The combination of congenital cystic adenomatoid malformation of the lung (CCAM) and hydrops fetalis entails compromised birth outcomes, and fetuses with a CCAM volume ratio (CVR) < 1. 6 are considered at high risk for the development of hydrops. A 33-year-old gravida 3, para 1, mother was admitted at 20+6 weeks of gestation owmg to a large fetal lung multilocular mass (macrocystic CCAM) originating in the right lower lobe and hydropic change indicated by skin edema and ascites. The overall size of the mass lesion corresponded to a CCAM volume ratio of 2.0. The mother underwent thoracoammotic shunting at 22+1 weeks of gestation, with successful dramage of the largest, second cyst. After the procedure, the hydrops resolved completely. The shunt continued to be functional throughout the remainder of the pregnancy; however, the CVR persisted at 0.6-0.8 because of residual multilocular cysts. The mother underwent cesarean delivery at 37+0 weeks of gestation. On the day of birth, the infant underwent right thoracotomy and CCAM resection. The infant survived; however, respiratory demise due to lung hypoplasia and the subsequent pulmonary hypertension was severe and refractory. Our experience suggests the curative effect of thoracoamniotic shunting for hydropic state in a fetus with macrocystic CCAM. However, the criteria for the treatment of multilocular macrocystic CCAM requires further discussion.
All Science Journal Classification (ASJC) codes
- Radiology Nuclear Medicine and imaging