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Congenital Pulmonary Venolobar Syndrome: Spectrum of Helical CT Findings with Emphasis on Computerized Reformatting¹

¹ From the Department of Diagnostic Imaging (E.K., L.R.Z., M.K., G.G., J.R.) and the Pediatric Cardiology Unit (J.H.), Chaim Sheba Medical Center, Tel-Aviv University, Tel Hashomer 52662, Israel; the Department of Diagnostic Imaging, Children’s Hospital, Oakland, Calif (R.A.C.); the Department of Diagnostic Imaging, Rambam Medical Center, Technion-Israel Institute of Technology, Haifa, Israel (M.E., A.O.); the Department of Diagnostic Imaging, Hadassah University Hospital, Jerusalem, Israel (I.B.M., J.B.Z.); and the Department of Diagnostic Imaging, Sapir Medical Center, Tel-Aviv University, Kfar Sava, Israel (O.K.). Recipient of a Cum Laude award for an education exhibit at the 2002 RSNA scientific assembly. Received January 8, 2003; revision requested March 6 and received April 17; accepted April 21. Address correspondence to E.K. (e-mail: konen_e@yahoo.ca).

View larger version (115K): [in a new window]   Figure 1a.  Pulmonary sequestration in a 6-year-old boy with recurrent episodes of right lower lobe pneumonia. (a) Axial CT angiogram obtained at the level of the lung bases shows a consolidation with large vessels centrally (arrows) in the right lower lobe. (b) Axial CT angiogram obtained inferior to a shows that the vessels originate from a large artery (arrow) that crosses inferior to the diaphragm. (c, d) SSD (c) and magnified volume-rendered (d) reformatted images clearly demonstrate a systemic aberrant artery (arrow) that originates from the celiac trunk (arrowhead in d) and supplies the right lower lung. In c, the trachea and lungs are highlighted in blue and the ribs and lungs are partially transparent. AO = aorta. Surgery revealed an intralobar sequestration.

View larger version (120K): [in a new window]   Figure 1b.  Pulmonary sequestration in a 6-year-old boy with recurrent episodes of right lower lobe pneumonia. (a) Axial CT angiogram obtained at the level of the lung bases shows a consolidation with large vessels centrally (arrows) in the right lower lobe. (b) Axial CT angiogram obtained inferior to a shows that the vessels originate from a large artery (arrow) that crosses inferior to the diaphragm. (c, d) SSD (c) and magnified volume-rendered (d) reformatted images clearly demonstrate a systemic aberrant artery (arrow) that originates from the celiac trunk (arrowhead in d) and supplies the right lower lung. In c, the trachea and lungs are highlighted in blue and the ribs and lungs are partially transparent. AO = aorta. Surgery revealed an intralobar sequestration.

View larger version (133K): [in a new window]   Figure 1c.  Pulmonary sequestration in a 6-year-old boy with recurrent episodes of right lower lobe pneumonia. (a) Axial CT angiogram obtained at the level of the lung bases shows a consolidation with large vessels centrally (arrows) in the right lower lobe. (b) Axial CT angiogram obtained inferior to a shows that the vessels originate from a large artery (arrow) that crosses inferior to the diaphragm. (c, d) SSD (c) and magnified volume-rendered (d) reformatted images clearly demonstrate a systemic aberrant artery (arrow) that originates from the celiac trunk (arrowhead in d) and supplies the right lower lung. In c, the trachea and lungs are highlighted in blue and the ribs and lungs are partially transparent. AO = aorta. Surgery revealed an intralobar sequestration.

