**6.7 Cystic pulmonary airway malformations (CPAM)**

Most prenatally detected lung lesions are cystic pulmonary airway malformations (CPAM), broncho-pulmonar sequestrations or so called 'hybrid' lesions, containing features of both. The outcome of most lesions are favorable even without pre-natal intervention, despite often impressive appearance at mid-gestation. During pregnancy many lesions may regress, or disappear completely. Therefore, non-operative treatment (watchful waiting) is preferred by most fetal surgeons. Surprisingly, pressure effect or hemo-dynamic changes may cause sudden physiologic derangements, which may end with progressive heart failure and intrauterine demise. Therefore, pre-natal intervention may be warranted to improve outcome. Pre-natal interventions for fetal lung lesions aim to alleviate the pressure effect of the mass by partial or complete removal of the lesion. Many surgical and non-surgical options have been reported.

• In macrocystic lesions, needle thoraco-centesis or thoraco-amniotic shunt drainage under ultrasound guided may be used for decompression.


Routine ultrasound used as screening method for detection of congenital lung lesions and require referral to a specialist center. Other co-existing problems of the fetus should be carefully evaluated to determine, the magnitude of related complications, delivery place, time and type, and if intra-uterine intervention is needed. Minimally invasive intra-uterine fetal intervention for severe lesions can greatly improve the prognosis of these fetuses. In a large study of thoraco-amniotic shunt placement for congenital lung mass or pleural effusion, performed on 75 fetuses at Children's Hospital of Philadelphia, they showed 55% decrease in congenital cystic adenomatoid malformation volume and 27% of cases showed complete drainage of pleural effusion (73% showed partial drainage of effusions) with hydrops resolution in 83% of fetuses (43/53), which was greatly correlated with survival. Survival to delivery was 93% (70/75), median gestational age was 36 weeks, with 68% (51/75) long-term survival rate. Fifty-six percent of fetuses were delivered at an average of 10 weeks after shunt placement. Duration of stay in the neonatal intensive care unit of 21 days, with for greater than 24 hours. This series affirms the survival benefit risk patients, but underscores the risks inherent to in utero intensive neonatal therapy required [79, 80].
