P18.11: Unilateral pulmonary agenesis: prenatal imaging and outcome in three cases

Abstract

Objectives: To review the imaging findings and outcome of unilateral pulmonary agenesis in the fetus. Methods: Retrospective review at a single academic center. Results: Case 1: Presented at 29 weeks with severe IUGR, occipital encephalocele, left renal agenesis, arthrogryposis, small stomach and left lung agenesis. The infant died at 30 hrs of age, no intervention occured. Case 2: Presented at 18 weeks with unilateral left lung agenesis, small right lung, right renal agenesis, interrupted aortic arch, facial dysmorphism. The infant lived about 30 mins after resuscitation attempts ceased. Case 3: Presented at 20 weeks with left pulmonary agenesis, right renal agenesis, club foot, caudal regression sequence and other vertebral segmentation anomalies as well as a single umbilical artery. The infant survived; she had emergency repair of aortic coarctation and is alive at age 3. All had normal karyotype. Conclusions: Pulmonary agenesis is described as an isolated finding in the pediatric literature however 50% of live born infants have features of the VACTERL association. Our series suggests that the prognosis is guarded when the diagnosis is made prenatally. All three had significant associated abnormalities, some of which were not appreciated on fetal US or MRI. Two infants died in the neonatal period and the third had a difficult time during repair of an unanticipated coarctation of the aorta.