CTICasesCongenital Hypoplasia of the Right Upper Lobe
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Imaging findings
Chest radiography and CT demonstrate significant right lung volume loss and a small, hypoplastic right upper lobe with developmental bronchovascular crowding. To compensate for the hypoplastic lobe, the right middle lobe is hyperinflated and expanded posteriorly, and fat is sucked in from the chest wall into a deep posterior fissure. The patient also has scoliosis and an atrial septal defect closure device.
Key takeaways
Congenital lobar hypoplasia is a developmental abnormality characterized by an abnormally small lobe with normal bronchovascular relationships. This hypoplasia results in compensatory hyperexpansion of adjacent lobes and chest wall retraction, which must be distinguished from Congenital Lobar Emphysema. The co-occurrence of skeletal, cardiac, and pulmonary developmental anomalies should raise suspicion for a syndromic association such as VACTERL.
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