A health condition where a fetus or baby’s lungs develop improperly is called pulmonary hypoplasia. While it can be present at birth (congenital), doctors may detect this condition on an ultrasound during pregnancy. In most cases, pulmonary hypoplasia occurs due to an underlying condition (such as congenital diaphragmatic hernia or low levels of amniotic fluid).
This is a serious condition that may cause problems to the rest of the body. Normally, the alveoli (small air sacs in the lungs) deliver oxygen into the bloodstream. Therefore, oxygen reaches the tissues and organs in the body through the blood that transports it. When the lungs are not developed properly, there is a reduced alveoli count in the lungs, which makes it harder to supply the body’s needs. Furthermore, when there is not enough oxygen in the blood, it puts extra strain on the heart muscle that begins to work harder to pump more blood. As a result, it may lead to pulmonary hypertension (high blood pressure in the lungs).
Symptoms
Usually, people with pulmonary hypoplasia experience the following symptoms. Examples include:
- Flaring nostrils
- Difficulty breathing
- Tachypnea (fast breathing)
- Cyanosis (bluish lips, nails, or skin), which is a symptom of reduced oxygen levels in the blood
You should contact your healthcare professional right away if any of the symptoms listed above occur.
Causes
Mostly, pulmonary hypoplasia occurs due to an underlying condition that affects the fetus’s lung development. Usually, these disorders leave too little space for the lungs to grow. Check below for the most common diseases that cause pulmonary hypoplasia:
- Oligohydramnios (low amniotic fluid)
- Omphalocele
- PROM (prolonged premature rupture of membranes)
- CDH (congenital diaphragmatic hernia)
- Cystic lung disease – This medical term includes all conditions that cause benign (noncancerous) growths on the lungs
- Tricuspid atresia or Ebstein’s anomaly (these conditions may cause an enlarged heart muscle)
- Abnormal or a lack of diaphragm activity – The Diaphragm is a muscle that helps the lungs work correctly
However, doctors cannot always identify the underlying cause of this condition. In such cases, the condition is called primary pulmonary hypoplasia, and it accounts for approximately 10% to 15% of cases.
Risk Factors
There are some factors that may elevate your baby’s risk of pulmonary hypoplasia. Examples include:
- Short cervical length
- Infections, including bacterial vaginosis, sexually transmitted infections (STIs)
- Expecting multiple (including twins, triplets, or more)
- Amniocentesis
- Hypertension or preeclampsia during pregnancy
- Diabetes
- Dehydration
What Are The Potential Complications of Pulmonary Hypoplasia?
Babies with pulmonary hypoplasia may also experience some complications, especially if they do not get treatment. Check below some of them:
- Hypoxemia (reduced blood oxygen levels)
- Pulmonary hypertension (increased blood pressure in the lung arteries)
- Tracheomalacia (weak or underdeveloped windpipe)
- Pneumothorax (collapsed lung)
- Breathing condition – For example, bronchopulmonary dysplasia and others
- Hearing loss
- Developmental delays
- Feeding problems
- Acute or chronic (long-term) respiratory failure
- Reduced exercise tolerance
- Alveolar hemorrhage
- Frequent lung infections
- Heart problems
The previous list does not contain all possible pulmonary hypoplasia complications. In any case, you can consult your healthcare professional about measures to reduce the risk or even prevent the previous complications.
How to Prevent Pulmonary Hypoplasia?
Unfortunately, there is no sure way to prevent this condition, but you can take some steps to reduce the risk. Check below some tips that doctors often recommend:
- Stop smoking or drinking alcoholic drinks (especially during pregnancy) – If you have problems with smoking cessation, discuss it with your doctor.
- Drink plenty of water to prevent dehydration
- Manage chronic health conditions, including hypertension (high blood pressure), diabetes, high cholesterol, and others
- Get protection against STIs and other infectious diseases
- Regularly visit your doctor (especially during pregnancy)
Regular visits to your doctor during pregnancy may help identify this condition before delivery. Sometimes, it is possible to prevent the baby from being born with underdeveloped lungs.
Diagnosis
Usually, this condition is diagnosed with a routine ultrasound during pregnancy. However, to confirm the condition, doctors often perform an MRI (magnetic resonance imaging). In some cases, doctors diagnose pulmonary hypoplasia at birth or shortly after birth. Moreover, if your baby has breathing problems, doctors may perform a chest X-ray to check for other lung disorders.
Treatment
Commonly, doctors prescribe different treatments for people with pulmonary hypoplasia because they depend on the exact cause, the severity of the condition, when it is diagnosed (during pregnancy or after birth), and other factors. In most cases, babies with pulmonary hypoplasia need help breathing until their lungs develop and can do it on their own. Check below for other options often recommended by doctors:
- Surgery during pregnancy (fetal surgery) or after birth
- Mechanical ventilation
- Oxygen therapy
- ECMO (extracorporeal membrane oxygenation)
- Amnioinfusion (during this procedure, doctors will add fluid to the amniotic sac)
Frequently Asked Questions
What happens if your child is born with pulmonary hypoplasia?
This condition causes the lungs not to develop properly during pregnancy. Thus, your child can be referred to a neonatologist and other healthcare professionals to evaluate him/her. The healthcare team may include:
- Cardiothoracic surgeons
- Gastroenterologists
- Pulmonologists
- Nephrologists
Usually, babies with this condition receive treatment in the neonatal intensive care unit (NICU).
What are the survival rates of pulmonary hypoplasia?
In general, children with pulmonary hypoplasia can and do survive. However, the survival rates are different because they depend on some factors. These include the severity and underlying cause of the condition, when it is diagnosed, and others. The death rate (mortality) for this condition is over 55%, and in severe cases, it is not survivable.
Can hypoplasia be cured?
Unfortunately, there is no way to cure this condition. That’s why the treatment goal is to reduce the symptoms and improve your baby’s quality of life. Ask your healthcare provider if you have additional questions.
