Congenital alveolar dysplasia

Abstract

Congenital alveolar dysplasia is an uncommon congenital condition characterized by decrease of the alveolar units and abnormalities of the development of the pulmonary vasculature. This condition causes severe pulmonary hypertension and persistent hypoxemia with fatal course in most of the cases. It generally affects full-term newborns, with normal Apgar test, who subsequently develop breathing problems, usually in the first day of life.
The disease is refractory to conventional pulmonary support in most of the cases. We are publishing the case of a full-term newborn, without perinatal complications, who at a few hours of life presented with severe breathing difficulties that required prolonged mechanical ventilation, with subsequent multiorgan affectation, dying at 54 days of life.
The case was diagnosed through autopsy. In accordance with the case collected, the need to perform early pulmonary biopsy for patients having high clinical suspicion of this condition is proposed in order to offer adequate management and family support, given the poor course of the disease.