Congenital pulmonary venolobar syndrome with thoracic ectopic kidney

Fig. 1

Cardiac computed tomography angiography was performed in a 14-month-old girl with a history of recurrent pneumonia in whom chest radiography revealed a hypoplastic right lung with a homogenous opacity in the right cardiophrenic region. The constellation of cardiopulmonary defects suggested congenital pulmonary venolobar syndrome with a rare association of thoracic ectopic kidney. a 3-dimensional (D) reconstructed posterior view shows a hypoplastic right pulmonary artery (white arrow) with systemic arterial supply to the right lower lobe from a branch of the abdominal aorta (broken white arrow). The hypoplastic right lung is drained by an anomalous pulmonary (scimitar) vein (black arrow) into the inferior vena cava (broken black arrow). There is focal stenosis (arrowhead) at the site of drainage. The right kidney is ectopic (asterisk). A malformed right upper lobar bronchial stump and compensatory hyperinflation of the left lung were also noted (not shown). b 3-D reconstructed anterior view shows the right kidney (asterisk) herniating into the thoracic cavity with the right renal hilum directed inferomedially and the right renal artery originating from the abdominal aorta (arrow)