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Developmental Lung Anomalies in the Adult: Radiologic-Pathologic Correlation¹

¹ From the Departments of Diagnostic Radiology (C.J.Z., W.R.E., D.L.S.) and Anatomic Pathology (C.H.S.), Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202-2689. Presented as an education exhibit at the 2000 RSNA scientific assembly. Received January 21, 2002; revision requested February 20 and received April 15; accepted April 26. Address correspondence to C.J.Z. (e-mail: zylak@rad.hfh.edu).

View larger version (144K): [in a new window]   Figure 1a.  Agenesis of the right upper lobe. (a, b) Posteroanterior (a) and lateral (b) radiographs show a reduction in the volume of the right lung with obscuration of the right border of the heart. An anterior area of increased opacity on the lateral view (b) simulates collapse of the right upper and middle lobes. (c) Computed tomographic (CT) scan shows absence of the right upper lobe bronchus and shift of the heart to the right. This shift simulates collapse of the right upper and middle lobes and accounts for the anterior area of increased opacity on the lateral radiograph (b). Ventilation and perfusion are otherwise normal.

View larger version (128K): [in a new window]   Figure 1b.  Agenesis of the right upper lobe. (a, b) Posteroanterior (a) and lateral (b) radiographs show a reduction in the volume of the right lung with obscuration of the right border of the heart. An anterior area of increased opacity on the lateral view (b) simulates collapse of the right upper and middle lobes. (c) Computed tomographic (CT) scan shows absence of the right upper lobe bronchus and shift of the heart to the right. This shift simulates collapse of the right upper and middle lobes and accounts for the anterior area of increased opacity on the lateral radiograph (b). Ventilation and perfusion are otherwise normal.

View larger version (116K): [in a new window]   Figure 1c.  Agenesis of the right upper lobe. (a, b) Posteroanterior (a) and lateral (b) radiographs show a reduction in the volume of the right lung with obscuration of the right border of the heart. An anterior area of increased opacity on the lateral view (b) simulates collapse of the right upper and middle lobes. (c) Computed tomographic (CT) scan shows absence of the right upper lobe bronchus and shift of the heart to the right. This shift simulates collapse of the right upper and middle lobes and accounts for the anterior area of increased opacity on the lateral radiograph (b). Ventilation and perfusion are otherwise normal.

View larger version (106K): [in a new window]   Figure 2a.  Bronchial atresia in a 38-year-old man who experienced repeated pulmonary infections. (a, b) Frontal radiograph (a) and CT scan (b) show an appearance suggestive of congenital bronchial atresia. These images accompanied a request for biopsy of a mass in the left upper lobe. The mass contained air and fluid. There was distortion of the lung peripheral to the mass. (c) Follow-up posteroanterior radiograph obtained 1 month later shows a decrease in the fluid within the mass. Peripheral linear areas of increased opacity are also present, which likely represent scarring. (d) CT scan obtained at the same time as c shows distortion of the left upper lobe architecture with linear scarring. The dilated bronchi are air filled or contain air and fluid. A supplying bronchus could not be identified. The patient underwent a left upper lobectomy. (e) Photograph of the specimen shows an opened bronchus that communicates with a 4 x 3 x 2-cm cavity, which contained mucus and firm debris. A second cavity 2 cm in diameter was also found. The lingula was not involved.

View larger version (114K): [in a new window]   Figure 2b.  Bronchial atresia in a 38-year-old man who experienced repeated pulmonary infections. (a, b) Frontal radiograph (a) and CT scan (b) show an appearance suggestive of congenital bronchial atresia. These images accompanied a request for biopsy of a mass in the left upper lobe. The mass contained air and fluid. There was distortion of the lung peripheral to the mass. (c) Follow-up posteroanterior radiograph obtained 1 month later shows a decrease in the fluid within the mass. Peripheral linear areas of increased opacity are also present, which likely represent scarring. (d) CT scan obtained at the same time as c shows distortion of the left upper lobe architecture with linear scarring. The dilated bronchi are air filled or contain air and fluid. A supplying bronchus could not be identified. The patient underwent a left upper lobectomy. (e) Photograph of the specimen shows an opened bronchus that communicates with a 4 x 3 x 2-cm cavity, which contained mucus and firm debris. A second cavity 2 cm in diameter was also found. The lingula was not involved.

