LAUSR

Abstract Rationale: Klippel–Trenaunay Syndrome (KTS) is a congenital vascular disease characterized by cutaneous hemangiomas, venous varicosities, and limb hypertrophy. Although extremely rare in pregnant women, the present vascular alterations may be aggravated, consequent to postural and hormonal changes inherent to the pregnancy. Pregnancy is not advised in KTS women due to increased obstetrical risk. Patient concerns: A 31-year-old pregnancy woman presented with prominent vascularity in pelvis, right lower limb, spleen, and liver at 28 weeks of gestation. We started administration of anticoagulant therapy and obstetrics management. Diagnosis: MRI and ultrasound revealed that multiple varicosities in her pelvis, right lower limb, spleen, and liver. She was diagnosed with KTS. Interventions: At her first visit at 28 weeks of gestation, multidisciplinary evaluation had been done. Blood transfusion and iron supplement had been given for anemia correction. Anticoagulant therapy was performed to prevent potential thrombus risk. She had a vaginal delivery with a healthy newborn in her second visit without any complications at the gestation of 36+6 weeks due to rupture of preterm membranes. Outcomes: After successful management, the patient was discharged without any complications 2 days after vaginal delivery. No symptoms of hemorrhage or thrombus were observed. At 6 months follow-up, her right lower toes enlarged obviously, MRI revealed that no obvious changes of hemangiomas was found compared to those during the pregnancy and ultrasound revealed that there was no thrombus in her right lower limb. Lessons: Patients with KTS can be pregnant and have healthy babies safely with regularly monitor and reasonable treatment during pregnancy. A careful follow-up and guidance are necessary.