CTICasesPulmonary Hypoplasia in Prune Belly Syndrome
Imaging findings
CT chest in an adult patient with Prune Belly syndrome shows severe lung hypoplasia. The upper lobes appear 'empty' with a marked paucity of pulmonary vessels, regional lucency, and thin, scrawny, disorganized pulmonary arterial branches.
Key takeaways
Prune Belly syndrome (Eagle-Barrett syndrome) is characterized by abdominal wall muscle deficiency, urinary tract anomalies, and cryptorchidism. Pulmonary hypoplasia is a known association, likely resulting from fetal oligohydramnios and thoracic compression. Survivorship into adulthood is rare, and imaging shows a characteristic scrawny, deficient pulmonary vasculature.
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