Management of prenatally diagnosed congenital cystic adenomatoid malformation of the lung

We have treated four prenatally diagnosed cases of extensive congenital cys tic adenomatoid malformation (CCAM) of the lung. The first case in 1982 was associated with severe fetal hydrops. After thoracentesis at 31 weeks of g estation abruptio placentae occurred, and a female baby was delivered by ce sarean section, She underwent a right lower lobectomy, but soon died, The s econd baby without hydrops, diagnosed as having CCAM at 26 weeks of gestati on, was followed conservatively until full term. After birth, it was necess ary to treat the baby boy with extracorporeal membrane oxygenation (ECMO), but he survived. The third baby with fetal hydrops had an indwelling draina ge catheter inserted into the CCAM at 27 weeks of gestation. The hydrops su bsided and the baby was delivered at 37 weeks of gestation. He was allowed to breathe spontaneously, but was intubated 16 hours after birth. A right l ower lobectomy was successfully performed 24 hours after delivery. The four th baby without fetal hydrops was followed conservatively until delivery. H e underwent left lower lobectomy successfully on the 4th day of life. Altho ugh management of prenatally diagnosed CCAM varies among patients, insertio n of an indwelling catheter into the cyst appears to be the treatment of ch oice if indicated; the catheter can be maintained for as long as 10 weeks, as shown in Case 3. Cases of CCAM without fetal hydrops should also be trea ted carefully, because persistent fetal circulation may occur postnatally.