Two Cases of Congenital Cystic Lung Diseases (CCLDs) With Fetal Hydrops, for Which Thoracoamniotic Shunting Was Performed

Abstract

A CVR (CPAM volume ratio) of >1.6 is considered to be associated with a high risk of fetal hydrops and poor prognosis. We experienced treating two cases of CCLD with fetal hydrops or cavity effusion (CVR > 1.6), for which thoracoamniotic shunting was performed in the prenatal period. Both cases were rescued by a multidisciplinary approach that included emergency surgery after birth. Patient 1 underwent thoracoamniotic shunting at 25 weeks of gestation. After birth, we performed chest drainage and cyst drainage because of pneumothorax and the expansion of the cyst. At 17 days of age, we performed right upper lobectomy. Patient 2 underwent thoracoamniotic shunting at 22 weeks of gestation. We performed emergency cystectomy immediately after birth owing to poor cardiac hemodynamics. At 8 months of age, we performed right middle lobectomy and segmental resection of the lower lobe. Thoracoamniotic shunting for CCLD with fetal hydrops can markedly improve the prognosis. However, multidisciplinary therapy is often required in the neonatal period, and medical support is needed after hospital discharge. In addition to the prognosis, the patient’s quality of life (QOL) should be considered when deciding the treatment for CCLD with fetal hydrops.