Pulmonary Hypoplasia – Causes, Symptoms, and Treatment

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pulmonary hypoplasiaThis may be a difficult article for parents and grandparents alike as pulmonary hypoplasia is a serious condition affecting newborns. It is a potentially life-threatening condition in which the lungs fail to fully develop in the womb. When the lungs don’t function to capacity after birth, the tissue cannot grow properly; lung failure can be the result. We are going to take a closer look into this disorder, as a confirmed diagnosis often comes too late. Early pulmonary hypoplasia treatment is crucial. Knowing the causes, signs, and common pulmonary hypoplasia symptoms can prepare you and the medical team for treating the condition.

What Is Pulmonary Hypoplasia?

Pulmonary hypoplasia is present when the number of small air sacs in the lungs, known as alveoli, is abnormally low in a developing fetus. The alveoli are where the exchange of carbon dioxide and oxygen occurs. This condition can affect the newborn’s ability to breathe properly on its own following delivery. If new lung tissue does not generate with the pulmonary hypoplasia, the lungs may stop functioning completely.

What Causes Pulmonary Hypoplasia?

The pulmonary hypoplasia condition is sometimes referred to as secondary pulmonary hypoplasia because it is present with many existing health situations. We will look at the causes behind this condition.

1. Oligohydramnios

Known as insufficient amniotic fluid, this is a common cause of pulmonary hypoplasia. Oligohydramnios occurs when the amniotic fluid leaks from a ruptured premature membrane. With the loss of the fluid, the baby has trouble breathing and this breathing issue, as well as improper amniotic fluid amount and pressure, could result in the improper development of the lungs.

Oligohydramnios can also be caused by fetal renal abnormality. This type of amniotic fluid loss refers to the fluid produced by the kidneys of the fetus. The irregular growth of one or both kidneys can also alter the fluid.

2. Congenital Conditions

Incomplete development or problems with the structure of the fetus can influence the growth of the lungs. We will cover several conditions of congenital pulmonary hypoplasia.

Congenital Diaphragmatic Hernia

Congenital diaphragmatic hernia sees the abnormal development of the diaphragm. It can cause a hole in the organ that can be up to several inches in width. As the diaphragm separates the lung and the abdomen, this hole can allow the small intestine, the liver, or the spleen to relocate into the chest cavity. This may result in the lungs’ inability to develop properly.

Dwarfism

Dwarfism is another cause of an abnormal lung growth. In one form, known as thanatophoric dwarfism, the bones develop in various densities, sizes, and shapes. The chest may be narrower than the average infant, with underdeveloped lungs and shortened rib bones.

Cystic Hygroma

Cystic hygroma is a cyst on a newborn’s neck that interferes with the breathing process. This sac is filled with fluid and can cause compression on the airways.

Cystic Adenomatosis Malformation

Cystic adenomatosis malformation is linked to pulmonary hypoplasia as it affects one of the five lobes of the lungs. The left lung has two lobes and the right lung has three lobes. A cyst can form on one lobe as a result of improper development of lung tissue.

Hydrops Fetalis

Hydrops fetalis is a life-threatening condition where fluid builds within the chest cavity, including the lungs. This fluid surrounds the lungs, heart, and abdomen and causes swelling.

3. Primary Pulmonary Hypoplasia

This is a rare condition presenting undeveloped lungs without any structure issues. Most times, the cause cannot be determined.

Symptoms of Pulmonary Hypoplasia

Pulmonary hypoplasia symptoms show immediately at birth as respiratory distress is prevalent. Depending on the underlying cause, you can expect to see:

  • Rapid breathing
  • Blueish fingernails and lips
  • Chest cavity, nose, and mouth areas may appear sunken
  • Abdomen with a boat shape
  • High levels of carbon dioxide in bloodstream
  • Eyes set wide apart
  • Flat nose
  • Lowered ears
  • Sunken chin

Treatment for Pulmonary Hypoplasia

Before treatment, we always discuss diagnosis. However, with pulmonary hypoplasia, a confirmed diagnosis can only be made with an autopsy. This is why it is so vital to watch for signs at birth, so that treatment can start immediately.

Once born, the baby will be intubated and receive low pressure assisted ventilation. If the baby has a congenital diaphragmatic hernia, before ventilation, the bowels and stomach need to be attended to and decompressed. The pH and blood gases will be checked. The baby will also have a chest X-ray, and medical staff will monitor their oxygen levels.

Further treatment may include nitric oxide administration. The newborn may require multiple resuscitation interventions during the first days. As a last resort, surgery may be necessary to repair a hernia.

A pulmonary hypoplasia condition can be a terrifying event for a parent to face on what should be one of the best days of their life. When a child is born, there may be immediate signs of this condition to alert the medical team, such as abnormal facial features and breathing difficulty. With the lungs unable to wholly develop in their function or structure, the newborn will face many challenges in the hours after birth. Underlying causes are usually linked to health conditions already in existence. Immediate treatment rescues the damaged respiratory system. Time is of the essence when it comes to the breath of a newborn.


 

Sources:
“Pulmonary Hypoplasia”, Patient; https://patient.info/doctor/pulmonary-hypoplasia, last accessed June 26, 2017.
Oakley, D., “Pulmonary Hypoplasia and Your Baby’s Lungs,” Empowher; http://www.empowher.com/lung-conditions/content/pulmonary-hypoplasia-and-your-babys-lungs?page=0,1, last accessed June 26, 2017.




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After raising a son on her own, Tina knew it was time to find herself again. She moved from a small New Brunswick village to Toronto to pursue her first love: writing. With her journalism diploma and past reporter experience, she set out to make her mark on the world. Along with more than 25 years of experience in the financial, health, and business fields, Tina brings a wealth of knowledge and a nose for research to the Doctors Health... Read Full Bio »