Congenital Pulmonary Venolobar Syndrome

Eli Konen, MD ; Lisa Raviv-Zilka, MD ; Ronald A. Cohen, MD ; Monica Epelman, MD ; Inbal Boger-Megiddo, MD ; Jacob Bar-Ziv, MD ; Julius Hegesh, MD ; Amos Ofer, MD ; Osnat Konen, MD ; Miriam Katz, MD ; Gabi Gayer, MD ; Judith Rozenman, MD

The term congenital pulmonary venolobar syndrome refers to a wide spectrum of pulmonary developmental anomalies that may appear singly or in combination. The main components of congenital pulmonary venolobar syndrome are hypogenetic lung (including lobar agenesis, aplasia, or hypoplasia), partial anomalous pulmonary venous return, absence of pulmonary artery, pulmonary sequestration, systemic arterialization of lung, absence of inferior vena cava, and accessory diaphragm. The recent introduction of multisection helical computed tomography (CT), combined with use of advanced postprocessing graphic workstations, allows improved noninvasive delineation of complex congenital anomalies. A single fast (5–15-second) CT scan now enables the radiologist to (a) generate angiogram-like images of the anomalous pulmonary arteries and veins; (b) demonstrate tracheobronchial abnormalities by generating simulated bronchographic or bronchoscopic images; and (c) depict associated parenchymal abnormalities on axial, coronal, or sagittal images, which once represented an important advantage of magnetic resonance imaging over CT. Multisection helical CT is a helpful diagnostic tool in the preoperative evaluation of patients with suspected congenital pulmonary venolobar syndrome.