Prenatal diagnosis of unilateral pulmonary dysplasia

Abstract

Pulmonary hypoplasia is a rare congenital disorder; most cases occur in association with other congenital abnormalities, including congenital diaphragmatic hernia, oligohydramnios, and/or skeletal deformities. The authors report a case of unilateral pulmonary hypoplasia diagnosed prenatally and confirmed at autopsy.

Keywords: Fetal lung development; Prenatal diagnosis; Pulmonary hypoplasia; Pulmonary vasculature.

Figure 1

Prenatal diagnosis and confirmation of pulmonary hypoplasia. At prenatal ultrasound, axial imaging of the fetal thorax revealed normal lung tissue on the right-hand side, but no lung tissue on the left-hand side (A, thick arrow). Mediastinal shift is evident with the heart pushed over to the left and contiguous with the ribs. A ventricular septal defect (VSD) was identified. A hyperechogenic mass measuring 1.7 × 1.7 cm was revealed behind the heart, which likely represents the rudimentary left lung (B). At autopsy, no lung tissue was evident in the left hemithorax (C, thick arrow). A small portion of lobulated tissue was identified behind the right lung, which was subsequently identified as the rudimentary left lung (L-LU) (D). The diaphragm (DI) was intact. After lifting the ascending aorta (AAO) aside, the right pulmonary artery (RPA) was identified (E). A right-sided lateral view showed the rudimentary left lung (ML-LU) supplied by the left pulmonary artery (LPA) and connected to esophagus (E) via an aberrant left bronchus (LB) (F).