View larger version (126K): [in a new window]   Figure 2c.  Bronchial atresia in a 38-year-old man who experienced repeated pulmonary infections. (a, b) Frontal radiograph (a) and CT scan (b) show an appearance suggestive of congenital bronchial atresia. These images accompanied a request for biopsy of a mass in the left upper lobe. The mass contained air and fluid. There was distortion of the lung peripheral to the mass. (c) Follow-up posteroanterior radiograph obtained 1 month later shows a decrease in the fluid within the mass. Peripheral linear areas of increased opacity are also present, which likely represent scarring. (d) CT scan obtained at the same time as c shows distortion of the left upper lobe architecture with linear scarring. The dilated bronchi are air filled or contain air and fluid. A supplying bronchus could not be identified. The patient underwent a left upper lobectomy. (e) Photograph of the specimen shows an opened bronchus that communicates with a 4 x 3 x 2-cm cavity, which contained mucus and firm debris. A second cavity 2 cm in diameter was also found. The lingula was not involved.

View larger version (117K): [in a new window]   Figure 2d.  Bronchial atresia in a 38-year-old man who experienced repeated pulmonary infections. (a, b) Frontal radiograph (a) and CT scan (b) show an appearance suggestive of congenital bronchial atresia. These images accompanied a request for biopsy of a mass in the left upper lobe. The mass contained air and fluid. There was distortion of the lung peripheral to the mass. (c) Follow-up posteroanterior radiograph obtained 1 month later shows a decrease in the fluid within the mass. Peripheral linear areas of increased opacity are also present, which likely represent scarring. (d) CT scan obtained at the same time as c shows distortion of the left upper lobe architecture with linear scarring. The dilated bronchi are air filled or contain air and fluid. A supplying bronchus could not be identified. The patient underwent a left upper lobectomy. (e) Photograph of the specimen shows an opened bronchus that communicates with a 4 x 3 x 2-cm cavity, which contained mucus and firm debris. A second cavity 2 cm in diameter was also found. The lingula was not involved.

View larger version (88K): [in a new window]   Figure 2e.  Bronchial atresia in a 38-year-old man who experienced repeated pulmonary infections. (a, b) Frontal radiograph (a) and CT scan (b) show an appearance suggestive of congenital bronchial atresia. These images accompanied a request for biopsy of a mass in the left upper lobe. The mass contained air and fluid. There was distortion of the lung peripheral to the mass. (c) Follow-up posteroanterior radiograph obtained 1 month later shows a decrease in the fluid within the mass. Peripheral linear areas of increased opacity are also present, which likely represent scarring. (d) CT scan obtained at the same time as c shows distortion of the left upper lobe architecture with linear scarring. The dilated bronchi are air filled or contain air and fluid. A supplying bronchus could not be identified. The patient underwent a left upper lobectomy. (e) Photograph of the specimen shows an opened bronchus that communicates with a 4 x 3 x 2-cm cavity, which contained mucus and firm debris. A second cavity 2 cm in diameter was also found. The lingula was not involved.

View larger version (149K): [in a new window]   Figure 3a.  Congenital lobar emphysema (overinflation) in a 21-year-old man with sudden onset of shortness of breath and pain in the left anterior part of the chest that was worse during exertion and deep breathing. (a) Frontal radiograph shows hyperinflation of the left lower lobe with a paucity of blood vessels. There is a slight shift of the mediastinum to the right. (b) CT scan also shows marked overinflation of the left lower lobe. The patient underwent a left lower lobectomy. At gross examination, the bronchi were extremely thin and poorly developed. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows hyperaerated lung parenchyma with distended or hyperinflated alveoli and alveolar ducts. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) of a cross section of the bronchial tree shows thin, underdeveloped, immature-appearing cartilage plates (arrowhead).

