Congenital pulmonary adenomatoid malformation: Indication and management of fetal shunting in our center

Objective: Congenital pulmonary adenomatoid malformations (CPAM) are increasingly diagnosed in recent years thanks to the expert hands and the technology of ultrasonography used in antenatal diagnosis. The purpose was to examine a series of cases referred to our fetalmaternal tertiary center for lung disease and to present the natural course, treatment and outcome of antenatally diagnosed cystic lung disease. Material and Methods: We proposed a retrospective study (October 2003 to September 2016) in a single center. Prenatal and postnatal data of fetus, with suspected CPAM, where analyzed. Results: We studied 34 cases. One child was excluded post-natally because at birth diagnosis of esophageal duplication was made. Mean gestational age at the diagnosis was 22 weeks (range 21 to 28). Thoracoamniotic shunting was performed in 9 cases. In 8 cases, it was an intra-cystic drain and in 1 case, a pleuroamniotic drain. Mean gestational age for drainage was 25 weeks (range 22 to 28). Mean gestational age at delivery was 38 weeks. All patients underwent postnatal radiological assessment. Anatomical surgical resection was performed in all cases: immediately at birth in 7 symptomatic cases, in the first year of life for 1 case that became symptomatic and 1 patient died immediately at birth due to severe respiratory distress. Conclusion: Antenatally diagnosed CPAM have an excellent prognosis. We propose invasive prenatal thoracoamniotic shunting in rare cases with unequivocal fetal compromise (i.e., polydramnios and hydrops)