Pregnancy in Women With Klippel-Trenaunay Syndrome Complicated by Uterine Vascular Malformation: A Case Report and Literature Review.

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder characterized by the classical triad of cutaneous port-wine stain (capillary hemangioma), varicose veins, and hypertrophy of soft tissues and bones. KTS with uterine involvement is extremely uncommon. We present a 27-year-old pregnant woman with KTS complicated by uterine venous malformation. Comprehensive management was provided by a multidisciplinary team after 12 weeks gestation diagnosed with KTS. The patient wore full-length compression stockings and received low-molecular-weight heparin (LMWH) for thromboprophylaxis. At 34 weeks gestation, imaging studies, including colour Doppler ultrasound and pelvic MRI, were performed to evaluate venous malformations throughout the myometrium. Given varicose veins affecting the lumbar region and vagina, an elective cesarean section under general anesthesia was performed at 37 weeks. The patient's total blood loss of 2200 mL. An infant weighing 2,940 g was delivered with Apgar scores of 8/9/10. Postoperatively, prophylactic LMWH 5000 IU once daily was resumed, and based on marked elevations in fibrin degradation products (FDP), plasmin-antiplasmin complex (PIC), D-dimer, and thrombin-antithrombin (TAT), along with a decline in fibrinogen, adjusted LMWH 5000 IU twice daily for six weeks. This case highlights the challenges of managing pregnancy in women with KTS complicated by uterine venous malformation.