Case of thoracoamniotic shunt placement for treatment of pleural effusion in the setting of congenital diaphragmatic hernia complicated by hydrops

PURPOSE:To review a case of novel in utero thoracoamniotic shunt placement for pleural effusion in the setting of congenital diaphragmatic hernia (CDH) A 37 yo G1P0 with known diagnosis of right CDH on growth scan at 20+1 weeks GA with moderate pleural effusion was evaluated for fetoscopic endoluminal tracheal occlusion (FETO) candidacy at 24+2 weeks GA.The US demonstrated normal growth with right CDH containing liver and gallbladder with moderate right pleural effusion.ECHO obtained with exaggerated levoposition due to right pleural effusion but otherwise normal structure and function.Thoracoamniotic shunt placement was recommended at that time but the patient declined due to concern for preterm delivery with a poor outcome.Repeat evaluation at 26+2 weeks GA demonstrated similar findings of normal growth and large pleural effusion with new small ascites.Worsening polyhydramnios with preterm contractions warranted urgent repeat evaluation at 29+3 weeks GA, and US demonstrated concern for hydrops fetalis with severe polyhydramnios, skin edema, large abdominal ascites, and worsened large pleural effusion.Patient underwent placement of a singular thoracoamniotic shunt at 29+4 weeks gestation and was hospitalized on tocolytics for five days post-procedure.Amniocentesis was obtained at that time and demonstrated a normal male karyotype of 46, XY.Following placement of the shunt, the patient was transferred under our care at 30+5 weeks GA for the remainder of pregnancy.Evaluation of the fetus was performed including fetal echocardiogram, serial ultrasounds with maternal fetal medicine (MFM), and consultation with the CDH team at Johns Hopkins All Children's Hospital (JHACH).Imaging at 34 weeks was consistent with a right-sided CDH containing bowel, liver, and gallbladder.There was no evidence of residual pleural effusion or ascites and the thoracoamniotic shunt remained in place for the duration of pregnancy.The CDH was categorized as moderate to less severe based on MRI with a 95% likelihood of survival and 5-30% ECMO risk based on protocolized treatment at JHACH.Patient was monitored regularly by ultrasound through the MFM office with planned delivery via scheduled cesarean at 37+3 weeks gestation.Per CDH protocol, two doses of antenatal corticosteroids were administered prior to delivery.The male infant was delivered via primary low transverse cesarean section with Apgar scores of 7 and 8 at 1 and 5 minutes respectively.Birthweight was noted to be 2770 g.Immediately following delivery, the infant was evaluated by the CDH team Case of thoracoamniotic shunt placement for treatment of pleural effusion in the setting of congenital diaphragmatic hernia complicated by hydrops