at 35 weeks revealed hemangiomas developed newly around the lumbar. We therefore performed a cesarean delivery under general anesthesia at GA at 37 weeks. During the operation, we examined the location of the hemangiomas in abdominal wall by transabdominal ultrasound and avoided injuring the hemangiomas. The patient delivered a 2518g healthy female infant and received anticoagulation therapy for six weeks after delivery, preventing thrombosis and embolism. In conclusion, anticoagulation therapy and decision of style and method of parturient based on the assessment of the location of hemangiomas by MRI, ultrasonography and bronchoscopy may enable safer perinatal management for pregnancies complicated by KTWS. [Adv Obstet Gynecol, 68 (2) : 93-98, 2016 (H28.5)]

A case report of a pregnant woman with Klippel-Trenaunay-Weber syndrome

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