RESUMO
BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a vascular malformation overgrowth syndrome characterized by capillary malformation, venous malformation, and limb overgrowth, with or without lymphatic malformation. Patients are at an increased risk of hemorrhage and venous thromboembolism (VTE). Consequently, women with this condition often are counseled to avoid pregnancy, but minimal data are available on the relationship between pregnancy, VTE, and bleeding risk. OBJECTIVE: To review the risk of VTE and bleeding in pregnant and nulligravid women with KTS. METHODS: A retrospective medical record review was performed of women with KTS, aged ≥18 years, evaluated at Mayo Clinic Rochester, Minnesota, from August 1945 to April 2018. RESULTS: We identified 75 women with ≥1 pregnancy and 64 nulligravid women. VTE prevalence was 14 of 70 (20%) for women with a history of pregnancy and 16 of 64 (25%) for nulligravid women (P = .93). Among the 70 women with a history of pregnancy, 7 of 18 VTE events (39%) occurred in association with pregnancy, with VTE affecting 7 of 151 pregnancies (4.6%). Significant bleeding prevalence was 6 of 70 (8.6%) for women with a history of pregnancy and 6 of 64 (9.4%) for nulligravid women (P = .54). LIMITATIONS: This was a retrospective review. CONCLUSION: The prevalence of VTE and bleeding was similar in patients with KTS, irrespective of pregnancy status.