Fetal Presentation Of Klippel-Trenaunay-Weber Syndrome With Massive Pleural Effusion And Ascites

Background: Although fetuses with Klippel-Trenaunay-Weber syndrome (KTS) show various morphological abnormalities on imaging studies, fetal presentation with hydrops fetalis is relatively uncommon in KTS.Case: A 28-year-old Japanese woman who had previously given birth to a healthy infant was referred to us at gestational week (GW) 22 due to huge pleural effusion and ascites. The possibility of fetal pulmonary hypoplasia prompted us to place bilateral thoracoamniotic shunts at GW 23 after extensive discussion with both parents. The bilateral shunts were effective in preventing recurrence of pleural effusion. However, ascites gradually increased and clinical signs of fetal cardiac failure necessitated cesarean section at GW 34. A male infant, weighing 4252 g at birth and 2860 g after removal of ascites, survived to the neonatal period and did not require oxygen after postnatal day 63. The infant left hospital on day 103 with a diagnosis of KTS.Conclusion: Fetuses with KTS may present with massive pleural effusion and ascites. Thoracoamniotic shunting may be effective in such hydropic fetuses with KTS.