Klippel-Trenaunay-Type Syndrome in Pregnancy

Introduction: Klippel-Trenaunay-Type Syndrome (KTTS) is a rare congenital anomaly with variable expression and an unknown etiology characterized by capillary and venous malformations and hypertrophy of bone and soft tissue. Pregnancy has been rarely reported in patients with KTTS and since 1989 there have been only 13 case reports of pregnancy in women with KTTS reported in the literature. Concurrent pregnancy is associated with adverse perinatal outcomes. To the best of our knowledge this is the second reported, and largest, series of cases. Study Design: After a thorough review of the literature, the medical records of four obstetrical patients with KTTS were reviewed. Results: The obstetrical course of women with KTTS varies. Complications include bleeding, DIC, thromboembolic events, and pain. Conclusions: The maternal and fetal risks associated with pregnancy in women with KTTS are proportional to the severity of disease, which can be exacerbated by pregnancy. Thoughtful preconceptional counseling, along with methodical and systematic intrapartum and postpartum care are keys to reducing mortality and morbidity. Target Audience: Obstetricians & Gynecologists, Family Physicians Learning Objectives: After completion of this article, the reader should be able to recall that the Klippel-Trenaunay-Type Syndrome (KTTS) is rare; explain that it is associated with vascular malformations and hypertrophy of bone and limb which may be manifested at birth; and state that pregnancy reports are sparse but adverse pregnancy outcomes may include bleeding, DIC, thromboembolic events, and pain in the affected limb.