Congenital Acinar Dysplasia: Report of a Case and Review of Literature

Congenital acinar dysplasia (CAD) is a very rare form of primary interstitial lung disease characterized by diffuse bilateral impairment of pulmonary acini (the respiratory bronchioles, alveolar ducts, and alveoli) development.1 His-tologically the appearance of lung at term resembles the 16-week pseudoglandular phase with no alveolar spaces for gas exchange. Although there is probably a genetic compo-nent, the exact etiology is unknown. The prognosis of known cases is fatal with most children dying within days of birth from respiratory insufficiency. Along with congenital alveolar dysplasia and alveolar capillary dysplasia with misalignment of pulmonary veins, CAD belongs to a group of poorly under-stood diffuse developmental disorders of the lung unique to infants and together account for a very small percent of diffuse lung disease in children.