View larger version (129K): [in a new window]   Figure 3b.  Congenital lobar emphysema (overinflation) in a 21-year-old man with sudden onset of shortness of breath and pain in the left anterior part of the chest that was worse during exertion and deep breathing. (a) Frontal radiograph shows hyperinflation of the left lower lobe with a paucity of blood vessels. There is a slight shift of the mediastinum to the right. (b) CT scan also shows marked overinflation of the left lower lobe. The patient underwent a left lower lobectomy. At gross examination, the bronchi were extremely thin and poorly developed. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows hyperaerated lung parenchyma with distended or hyperinflated alveoli and alveolar ducts. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) of a cross section of the bronchial tree shows thin, underdeveloped, immature-appearing cartilage plates (arrowhead).

View larger version (152K): [in a new window]   Figure 3c.  Congenital lobar emphysema (overinflation) in a 21-year-old man with sudden onset of shortness of breath and pain in the left anterior part of the chest that was worse during exertion and deep breathing. (a) Frontal radiograph shows hyperinflation of the left lower lobe with a paucity of blood vessels. There is a slight shift of the mediastinum to the right. (b) CT scan also shows marked overinflation of the left lower lobe. The patient underwent a left lower lobectomy. At gross examination, the bronchi were extremely thin and poorly developed. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows hyperaerated lung parenchyma with distended or hyperinflated alveoli and alveolar ducts. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) of a cross section of the bronchial tree shows thin, underdeveloped, immature-appearing cartilage plates (arrowhead).

View larger version (151K): [in a new window]   Figure 3d.  Congenital lobar emphysema (overinflation) in a 21-year-old man with sudden onset of shortness of breath and pain in the left anterior part of the chest that was worse during exertion and deep breathing. (a) Frontal radiograph shows hyperinflation of the left lower lobe with a paucity of blood vessels. There is a slight shift of the mediastinum to the right. (b) CT scan also shows marked overinflation of the left lower lobe. The patient underwent a left lower lobectomy. At gross examination, the bronchi were extremely thin and poorly developed. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows hyperaerated lung parenchyma with distended or hyperinflated alveoli and alveolar ducts. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) of a cross section of the bronchial tree shows thin, underdeveloped, immature-appearing cartilage plates (arrowhead).

View larger version (137K): [in a new window]   Figure 4a.  Congenital cystic adenomatoid malformation in a 31-year-old woman with a history of recurrent pneumonia. (a) Frontal radiograph shows a long air-fluid interface and a large rounded area of increased opacity that dips into the air. Medially, there is a second air-fluid interface and a small oval area of increased opacity. The volume of the left lung is increased with shift of the mediastinum to the right. (b) CT scan shows thin-walled spaces containing air and fluid in the basal segments of the left lower lobe. At the time of the frontal radiography study, the largest cyst contained air and fluid, whereas the two opaque cysts were filled with fluid. The diagnosis was confirmed at surgery. The specimen demonstrated an expansile, septated cystic mass. (Courtesy of N. L. Müller, MD, Vancouver Hospital, Vancouver, Canada.)

View larger version (95K): [in a new window]   Figure 4b.  Congenital cystic adenomatoid malformation in a 31-year-old woman with a history of recurrent pneumonia. (a) Frontal radiograph shows a long air-fluid interface and a large rounded area of increased opacity that dips into the air. Medially, there is a second air-fluid interface and a small oval area of increased opacity. The volume of the left lung is increased with shift of the mediastinum to the right. (b) CT scan shows thin-walled spaces containing air and fluid in the basal segments of the left lower lobe. At the time of the frontal radiography study, the largest cyst contained air and fluid, whereas the two opaque cysts were filled with fluid. The diagnosis was confirmed at surgery. The specimen demonstrated an expansile, septated cystic mass. (Courtesy of N. L. Müller, MD, Vancouver Hospital, Vancouver, Canada.)

