Symptoms Of Pulmonary Hypoplasia: Causes & Treatment Options

Pulmonary hypoplasia is a partial development of the lungs and which results in a poor number and / or size of the alveoli. This congenital malformation is rather rare and not associated with other maternal or fetal anomalies.

This is failure of development of the lungs in the uterus, which is most often unilateral. Consequently, the lungs do not have adequate tissue and blood flow for normal gas exchange.

Symptoms of pulmonary hypoplasia

Congenital anomalies of the lung are detected at the time of routine prenatal ultrasound scan. Common signs and symptoms of the condition, at birth include:

  • Difficulty in breathing with respiratory distress; there will be cyanosis, intercostal recession with tachypnoea, acidosis and hypoxia.
  • There may be features of other fetal disorders and anomalies such as skeletal dysplasia and cardiac malformations.

What Are The Causes Of Pulmonary Hypoplasia?

The following causes and etiological factors have been postulated and verified as causing pulmonary hypoplasia:

  • Abnormal thorax: A malformation of the thoracic cavity and a congenital diaphragmatic hernia are important factors.
  • Anomalous breathing movements: Neuromuscular disease in utero can result in decreased fetal breathing movement
  • Abnormal amniotic fluid volume: Is a contributing factor.
  • Defect in fetal lung fluid and lung fluid pressure: Is responsible for pulmonary hypoplasia.

Experts say that an overlap of aetiologies causes the condition. Conversely, pulmonary hypoplasia may be idiopathic (with no known cause) or associated with other congenital abnormalities – such as, multiple pterygium syndrome and trisomy 21.

Treatment Options For Pulmonary Hypoplasia

Early detection, preferably, in utero is very essential and starting the correct treatment regimen promptly plays a pivotal role in a case of pulmonary hypoplasia.

  • In case a newborn has immediate respiratory distress, you must suspect pulmonary hypoplasia.
  • The neonate needs to be resuscitated for several hours.
  • He will require intubation and ventilation.
  • Measurement of blood gases must be done via umbilical artery catheterisation.
  • Management of this condition has 3 aspects – intervention during pregnancy, timing and place of delivery, and treatment regimen after the child is born.
  • In some cases, fetal therapy is given for the underlying condition; this helps reduce the intensity of pulmonary hypoplasia. In a few cases, therapy includes fetal surgery.
  • The baby needs to have a planned delivery in a specialty hospital with a top-notch level of neonatal intensive care unit.
  • Premature delivery is usually rescheduled to rescue the fetus from an underlying condition causing the hypoplasia. The choice whether to deliver the baby prematurely includes a very watchful evaluation of the degree to whether delaying the delivery can increase or diminish the pulmonary hypoplasia.
  • After delivery, most of the afflicted babies need supplemental oxygen.
  • Discuss with your health care provider, pediatrician and OB/GYN and see what the best is for the baby and you.
  • Endoscopic tracheal occlusion in a fetus has been used in a few cases to manage congenital diaphragmatic hernia and pulmonary hypoplasia with a little success.

Prognosis Of Pulmonary Hypopalsia

The prognosis of pulmonary hypopalsia depends upon the extent of hypoplasia, the underlying etiological factor and other related anomalies.

Clinical case studies show that the survivors tend to have chronic lung problems, a decreased lung capacity, recurring respiratory infections and poor growth.

In case of severe pulmonary hypoplasia there is a decreased survival rate in babies.