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OBJECTIVES: Klippel-Trenaunay Syndrome (KTS) is a congenital vascular anomaly characterized by a triad of varicose veins, cutaneous capillary malformations, and hypertrophy of bone and soft tissue.
METHODS: A series of three female patients are presented, initially diagnosed at the ages of 14, 26 and 30 years and followed for eight months, 14 years, and 22 years, respectively. The changes during their lives will be presented along with the lessons learned.
RESULTS: Hormonal changes of menarche were associated with significant upper extremity symptoms and development of multiple varying-age DVTs and SVTs in a 14-year-old patient previously undiagnosed. Gestational and infertility issues resulted in significant AVM development with bleeding from a kidney which required multiple transfusions, cessation of anticoagulation, resulting in fetal loss at 21 weeks. Subsequently, the patient has been successfully managed through pregnancy and delivery. Intracranial AVMs in a patient with a significant hypercoagulable state have been successfully managed for over 20 years.
CONCLUSIONS: Patients with KTS have a known increased risk of hypercoagulability with an incidence of thromboembolic complications estimated to be as high as 22%; postoperative thromboembolism may be as high as 10 times that seen in non-KTS patients. Vascular malformations may be a source of platelet sequestration and fibrinogen consumption, with a bleeding diaphysis ensuing as a result of chronic localized intravascular consumptive coagulopathy. This may progress to disseminated intravascular coagulation following trauma, surgical intervention, or bedside procedures.
The incidence of genitourinary (GU) manifestations in KTS patients is 30%, with more than 52% of those requiring intervention for GU complications. New lesions may develop during pregnancy, requiring frequent monitoring and altering anesthetic choices for labor and delivery. Varicosities of the pelvis, central nervous system, and spine must be evaluated before regional anesthetic is undertaken or before a vaginal delivery is contemplated as cerebral varicosities may preclude a vaginal delivery. Fetal growth and wellbeing must be frequently monitored throughout pregnancy.
The obstetrical course of KTS women is variable; the severity of the disease may be exacerbated by pregnancy. Hormonal changes during the life cycle of the KTS patient will alter the disease process and pain complaints. Increased awareness and anticipation of potential problems are necessary for the team that undertakes successful management of KTS patients.