Unilateral pulmonary agenesis associated with multiple malformations-a case report

Abstract

We describe a child with right pulmonary agenesis associated with multiple bilateral malformations. Pulmonary agenesis is a rare congenital anomaly that should be considered in the differential diagnosis of newborn RDS (respiratory distress syndrome), particularly in patients with multiple malformations. Symptom severity is variable and the prognosis is to a great extent dependent on the associated malformations. Diagnosis should be confirmed by CT or MR imaging. Management is usually conservative, although the use of tissue expanders to prevent mediastinal shift should be considered in patients with cardiovascular instability.