What is pulmonary hypoplasia?
Pulmonary hypoplasia is a condition in which the lungs are abnormally small, and do not have enough tissue and blood flow to allow the baby to breathe on his or her own. Sometimes this problem can be fatal.
Most often the pulmonary hypoplasia is a secondary problem due to another diagnosis. Either something is compressing the lungs or preventing the lungs from developing properly.
- In babies with congenital diaphragmatic hernia (CDH), the lungs are compressed by the abnormal position of the intestines in the chest.
- A baby with massive pleural effusions (fluid buildup around the lungs) can also have very small, underdeveloped lungs.
- In babies with bladder outlet obstruction (BOO) or severe oligohydramnios, there is very little amniotic fluid. The amniotic fluid puts pressure on the lungs to grow and without the pressure, pulmonary hypoplasia develops.
How is pulmonary hypoplasia diagnosed?
The diagnosis of pulmonary hypoplasia is based upon measurements taken from ultrasound and MRI images. We use measurements of lung volume and size to determine how small the fetal lungs are and whether the problem is life-threatening for the baby.
How is pulmonary hypoplasia treated?
Depending on the size of the fetal lungs and the cause of the pulmonary hypoplasia, different treatment plans can be recommended. When the problem is due to compression on the lungs, then fetal intervention can sometimes be used to remove the compression.
In some cases, such as with babies who have CDH, BOO, BPS (bronchopulmonary sequestration) or CCAM (Congenital Cystic Adnomatoid Malformation), fetal surgery can help reverse the effect of the pulmonary hypoplasia while the baby is still developing in the womb. If the primary condition is treated, the lungs are given the opportunity to develop, while still in the womb.
What can I expect if my baby is born with pulmonary hypoplasia?
Although the lungs continue to develop for up to a year after your baby is born, pulmonary hypoplasia does present some challenges at birth. Babies with pulmonary hypoplasia often have difficulty breathing and eating, and may have cardiac challenges as well. Some babies may also require ECMO immediately following birth to assist them with breathing. These challenges often require support in the NICU (Neonatal Intensive Care Unit) until the baby’s lungs can function on their own.