An unusual case of Kippel-Trenaunay syndrome complicated with intrahepatic cholestasis of pregnancy and disseminated intravascular coagulation

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder characterized by capillary malformation, varicose veins, and limb hypertrophy. Women with KTS face increased risks of thromboembolism and hemorrhage during pregnancy, delivery, and the postpartum period. Intrahepatic cholestasis of pregnancy (ICP), the most common pregnancy-related liver disorder, is associated with adverse perinatal outcomes. However, coexistence of KTS and ICP has not been previously reported. We describe the case of a 29-year-old pregnant woman with known KTS who developed ICP in the third trimester. She was managed with ursodeoxycholic acid and prophylactic anticoagulation. Despite therapy, her serum bile acids remained elevated, and fetal monitoring revealed decelerations, prompting cesarean delivery at 35 weeks of gestation. The newborn was preterm but otherwise healthy. Postpartum, the patient experienced progressive coagulopathy with markedly elevated D-dimer levels, superficial vein thrombosis, and clinical signs of disseminated intravascular coagulation (DIC). With adjustment of anticoagulation and administration of fresh frozen plasma and fibrinogen, her coagulation profile gradually normalized. She was discharged on rivaroxaban and remained stable during follow-up. This case highlights the complexity of managing pregnancy in women with KTS complicated by ICP. The coexistence of two high-risk conditions poses challenges in balancing thrombosis and bleeding risks, as well as safeguarding fetal outcomes. Multidisciplinary care, close monitoring, and individualized anticoagulation strategies are essential to optimize maternal and neonatal prognosis.