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Congenital Diaphragmatic Hernia with Lung Hypoplasia

Imaging findings

CT chest in a 4-month-old shows a large left-sided congenital diaphragmatic hernia (CDH) containing abdominal contents. The left lung is severely hypoplastic, showing arrested alveolar development, regional fibrosis, and air trapping. A quantitative VQ scan showed that the right lung received 90% of the total pulmonary blood flow.

Key takeaways

Congenital diaphragmatic hernia (CDH) restricts lung development in utero, leading to severe unilateral pulmonary hypoplasia. Postnatal mechanical ventilation and high oxygen exposure in the ICU can cause secondary BPD-like fibrocystic changes and severe pulmonary vascular remodeling, resulting in persistent pulmonary hypertension.

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