Pulmonary Defects are problems in the lung.
- Congenital Cystic Adenomatoid Malformation (CCAM) most common
- Pulmonary Sequestration
- Congenital Lobar Emphysema
Diagnosis
- Any of these lung masses are usually discovered during a prenatal ultrasound of the fetus (unborn baby). The mass may be fluid filled or solid.
- Color flow Doppler ultrasound may be used to differentiate the diagnosis
- Sometimes a fetal MRI is done to confirm the diagnosis.
- Occasionally, the lung mass will resolve while the mother is pregnant.
- Sometimes, a small lung mass can go undiscovered until later in childhood when an infection occurs.
Causes
The exact cause is unknown. The mass in the chest can cause poor lung growth in the fetus, resulting in breathing and heart problems at birth. Survival rate is as high as 90% when fetal hydrops has not developed. Hydrops is retention of fluid in the body causing severe complications. The high risk obstetrician will closely monitor the fetus with ultrasound for hydrops and other signs of complications.
Treatment
- The mass will need to be surgically removed by a pediatric surgeon. The time of the surgery depends on how the baby is breathing at birth.
- The baby will be closely monitored by a neonatologist (doctor who specializes in newborns) at birth. An x-ray and or chest CT will be done to confirm the diagnosis.
- In severe cases, a ventilator or a heart lung bypass machine might be required and surgery might be done sooner.
- If the baby is not having breathing problems, the baby can go home and be followed up with the pediatric surgeon at a few weeks of age. A follow up X-Ray and or chest CT will be done. The pediatric surgeon will decide when surgery is appropriate.
- It is important that the mass be surgically removed because there is risk of infection and malignancies if it is not removed.