View larger version (141K): [in a new window]   Figure 5a.  Pulmonary bronchogenic cyst in a 15-year-old boy with right lower lobe pneumonia. (a) Frontal radiograph shows a well-defined lesion with an air-fluid level near the superior margin of the right lower lobe. (b) CT scan shows the air-containing space and its well-defined wall. The lesion was resected. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows an intrapulmonary cystically dilated space (straight arrow) adjacent to the bronchovascular tree (curved arrow). (d) Photomicrograph (original magnification, x25; hematoxylin-eosin stain) of the cyst wall shows no epithelial lining. The presence of discontinuous, discrete smooth muscle bundles in the cyst wall (arrows) suggests a bronchogenic origin for the cyst.

View larger version (105K): [in a new window]   Figure 5b.  Pulmonary bronchogenic cyst in a 15-year-old boy with right lower lobe pneumonia. (a) Frontal radiograph shows a well-defined lesion with an air-fluid level near the superior margin of the right lower lobe. (b) CT scan shows the air-containing space and its well-defined wall. The lesion was resected. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows an intrapulmonary cystically dilated space (straight arrow) adjacent to the bronchovascular tree (curved arrow). (d) Photomicrograph (original magnification, x25; hematoxylin-eosin stain) of the cyst wall shows no epithelial lining. The presence of discontinuous, discrete smooth muscle bundles in the cyst wall (arrows) suggests a bronchogenic origin for the cyst.

View larger version (135K): [in a new window]   Figure 5c.  Pulmonary bronchogenic cyst in a 15-year-old boy with right lower lobe pneumonia. (a) Frontal radiograph shows a well-defined lesion with an air-fluid level near the superior margin of the right lower lobe. (b) CT scan shows the air-containing space and its well-defined wall. The lesion was resected. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows an intrapulmonary cystically dilated space (straight arrow) adjacent to the bronchovascular tree (curved arrow). (d) Photomicrograph (original magnification, x25; hematoxylin-eosin stain) of the cyst wall shows no epithelial lining. The presence of discontinuous, discrete smooth muscle bundles in the cyst wall (arrows) suggests a bronchogenic origin for the cyst.

View larger version (124K): [in a new window]   Figure 5d.  Pulmonary bronchogenic cyst in a 15-year-old boy with right lower lobe pneumonia. (a) Frontal radiograph shows a well-defined lesion with an air-fluid level near the superior margin of the right lower lobe. (b) CT scan shows the air-containing space and its well-defined wall. The lesion was resected. (c) Photomicrograph (original magnification, x2.5; hematoxylin-eosin stain) shows an intrapulmonary cystically dilated space (straight arrow) adjacent to the bronchovascular tree (curved arrow). (d) Photomicrograph (original magnification, x25; hematoxylin-eosin stain) of the cyst wall shows no epithelial lining. The presence of discontinuous, discrete smooth muscle bundles in the cyst wall (arrows) suggests a bronchogenic origin for the cyst.

View larger version (89K): [in a new window]   Figure 6a.  Displaced tracheal bronchus. (a) CT scan shows that the right upper lobe bronchus bifurcates. (b) CT scan shows that the apical bronchus originates from the trachea.

View larger version (88K): [in a new window]   Figure 6b.  Displaced tracheal bronchus. (a) CT scan shows that the right upper lobe bronchus bifurcates. (b) CT scan shows that the apical bronchus originates from the trachea.

View larger version (113K): [in a new window]   Figure 7a.  Accessory cardiac bronchus. Axial CT scan (a) and coronal reformatted image (b) show an accessory cardiac bronchus (arrowhead) that originates from the medial aspect of the right main bronchus. (Case courtesy of the Department of Radiology, University of Michigan, Ann Arbor.)