View larger version (125K): [in a new window]   Figure 1d.  Pulmonary sequestration in a 6-year-old boy with recurrent episodes of right lower lobe pneumonia. (a) Axial CT angiogram obtained at the level of the lung bases shows a consolidation with large vessels centrally (arrows) in the right lower lobe. (b) Axial CT angiogram obtained inferior to a shows that the vessels originate from a large artery (arrow) that crosses inferior to the diaphragm. (c, d) SSD (c) and magnified volume-rendered (d) reformatted images clearly demonstrate a systemic aberrant artery (arrow) that originates from the celiac trunk (arrowhead in d) and supplies the right lower lung. In c, the trachea and lungs are highlighted in blue and the ribs and lungs are partially transparent. AO = aorta. Surgery revealed an intralobar sequestration.

View larger version (113K): [in a new window]   Figure 2a.  Pulmonary sequestration with an associated suspected gastric duplication in an asymptomatic male neonate. (a) Coronal multiplanar reformatted image shows an aberrant artery (arrowhead) that arises from the descending thoracic aorta and supplies a mass in the left lung base. An adjacent infradiaphragmatic cystic mass is also noted (C). (b) On an axial image obtained at the level of the gastric fundus, the cystic mass (C) is seen to project into the stomach, a finding that is compatible with gastric duplication.

View larger version (130K): [in a new window]   Figure 2b.  Pulmonary sequestration with an associated suspected gastric duplication in an asymptomatic male neonate. (a) Coronal multiplanar reformatted image shows an aberrant artery (arrowhead) that arises from the descending thoracic aorta and supplies a mass in the left lung base. An adjacent infradiaphragmatic cystic mass is also noted (C). (b) On an axial image obtained at the level of the gastric fundus, the cystic mass (C) is seen to project into the stomach, a finding that is compatible with gastric duplication.

View larger version (154K): [in a new window]   Figure 3a.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (136K): [in a new window]   Figure 3b.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (135K): [in a new window]   Figure 3c.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (129K): [in a new window]   Figure 3d.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (131K): [in a new window]   Figure 3e.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (123K): [in a new window]   Figure 3f.  Bilateral pulmonary sequestration that caused congestive heart failure in a male neonate. The patient’s condition was diagnosed prenatally at US. (a) Combined SSD and multiplanar reformatted image shows an anomalous artery that arises from the descending thoracic aorta and bifurcates to supply two enhancing soft-tissue masses in the lung bases. (b, c) Curvilinear multiplanar reformatted images clearly delineate the anomalous arteries in the right (b) and left (c) lung bases. (d) Findings on an aortogram obtained before successful embolization of the aberrant arteries are virtually identical to and thereby help confirm the CT angiographic findings (cf a). (e) Axial minimum-intensity-projection reformatted image reveals an accessory aerated aberrant bronchial tree (arrow) that connects the two masses. The aberrant bronchial tree is separated from the normal tracheobronchial tree, a finding that suggests a rare connection to the esophagus or stomach. AO = aorta. (f) On a follow-up CT angiogram obtained 1 year after embolization, the volume of the left sequestration is significantly reduced and the right sequestration has almost totally disappeared. Note the steel coils (arrows) used for embolization of the right aberrant artery.

View larger version (129K): [in a new window]   Figure 4a.  Hypogenetic lung syndrome in a 22-year-old asymptomatic woman. (a) Axial CT angiogram obtained at the level of the lung bases shows a large anomalous vein (*) that drains into a dilated inferior vena cava (IVC). (b) Volume-rendered reformatted image demonstrates that the anomalous vein (*) drains into the IVC below the level of the right hemidiaphragm. L = liver. (c) Anteroposterior venous phase pulmonary angiogram obtained for shunt quantification helps confirm the presence of an anomalous pulmonary vein. (d) On a minimum-intensity-projection reformatted image, the right upper lobe bronchus is not seen, allowing visualization of the associated hypogenetic lung and central bronchi.

