**5.1. Gastroschisis**

count to exclude an infectious etiology [68]. Complications of shunts include displacement or malfunction of the catheter, thrombus occlusion of the catheter, fatal fetal hemorrhage, procedure-related abruptio placentae, premature rupture of membranes, and preterm labor [68]. There is also a risk of trauma to the fetal chest wall, especially if the procedure is

• In-utero open resection, percutaneous laser ablation of the feeding vessel has been successfully performed in several small studies [68]. Percutaneous ultrasound-guided fetal sclero-

If the lung mass has resolved or is small with no mediastinal shift or hydrops, BPS itself is not an indication for early delivery or cesarean delivery [68]. Neonatal respiratory problems would be unlikely. For fetuses with large masses that cause mediastinal shift and/or hydrops, delivery should be planned for a tertiary care center with an intensive care nursery capable of resuscitation of a neonate with respiratory difficulties, including capability of extracorporeal membrane oxygenation (ECMO), and with pediatric surgeons experienced in care of these infants [68]. If hydrops develops after 32 weeks of gestation, early delivery is recommended, possibly with the use of EXIT. In EXIT, the fetus is partially delivered and intubated without clamping the umbilical cord. Uteroplacental blood flow and gas exchange are maintained by using inhalational agents to provide uterine relaxation and amnioinfusion to maintain uterine volume. This provides time for initiating extracorporeal membrane circulation to stabilize the infant, thus

therapy has also been described [68]. Sometimes two procedures were necessary.

creating a controlled situation before resection of BPS in another operating room [68].

Congenital lobar emphysema (CLE) is a rare congenital malformation and sometimes is detected by prenatal ultrasonography. Lung lesions have increased echogenicity and/or a cystic appearance and usually can be differentiated from other congenital lung lesions [70]. A chest mass may even disappear on prenatal ultrasound and become apparent again on postnatal evaluation [70]. Predictors of severe respiratory distress or mortality include polyhydramnios, fetal hydrops, and lung to thorax transverse area ratio (L/T value) of less than 0.25 [70]. Approximately 25% of cases present at birth, 50% by 1 month of age, and nearly all by 6 months of age. Infants typically have tachypnea and increased work of breathing and often have cyanosis. Recurrent pneumonia or poor feeding with failure to thrive are less frequent

Any fetus with suspected bilateral pulmonary agenesis should have a detailed sonographic assessment to confirm the diagnosis. If the diagnosis is made till periviable pregnancy, the pregnancy termination is an option. If the diagnosis is made at a later gestational age, the

delivery should be planned without monitoring for fetal distress [16].

performed before 20 weeks [68].

488 Congenital Anomalies - From the Embryo to the Neonate

*4.3.2. Delivery management*

**4.4. Congenital lobar emphysema**

**4.5. Pulmonary agenesis**

presentations that may occur in milder forms [70].
