Also known as: pediatric pulmonary hypoplasia, aplasia, PH.
What is pulmonary hypoplasia?
Pulmonary hypoplasia is a rare birth defect where there is incomplete development of lung tissue/blood vessels in one or both lungs, not allowing the baby to breathe normally. It may be Primary, or Secondary to another problem like congenital diaphragmatic hernia, fluid around the lungs (pleural effusions) or problems associated with the fetus making urine (resulting in decreased amniotic fluid- oligohydramnios). It is often associated with heart, gut, genitourinary and bone malformations.
What causes pulmonary hypoplasia?
The cause of Primary Pulmonary hypoplasia where structurally normal lungs are undeveloped, is unknown.
For the lungs to develop normally, the chest size must be normal and the air sacs of the developing lung must be distended by amniotic flies inhaled by the fetus making breathing movements. Secondary pulmonary hypoplasia may result from oligohydramnios (not enough amniotic fluid for more than 2 weeks, early rupture of membranes, early delivery), congenital diaphragmatic hernia, a very narrow chest from dwarfism, congenital problems of the heart, cysts in the lungs and other problems.
What are the symptoms of pulmonary hypoplasia?
Symptoms vary depending on the degree of the hypoplasia and associated problems. Common symptoms include rapid and difficulty with breathing, blue lips and toenails, and a sunken chest. Infants may need help breathing, and machines (ventilators) may be used to assist them. Later they may develop pulmonary hypertension (which is high blood pressure in the lungs’ arteries), heart problems, have recurrent chest infections and difficulty growing. Hearing problems can also occur.
What are pulmonary hypoplasia care options?
Pre and postnatal treatments for pulmonary hypoplasia can vary widely based on the nature and severity of the disease and may be complex. Your perinatologist, neonatologist, pediatric surgeon, pulmonologist and cardiac surgeon will explain and help you decide which of a number of treatment options will be the best for your baby.
Reviewed by: Jack Wolfsdorf, MD, FAAP
This page was last updated on: September 09, 2020 11:22 AM