Pulmonary agenesis with VACTERL association: A rare case with review of literature

Abstract

Pulmonary agenesis is an extremely rare congenital malformation with an estimated frequency of 1 in 10000-15000 autopsies and 34 per million live births. In 50% cases it is associated with other malformations i.e. cardiovascular, musculoskeletal, gastrointestinal and renal. We report a rare case of a male neonate with right pulmonary agenesis associated with VACTERL sequence (L5 hemi-vertebra, right absent kidney with left ectopic kidney and right radial agenesis). This association was never reported in published literature.