The role of perinatal center on neonatal surgery for lung

Many congenital defects can now be detected before birth. Fetal anatomy, normal and abnormal, can be accurately delineated by prenatal ultrasound. Prenatal diagnosis and treatment has decreased mortality rate in some life-threatening thoracic malformations, such as congenital diaphragmatic hernia (CDH) and congenital cystic adenomatoid malformation (CCAM) of the lung.
Although less severely affected babies survive with modern postnatal surgical care, including extracorporeal membrane oxygenation support, many neonates witli CDH defect die despite all intervention because of underdeveloped (hypoplastic) lungs and associated pulmonary hypertension. These lesions, when first evaluated and treated poslnatally, demonstrate a favorable selection bias because the most severely affected fetuses often die in utero or immediately after birth. Salvage of these severely affected babies remains an unsolved problem. It has been shown experimentally that repair before birth, allowing the lungs to grow while the fetus remains on placental support, is physiologically sound and technically feasible. Fetal intervention may lie recommended in the fetuses of <32 weeks' gestation who in the poor prognosis group (herniated early in gestation, herniated liver, low lung-to-head ratio, severe mediastinal shift, dilated intrathoracic stomach). Presently, fetal intervention for CDH consists of endoscopic (FETENDO) tracheal occlusion to induce lung growth; the hernia is repaired postnatally.
Although CCAM often presents as a benign pulmonary mass in infancy or childhood, some fetuses with large lesions die in utero or at birth from hydrops or pulmonary hypoplasia, or both. Differences in the survival rate of patients with CCAM are related to the associated hydrops. The potentially fatal outcome with large CCAM lesions may also lie related to lung hypoplasia secondary to prolonged compression in utero. Most lesions can be successfully treated after birth, and that some lesions resolve or significantly regress before birth. Less than 10% of all fetuses with CCAMs can be successfully treated by emergency resection of the cystic lobe in utero. For lesions with a single large cyst, percutanous thoracoamniotic shunting may be successful.
Mild hydrothorax especially when unilateral is relatively benign. The diagnosis of severe pleural effusion, particularly bilateral once, before 32 weeks' gestation may be associated with considerable morbidity and mortality. A small number of these lesions may progress rapidly and cause lung hypoplasia secondary to prolonged compression. In such cases, if fetal needling fails, thoracoamniotic shunting may improve the outcome by preventing lung hypoplasia and hydrops.