BACKGROUND:

Klippel-Trenaunay syndrome is a neurocutaneous syndrome with vascular repercussion whose characteristic triad is a cutaneous vascular nevus, varicose veins and asymmetric soft tissue and bone hypertrophy, which affect one or more limbs, during pregnancy these malformations increase, with pelvic and intra-abdominal repercussion. In the world literature, fewer than 100 cases of complicated pregnancies with this syndrome have been reported.

OBJECTIVE:

The second case of complicated pregnancy with Klippel-Trenaunay syndrome treated in our institution is reported, given its high morbidity, due to the high risk of severe complications such as venous thromboembolism or excessive bleeding in the intrapartum period.

CLINICAL CASE:

A 16-year-old, with a Klippel-Trenaunay syndrome diagnosed at fifteen, sent to our unit, with a third-trimester pregnancy, for resolution of pregnancy. Preoperative studies were performed blood count, coagulation tests, reported without alterations; as well as Doppler ultrasound of the uterine segment, without an increase in vasculature. It is scheduled for caesarean section, which is performed without intraoperative complications, with total bleeding of 600 cc, with an increase in vascularity at the colon level. She remains in immediate postoperative surveillance for 48 hours in an intensive care unit, without presenting haemorrhagic or ischemic complications. She is discharged at 72 hours, with thromboprophylaxis, analgesic and antibiotic.

CONCLUSION:

Pregnancy in patients with this syndrome implies high morbidity and mortality, which can be prevented with multidisciplinary management, anticipating potential complications.