Prenatal ultrasounds are used to monitor the size of the lesion, and to check fetal well-being. Additional antenatal testing may be used depending on maternal and fetal factors. More than 90 percent of babies with prenatally diagnosed lung lesions will not have any fetal problems. 

Close monitoring is important to ensure the fetus does not develop heart failure which is called hydrops. This is when fluid collects within the skins and around the heart, lungs and intestines of the baby. These very sick babies have a large lesion as measured by the CVR. 

Some large lesions can compress the esophagus, impair swallowing, and lead to increased fluid levels around the baby which is called polyhydramnios. The increased fluid in the uterus can cause preterm labor.

Video: Prenatal Care for a Bay with CPAM?

We are one of a few centers in the country that can provide the full range of fetal interventions to improve the outcome of your baby.

Maternal steroids (most common): We give the mother two injections of steroid medication for large microcystic lesions and/or signs of hydrops early in pregnancy. Maternal steroids appear to prevent onset of with large lesions and diminish the effects of hydrops if it is present. This therapy is now considered the standard of care of large lesions.

Thoracoamniotic shunts: A CPAM that has a main large cyst causing fetal hydrops can be treated with a shunt. A shunt is a tiny tube used to drain fluid, in this case, from the cyst to the amniotic fluid. The shunt is placed under ultrasound guidance and stays in place to drain the cyst thereby decreasing the size of the mass.

Fetal lobectomy: Fetal surgery to remove the CPAM (lobectomy) while the baby is still in the womb is rare. It is reserved for those babies with hydrops that have not responded to steroids. In these cases, an incision is made in the mother’s abdominal wall, and then the uterus is opened. The lesion is then surgically removed through a chest incision in the baby. The baby is returned to the womb to continue the pregnancy. Preterm labor is common.

Ex utero intrapartum treatment (EXIT to Resection): If maternal steroids were used, and the lesion remains very large, some patients benefit from fetal surgery at the end of pregnancy. The baby is partially delivered and prepared for surgery while still attached to the placenta. The malformation is removed while the baby remains connected to the umbilical cord. After surgery is completed, the baby is delivered. Sometimes, a special heart-lung machine known as ECMO may be required temporarily to allow the lungs to recover and adjust to life outside the womb. The neonatal team assists the baby with the transition to extra uterine life. Learn more about EXIT procedures.

Video: What are the treatment options for a baby with CPAM who develops hydrops?

The final delivery plan for babies with a lung malformation should be addressed at a specialized center with experience in the natural history of these lesions. Larger malformations (CVR>1.0) have an increased risk of respiratory problems at birth and the need for surgical removal in the newborn period.

Delivery at the University of Michigan is recommended close to your delivery date. The University of Michigan is one of only a few centers nationwide at which the birth center(link is external) is co-located within a comprehensive, free-standing children’s hospital. This unique setting allows for seamless integration between our private-room birth center, state-of-the-art newborn intensive care unit (NICU), and access to around-the-clock pediatric surgical services in one convenient location.

Lesions that are smaller (CVR<1.0) are less likely to cause problems at birth. You may have the option to delivery closer to home if that is your preference. Small lung lesions are at low risk of causing any breathing problems or needing lung surgery within the first few weeks of life.

Unless there is a special birth plan, a vaginal delivery is recommended. A cesarean section is reserved solely for obstetrical indications.

Video: Delivery of a baby with CPAM

These malformations should be removed on an elective basis before complications occur. After the baby is born, you will meet with one of our pediatric surgeons to review how your baby is doing and make plans to remove the malformation. Follow-up with one of our pediatric surgeons is recommended around 3 months of age. A CT scan is scheduled before the visit with the surgeon to better define the lesion and help with surgical planning.

For babies who have breathing problems, the lesion needs to be excised urgently through a chest incision on the affected side of the baby. In newborn babies with very large lesions causing pulmonary hypoplasia and pulmonary hypertension, supplemental oxygen, mechanical ventilation, or ECMO support may also be required until the remaining lungs recover. Because some of these children will have chronic lung disease, they are followed long-term in our newly established Aerodigestive Clinic, a multidisciplinary effort that includes specialists from pediatric surgery, pulmonary, and otolaryngology.

If lung surgery is recommended, this is typically performed between 3 and 6 months of age. In these cases, we obtain a chest x-ray as a baseline study until a high-resolution CT scan of the chest can be performed to fully characterize the lung lesion. A brief period of general anesthesia during the study is usually required. Immediately following the study, you will see one of our pediatric surgeons in clinic so that a formal recommendation can be made regarding the need to remove the lung malformation.

Since 2006, our pediatric surgeons have been removing lung malformations on a routine basis using state-of-the-art, minimally invasive surgery techniques. To date, about 90% of asymptomatic lung malformations have been successfully removed in this fashion with excellent outcomes. Instead of traditional methods that require a large chest incision with spreading of the ribs to perform the operation, our surgeons generally use minimally invasive surgical techniques requiring only three or four tiny incisions (each about the width of an eraser head). The surgeon then uses a thin telescope to look inside the chest and delicately detached the lung lesion from vital structures using special instruments. The malformation is then cut into pieces so that it can be removed through one of the tiny incisions. Usually, one entire lobe of the lung is removed (lobectomy).

The average recovery time in the hospital is about 4 days with minimal scarring (picture). Children have normal lung function once they have fully recovered from surgery and have no restrictions on activities. Recent studies have shown that children’s lungs can grow quite rapidly during early childhood to compensate for the missing tissue.

Video: Why does a CPAM need to be removed if there are no symptoms?