Primary Congenital Pulmonary Hypoplasia

Primary congenital pulmonary hypoplasia is a rare but lethal disease found in newborns causing respiratory distress. Hypoplasia is said to be primary when it occurs in the absence of any fetal or maternal disorders affecting lung development. Here is a case report of a term male newborn presenting with respiratory distress requiring ventilatory support followed by prolonged oxygen dependency. X-rays were showing homogenous opacities in right mid and lower zone for which CT was done and it came out to be pulmonary hypoplasia. It can be diagnosed antenatally using ultrasonography and MRI.