Pregnancy with Klippel-Trenaunay Syndrome: A Critical Case Report

Klippel-Trenaunay Syndrome (KTS) is a rare congenital disease characterized by a triad of cutaneous hemangioma (port-wine stain), varicose veins and bone or soft tissue hypertrophy. Cases of pregnancy complicated by KTS are rare and are associated with an increased risk of thrombo-embolic phenomena and hemorrhage. In this case, 33 years old woman, Para 1 gravida 2 (P1G2) with history of previous cesarean section and diagnosed case of KTS was presented in labour emergency of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh at 36+ weeks of gestation with labour pain. She had also a prominent hypertrophy and multiple venous varicosities on both her lower limbs; more marked on left. Her MRI done just before pregnancy revealed few prominent veins in both adnexa particularly on the left with normal caliber ovarian veins and unremarkable other pelvic structures and excludes pelvic congestion syndrome. USG done at her 35 weeks of gestation had shown numerous dilated and tortuous vessels in the parametrium, indicating pelvic congestion. Emergency caesarean section was done under spinal anesthesia. There was no postpartum hemorrhage (PPH). She was discharged along with her baby without any complication on her 4th postoperative day. Successful management of patients with KTS requires multidisciplinary team approach.