Most fetuses with pulmonary sequestrations do not require intervention during the pregnancy and will do well with management after birth (usually surgery to remove the abnormal piece of lung). Fetuses with pulmonary sequestrations, identified by an abnormal artery on ultrasound, usually do well with surgical removal after birth.
A few fetuses develop fluid collections in the chest cavity which may be treated by placing a Harrison catheter shunt to drain the chest fluid into the amniotic fluid. A few fetuses with this type of pulmonary sequestration that has large cystic spaces can be treated by draining the cysts into the amniotic fluid using a Harrison pleuro-amniotic shunt catheter.
If the fetus has a very large lesion that will make resuscitation after delivery dangerous, a specialized delivery can be planned called the EXIT (ex utero intrapartum treatment) procedure. We developed the EXIT procedure for management of fetal airway emergencies, but it can be adapted to facilitate immediate ECMO support or surgical resection of very large lesions while the baby remains attached to the placenta. This is rarely necessary for pulmonary sequestrations.
Fetuses with small or moderate-sized pulmonary sequestrations that do not change much during pregnancy can be successfully managed after a normal birth. They usually do not have any difficulty during pregnancy or after birth. Unlike congenital cystic adenomatoid malformation (CCAM), all pulmonary sequestrations should be surgically removed because of the high blood flow through the lesion that can cause heart failure. This surgery is quite safe even in the first year of life and does not compromise lung function or the normal development of the baby. These children will grow up normally and have normal lung function.
The innovative procedures at FTC saved the lives of both Angela and Guy, whom were both diagnosed with severe CCAMs.