Maternal Klippel – Trenaunay syndrome with complete hydatidiform mole and coexistent fetus: A case managed by surgical evacuation with review of literaturee

Case Report

Ahmed Samy El-Agwany

Department of Obstetrics and Gynecology, Faculty of Medicine , Alexandria University, Egypt

Correspondence: Ahmed S El-Agwany El-shat by Maternity University Hospital, Faculty of Medicine, Alexandria University, Alexandria, Egypt, Tel: 00201228254247, E – mail:


Introduction: Klippel-Trenaunay Syndrome (KTS) is a rare triad of congenital vascular malformations involving extensive Port wine stains, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation affecting limb, pelvic or abdominal organs. Pregnancies with a hydatidiform mole and a live fetus are extremely rare, arising in about 1 in 20,000 to 100,000 pregnancies. Aim: We report a case of a pregnant woman with KTS with twin pregnancy (one fetus and a coexistent mole) with its management dilemma. Patient: A 27 years old patient, G2P1, NVD, was admitted in our hospital with bleeding per vaginum. Uterus was 24 weeks. USG showed single live intrauterine fetus with 14 weeks gestational age with normal placenta with another placenta with molar changes with adnexa free. β HCG level on admission was 220,000 mIu/ml. Results: After thorough counseling, the pregnancy was terminated by patient’s desire. Induction of abortion with misoprostol was done but severe bleeding occurred mandated surgical evacuation with vaccum aspiration. Histopathological examination confirmed vesicular mole with normal fetus. Her β hCG reached normal levels at the end of five weeks, and she is now on post-molar surveillance for one year. Conclusion: Termination of pregnancy in twins with coexistent mole is recommended especially far from age of viability. KTS and molar pregnancy may be associated as both are vascular abnormalities that need further evaluation.

Keywords: Klippel-Trenaunay Syndrome, pregnancy, twin, ultrasound, complete mole, termination, surgical evacuation
p. 56-63