OBJECTIVE: Our purpose was to review our experience with fetal congeni
tal cystic adenomatoid malformation and extralobar pulmonary sequestra
tion emphasizing natural history, management, and outcome. STUDY DESIG
N: We conducted a retrospective review of 175 fetal lung lesions diagn
osed by antenatal ultrasonography at 2 fetal treatment centers. RESULT
S: There were 134 congenital cystic adenomatoid malformationcases. Fou
rteen women underwent elective abortion, 101 women were managed expect
antly 13 women had fetal surgery, and 6 women had placement of a thora
coamniotic shunt. For the congenital cystic adenomatoid malformation l
esions that were not associated with nonimmune hydrops, all babies sur
vived. Of 25 large congenital cystic adenomatoid malformations that ha
d associated hydrops that were followed expectantly, all fetuses died
before or shortly after birth. Fetal surgical resection of the tumor (
fetal lobectomy) was performed at 21 to 29 weeks' gestation in 13 hydr
opic fetuses with 8 fetuses continuing gestation with subsequent hydro
ps resolution, impressive in utero lung growth, and neonatal survival.
Six fetuses with a very large solitary cyst underwent thoracoamniotic
shunting and 5 survived. There were 41 extralobar pulmonary sequestra
tion cases. Twenty-eight extralobar pulmonary sequestrations dramatica
lly regressed on serial prenatal sonography, were asymptomatic after b
irth, and were only detectable by imaging studies postnatally (no rese
ction required). Of the remaining 13 extralobar pulmonary sequestratio
n cases, 2 underwent elective abortion, 7 symptomatic lesions were res
ected after birth with survival, 1 hydropic fetus died, and 3 fetuses
had an associated tension hydrothorax with secondary hydrops that was
successfully treated by either fetal thoracenteses or thoracoamniotic
shunting followed by postnatal resection. CONCLUSIONS: The natural his
tory of prenatally diagnosed lung masses is variable, and associated a
nomalies are rare. Most congenital cystic adenomatoid malformation les
ions can be managed with maternal transport, planned term delivery, an
d postnatal resection. Many extralobar pulmonary sequestrations dramat
ically decrease in size before birth and may not need treatment after
birth. Fetal therapy is now an option for lung lesions associated with
nonimmune hydrops.