View larger version (145K): [in a new window]   Figure 4b.  Hypogenetic lung syndrome in a 22-year-old asymptomatic woman. (a) Axial CT angiogram obtained at the level of the lung bases shows a large anomalous vein (*) that drains into a dilated inferior vena cava (IVC). (b) Volume-rendered reformatted image demonstrates that the anomalous vein (*) drains into the IVC below the level of the right hemidiaphragm. L = liver. (c) Anteroposterior venous phase pulmonary angiogram obtained for shunt quantification helps confirm the presence of an anomalous pulmonary vein. (d) On a minimum-intensity-projection reformatted image, the right upper lobe bronchus is not seen, allowing visualization of the associated hypogenetic lung and central bronchi.

View larger version (148K): [in a new window]   Figure 4c.  Hypogenetic lung syndrome in a 22-year-old asymptomatic woman. (a) Axial CT angiogram obtained at the level of the lung bases shows a large anomalous vein (*) that drains into a dilated inferior vena cava (IVC). (b) Volume-rendered reformatted image demonstrates that the anomalous vein (*) drains into the IVC below the level of the right hemidiaphragm. L = liver. (c) Anteroposterior venous phase pulmonary angiogram obtained for shunt quantification helps confirm the presence of an anomalous pulmonary vein. (d) On a minimum-intensity-projection reformatted image, the right upper lobe bronchus is not seen, allowing visualization of the associated hypogenetic lung and central bronchi.

View larger version (124K): [in a new window]   Figure 4d.  Hypogenetic lung syndrome in a 22-year-old asymptomatic woman. (a) Axial CT angiogram obtained at the level of the lung bases shows a large anomalous vein (*) that drains into a dilated inferior vena cava (IVC). (b) Volume-rendered reformatted image demonstrates that the anomalous vein (*) drains into the IVC below the level of the right hemidiaphragm. L = liver. (c) Anteroposterior venous phase pulmonary angiogram obtained for shunt quantification helps confirm the presence of an anomalous pulmonary vein. (d) On a minimum-intensity-projection reformatted image, the right upper lobe bronchus is not seen, allowing visualization of the associated hypogenetic lung and central bronchi.

View larger version (137K): [in a new window]   Figure 5a.  Horseshoe lung in a male neonate. (a) Axial CT scan obtained at the level of the lung bases shows an isthmus of pulmonary parenchyma (arrow) that extends behind the heart and joins the posterobasal segments of both lungs. An anomalous right pulmonary vein (*) drains into the IVC. (b) Volume-rendered reformatted image of the airways and lung parenchyma reveals partial fusion of the lungs posterior to the heart and an abnormal bronchus that arises from the left main bronchus and crosses the thoracic midline. (c) Coronal multiplanar reformatted image shows an anomalous right pulmonary vein (*) that drains into the IVC below the level of the right hemidiaphragm. (d) SSD reformatted image of the pulmonary artery demonstrates an inferior branch (*) that arises from the right pulmonary artery (•) and crosses the midline to the base of the left lung. (e) Anteroposterior arterial phase pulmonary angiogram helps confirm the CT angiographic findings. * = anomalous branch, • = right pulmonary artery.

View larger version (94K): [in a new window]   Figure 5b.  Horseshoe lung in a male neonate. (a) Axial CT scan obtained at the level of the lung bases shows an isthmus of pulmonary parenchyma (arrow) that extends behind the heart and joins the posterobasal segments of both lungs. An anomalous right pulmonary vein (*) drains into the IVC. (b) Volume-rendered reformatted image of the airways and lung parenchyma reveals partial fusion of the lungs posterior to the heart and an abnormal bronchus that arises from the left main bronchus and crosses the thoracic midline. (c) Coronal multiplanar reformatted image shows an anomalous right pulmonary vein (*) that drains into the IVC below the level of the right hemidiaphragm. (d) SSD reformatted image of the pulmonary artery demonstrates an inferior branch (*) that arises from the right pulmonary artery (•) and crosses the midline to the base of the left lung. (e) Anteroposterior arterial phase pulmonary angiogram helps confirm the CT angiographic findings. * = anomalous branch, • = right pulmonary artery.

