Isolated Unilateral Pulmonary Agenesis

Abstract

Pulmonary agenesis refers to the total absence of pulmonary parenchyma and blood vessels as well as bronchia beyond the bifurcation. It is classified as bilateral complete agenesis, unilateral agenesis, and lobar agenesis. The anomaly was first described in 1673 by De Pozze as a finding from the autopsy of a woman. Since then, several cases have been reported in the medical literature, yet both unilateral and bilateral pulmonary agenesis are rare congenital malformations. 1 - 3 Bilateral pulmonary agenesis is an exceedingly rare congenital malformation and is incompatible with extrauterine life, 2 whereas unilateral pulmonary agenesis does not result in the death of the infant and is compatible with long-term survival. Its prognosis depends largely on the presence of associated congenital cardiovascular, 3 , 4 gastrointestinal, 5 , 6 or genitourinary 7 malformations. Unilateral pulmonary agenesis has also been reported in association with ipsilateral skeletal anomalies 8 , 9 and nonimmune hydrops fetalis 5 as well as with certain chromosomal deletions (microdeletion 22q11). 1 0 , 1 1 This report documents a case of isolated unilateral pulmonary agenesis diagnosed prenatally in the first trimester of pregnancy by gray scale and color Doppler sonographywith good long-term outcome.