Pulmonary Hypoplasia

What is pulmonary hypoplasia?

Pulmonary hypoplasia is a congenital (present at birth) condition where your child’s lungs didn’t develop as expected during pregnancy. It’s almost always caused by an underlying health condition that doesn’t leave room for lung growth. Sometimes, a healthcare provider detects pulmonary hypoplasia during pregnancy and, other times, it’s diagnosed at or shortly after birth.

Pulmonary hypoplasia can lead to problems in the rest of your body. The small air sacs (alveoli) around your lungs deliver oxygen to your bloodstream. Your blood then transports it to your tissues. If your lungs don’t fully develop and have fewer alveoli than they should, your organs and tissues won’t have enough oxygen to function. Your heart has to work harder, possibly leading to high blood pressure in your lungs.

What are the symptoms of pulmonary hypoplasia?

Symptoms of pulmonary hypoplasia in newborns include:

• Bluish skin, lips or nails (cyanosis, a symptom of low oxygen in their tissues).
• Flaring nostrils.
• Trouble breathing.
• Fast breathing (tachypnea).

What causes pulmonary hypoplasia?

Pulmonary hypoplasia is almost always caused by other conditions that prevent a fetus’s lungs from developing as they should. These conditions often leave too little room for the lungs to grow. The most common conditions that cause pulmonary hypoplasia include:

• Low amniotic fluid (oligohydramnios).
• Congenital diaphragmatic hernia (CDH).
• Omphalocele.
• Prolonged premature rupture of membranes (PROM).
• Cystic lung disease. This is any condition that causes noncancerous growths on your lungs.
• Heart issues that cause an enlarged heart (like tricuspid atresia or Ebstein’s anomaly).
• Abnormal diaphragm activity or no diaphragm activity. Your diaphragm is a muscle that’s critical to your lungs working properly.

Sometimes, healthcare providers can’t find an underlying cause of pulmonary hypoplasia. This is called primary pulmonary hypoplasia. It accounts for about 10% to 15% of cases.

What are the risk factors for pulmonary hypoplasia?

Some conditions during pregnancy put the fetus at a higher risk for conditions that cause pulmonary hypoplasia, like low amniotic fluid and PROM. They include:

• Infections, like sexually transmitted infections (STIs) or bacterial vaginosis.
• Short cervical length.
• Expecting multiples (twins, triplets or more).
• Amniocentesis.
• High blood pressure or preeclampsia during pregnancy.
• Diabetes.
• Dehydration.

What are the complications of pulmonary hypoplasia?

Underdeveloped lungs can cause issues in other parts of your baby’s body. Complications of pulmonary hypoplasia include:

• Low blood oxygen levels (hypoxemia).
• High blood pressure in your baby’s lungs (pulmonary hypertension).
• Weak or underdeveloped windpipe (tracheomalacia).
• Collapsed lung (pneumothorax).
• Breathing disorders, like bronchopulmonary dysplasia. These can happen after being on a ventilator.
• Other complications of the underlying condition that caused pulmonary hypoplasia, like feeding issues, hearing loss and developmental delays.

How is pulmonary hypoplasia diagnosed?

A healthcare provider often diagnoses pulmonary hypoplasia with a routine ultrasound during pregnancy. They may do a follow-up MRI (magnetic resonance imaging) to confirm.

Sometimes, a provider diagnoses pulmonary hypoplasia at or shortly after birth. If your baby is having difficulty breathing, a provider will look for pulmonary hypoplasia and other lung conditions with a chest X-ray.

How is pulmonary hypoplasia treated?

Treatment for pulmonary hypoplasia depends on the cause, how severe it is and when it’s diagnosed (during pregnancy or after birth). If your baby is born with pulmonary hypoplasia, they’ll need help breathing until their lungs develop enough to do it on their own, or until providers can give them additional treatments.

Procedures for treating pulmonary hypoplasia

A pregnancy care provider or neonatal (newborn) specialist may use one or more of these treatments:

• Amnioinfusion (adding fluid to the amniotic sac).
• Surgery during pregnancy (fetal surgery) or after birth.
• Oxygen therapy.
• Mechanical ventilation.
• Extracorporeal membrane oxygenation (ECMO).

Can pulmonary hypoplasia be prevented?

There’s no one way to prevent pulmonary hypoplasia. But the best way to reduce your child’s risk for health complications at birth is to follow the guidance of your pregnancy care provider for a healthy pregnancy. You can take care of yourself by:

• Managing chronic health conditions.
• Attending all prenatal visits with your pregnancy care provider.
• Not smoking or drinking.
• Drinking plenty of water.
• Protecting yourself from STIs and other infectious diseases.

Attending regular appointments with your pregnancy care provider can help catch potential issues early. Providers can sometimes treat the underlying cause of pulmonary hypoplasia during pregnancy. This can help prevent your baby from being born with underdeveloped lungs.

What happens if your baby is born with pulmonary hypoplasia?

If your baby is born with underdeveloped lungs, a neonatologist and other specialists will evaluate them. Depending on the severity and other organs affected, their care team might include:

• Nephrologists.
• Pulmonologists.
• Cardiothoracic surgeons.
• Gastroenterologists.

Babies with pulmonary hypoplasia often receive treatment in the neonatal intensive care unit (NICU). They usually have lasting lung issues and other health conditions. Some babies with mild pulmonary hypoplasia may have symptom improvement as they grow.

What are the survival rates of pulmonary hypoplasia?

Babies with underdeveloped lungs can and do survive. But every case is different. Survival for pulmonary hypoplasia depends on:

• The severity.
• The underlying cause.
• When it’s diagnosed (during pregnancy or at birth).

The mortality (death rate) for pulmonary hypoplasia is over 55%. Severe hypoplasia isn’t usually survivable.

A note from Cleveland Clinic

If your baby is born with pulmonary hypoplasia, a team of healthcare providers will be with you every step of your journey. They’ll help you understand the severity, your treatment options and what the future could hold. Don’t hesitate to ask any questions you need to make informed decisions about your baby’s care — or your own. If you can, gather your own personal team of trusted loved ones. Their support can help you get through any challenges that lay ahead.