View larger version (99K): [in a new window]   Figure 5c.  Horseshoe lung in a male neonate. (a) Axial CT scan obtained at the level of the lung bases shows an isthmus of pulmonary parenchyma (arrow) that extends behind the heart and joins the posterobasal segments of both lungs. An anomalous right pulmonary vein (*) drains into the IVC. (b) Volume-rendered reformatted image of the airways and lung parenchyma reveals partial fusion of the lungs posterior to the heart and an abnormal bronchus that arises from the left main bronchus and crosses the thoracic midline. (c) Coronal multiplanar reformatted image shows an anomalous right pulmonary vein (*) that drains into the IVC below the level of the right hemidiaphragm. (d) SSD reformatted image of the pulmonary artery demonstrates an inferior branch (*) that arises from the right pulmonary artery (•) and crosses the midline to the base of the left lung. (e) Anteroposterior arterial phase pulmonary angiogram helps confirm the CT angiographic findings. * = anomalous branch, • = right pulmonary artery.

View larger version (119K): [in a new window]   Figure 5d.  Horseshoe lung in a male neonate. (a) Axial CT scan obtained at the level of the lung bases shows an isthmus of pulmonary parenchyma (arrow) that extends behind the heart and joins the posterobasal segments of both lungs. An anomalous right pulmonary vein (*) drains into the IVC. (b) Volume-rendered reformatted image of the airways and lung parenchyma reveals partial fusion of the lungs posterior to the heart and an abnormal bronchus that arises from the left main bronchus and crosses the thoracic midline. (c) Coronal multiplanar reformatted image shows an anomalous right pulmonary vein (*) that drains into the IVC below the level of the right hemidiaphragm. (d) SSD reformatted image of the pulmonary artery demonstrates an inferior branch (*) that arises from the right pulmonary artery (•) and crosses the midline to the base of the left lung. (e) Anteroposterior arterial phase pulmonary angiogram helps confirm the CT angiographic findings. * = anomalous branch, • = right pulmonary artery.

View larger version (92K): [in a new window]   Figure 5e.  Horseshoe lung in a male neonate. (a) Axial CT scan obtained at the level of the lung bases shows an isthmus of pulmonary parenchyma (arrow) that extends behind the heart and joins the posterobasal segments of both lungs. An anomalous right pulmonary vein (*) drains into the IVC. (b) Volume-rendered reformatted image of the airways and lung parenchyma reveals partial fusion of the lungs posterior to the heart and an abnormal bronchus that arises from the left main bronchus and crosses the thoracic midline. (c) Coronal multiplanar reformatted image shows an anomalous right pulmonary vein (*) that drains into the IVC below the level of the right hemidiaphragm. (d) SSD reformatted image of the pulmonary artery demonstrates an inferior branch (*) that arises from the right pulmonary artery (•) and crosses the midline to the base of the left lung. (e) Anteroposterior arterial phase pulmonary angiogram helps confirm the CT angiographic findings. * = anomalous branch, • = right pulmonary artery.

View larger version (140K): [in a new window]   Figure 6a.  Interruption of the left pulmonary artery in a 36-year-old man who was born with a persistent truncus arteriosus (surgically repaired in childhood). (a) Axial CT angiogram obtained at the level of the carina shows interruption of the left main pulmonary artery. The left lung is mildly hypoplastic with decreased vascularity. Note the calcified conduit (*) that connects the right ventricle to the main pulmonary artery and an aneurysmal ascending aorta (AO). (b) Coronal multiplanar reformatted image shows peripheral linear areas of increased attenuation in the left lung (arrows). This finding, which was confirmed at angiography, represents collateral vessels that invade the lung through the pleura. Inset demonstrates the plane of reformation.

