Management of a Klippel-Trenaunay syndrome in pregnant women with mega-cisterna magna and splenic and vulvar varices at birth: a case report

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare, sporadic and complex malformation characterized by the clinical triad of: (i) capillary malformation (port-wine stain); (ii) soft tissue and bone hypertrophy or occasionally, hypertrophy of one lower limb; and (iii) atypical lateral varicosity. The maternal and fetal risks associated with pregnancy in women with KTS are proportional to disease severity, which can be exacerbated by pregnancy. Complications include bleeding, disseminated intravascular coagulation, thromboembolic events and pain. Here, we report the case of a pregnant woman with KTS who had an uneventful pregnancy, labor and postpartum course, but had splenic and large vulvar vein varices. The obstetrical course of women with KTS varies. Management is largely conservative and multidisciplinary approaches form the mainstay for managing these patients based on their symptoms.