View larger version (155K): [in a new window]   Figure 6b.  Interruption of the left pulmonary artery in a 36-year-old man who was born with a persistent truncus arteriosus (surgically repaired in childhood). (a) Axial CT angiogram obtained at the level of the carina shows interruption of the left main pulmonary artery. The left lung is mildly hypoplastic with decreased vascularity. Note the calcified conduit (*) that connects the right ventricle to the main pulmonary artery and an aneurysmal ascending aorta (AO). (b) Coronal multiplanar reformatted image shows peripheral linear areas of increased attenuation in the left lung (arrows). This finding, which was confirmed at angiography, represents collateral vessels that invade the lung through the pleura. Inset demonstrates the plane of reformation.

View larger version (128K): [in a new window]   Figure 7a.  Interruption of the left main pulmonary artery in a 16-year-old boy. (a, b) Axial CT scans obtained at the level of the right main bronchus (a) and bronchus intermedius (b) (arrows) show interruption of the left main pulmonary artery. Note the compressed right main bronchus between the right-sided descending aorta (Ao) and a prominent right main pulmonary artery (RPA). The mediastinum is shifted to the left due to an associated hypoplastic left lung. (c) SSD reformatted image (superior view) delineates the spatial relationship between the prominent single pulmonary artery (blue), the right-sided descending aorta (AO [red]), and the compressed right main bronchus. (d) Virtual bronchoscopic reformatted image obtained at the level of the carina ("supine" position) shows the right main bronchus (R) with a compressed "fishmouth" shape. L = left main bronchus.

View larger version (123K): [in a new window]   Figure 7b.  Interruption of the left main pulmonary artery in a 16-year-old boy. (a, b) Axial CT scans obtained at the level of the right main bronchus (a) and bronchus intermedius (b) (arrows) show interruption of the left main pulmonary artery. Note the compressed right main bronchus between the right-sided descending aorta (Ao) and a prominent right main pulmonary artery (RPA). The mediastinum is shifted to the left due to an associated hypoplastic left lung. (c) SSD reformatted image (superior view) delineates the spatial relationship between the prominent single pulmonary artery (blue), the right-sided descending aorta (AO [red]), and the compressed right main bronchus. (d) Virtual bronchoscopic reformatted image obtained at the level of the carina ("supine" position) shows the right main bronchus (R) with a compressed "fishmouth" shape. L = left main bronchus.

View larger version (116K): [in a new window]   Figure 7c.  Interruption of the left main pulmonary artery in a 16-year-old boy. (a, b) Axial CT scans obtained at the level of the right main bronchus (a) and bronchus intermedius (b) (arrows) show interruption of the left main pulmonary artery. Note the compressed right main bronchus between the right-sided descending aorta (Ao) and a prominent right main pulmonary artery (RPA). The mediastinum is shifted to the left due to an associated hypoplastic left lung. (c) SSD reformatted image (superior view) delineates the spatial relationship between the prominent single pulmonary artery (blue), the right-sided descending aorta (AO [red]), and the compressed right main bronchus. (d) Virtual bronchoscopic reformatted image obtained at the level of the carina ("supine" position) shows the right main bronchus (R) with a compressed "fishmouth" shape. L = left main bronchus.

View larger version (119K): [in a new window]   Figure 7d.  Interruption of the left main pulmonary artery in a 16-year-old boy. (a, b) Axial CT scans obtained at the level of the right main bronchus (a) and bronchus intermedius (b) (arrows) show interruption of the left main pulmonary artery. Note the compressed right main bronchus between the right-sided descending aorta (Ao) and a prominent right main pulmonary artery (RPA). The mediastinum is shifted to the left due to an associated hypoplastic left lung. (c) SSD reformatted image (superior view) delineates the spatial relationship between the prominent single pulmonary artery (blue), the right-sided descending aorta (AO [red]), and the compressed right main bronchus. (d) Virtual bronchoscopic reformatted image obtained at the level of the carina ("supine" position) shows the right main bronchus (R) with a compressed "fishmouth" shape. L = left main bronchus.