View larger version (76K): [in a new window]   Figure 7b.  Accessory cardiac bronchus. Axial CT scan (a) and coronal reformatted image (b) show an accessory cardiac bronchus (arrowhead) that originates from the medial aspect of the right main bronchus. (Case courtesy of the Department of Radiology, University of Michigan, Ann Arbor.)

View larger version (143K): [in a new window]   Figure 8a.  Congenital absence of the left pulmonary artery in a 46-year-old woman with an incorrect diagnosis of Swyer-James syndrome since 1987. (a) Frontal radiograph shows a right aortic arch and shift of the mediastinum to the left. The white line that parallels the medial aspect of the left scapula represents the displaced anterior junction line. The round area of increased opacity in the left middle lung zone had been stable for several years. (b, c) CT scans show absence of the left pulmonary artery. The area of increased attenuation in the left middle lung zone represents a cavity with material within it, which was presumed to be a fungus ball. There are also small areas of decreased attenuation in the periphery of the left lung, which represent paraseptal emphysema. PA = main pulmonary artery, R = right pulmonary artery.

View larger version (125K): [in a new window]   Figure 8b.  Congenital absence of the left pulmonary artery in a 46-year-old woman with an incorrect diagnosis of Swyer-James syndrome since 1987. (a) Frontal radiograph shows a right aortic arch and shift of the mediastinum to the left. The white line that parallels the medial aspect of the left scapula represents the displaced anterior junction line. The round area of increased opacity in the left middle lung zone had been stable for several years. (b, c) CT scans show absence of the left pulmonary artery. The area of increased attenuation in the left middle lung zone represents a cavity with material within it, which was presumed to be a fungus ball. There are also small areas of decreased attenuation in the periphery of the left lung, which represent paraseptal emphysema. PA = main pulmonary artery, R = right pulmonary artery.

View larger version (124K): [in a new window]   Figure 8c.  Congenital absence of the left pulmonary artery in a 46-year-old woman with an incorrect diagnosis of Swyer-James syndrome since 1987. (a) Frontal radiograph shows a right aortic arch and shift of the mediastinum to the left. The white line that parallels the medial aspect of the left scapula represents the displaced anterior junction line. The round area of increased opacity in the left middle lung zone had been stable for several years. (b, c) CT scans show absence of the left pulmonary artery. The area of increased attenuation in the left middle lung zone represents a cavity with material within it, which was presumed to be a fungus ball. There are also small areas of decreased attenuation in the periphery of the left lung, which represent paraseptal emphysema. PA = main pulmonary artery, R = right pulmonary artery.

View larger version (126K): [in a new window]   Figure 9a.  Anomalous origin of the left pulmonary artery from the right in an asymptomatic adult. CT scan (a) and MR angiogram (b) show the course of an anomalous left pulmonary artery. Originating from the posterior surface of the right pulmonary artery, it passes posteriorly adjacent to the trachea and turns to the left to enter the left hilum. (Case courtesy of Eva Castaner, MD, Hospital Parc Jauli, Sabadell, Spain.)

View larger version (107K): [in a new window]   Figure 9b.  Anomalous origin of the left pulmonary artery from the right in an asymptomatic adult. CT scan (a) and MR angiogram (b) show the course of an anomalous left pulmonary artery. Originating from the posterior surface of the right pulmonary artery, it passes posteriorly adjacent to the trachea and turns to the left to enter the left hilum. (Case courtesy of Eva Castaner, MD, Hospital Parc Jauli, Sabadell, Spain.)

View larger version (170K): [in a new window]   Figure 10a.  Total anomalous pulmonary venous return in a young woman with a patent ductus arteriosus. (a) Anteroposterior radiograph shows that the mediastinum is widened, particularly on the left side, where there is a communication with the left brachiocephalic vein. The changes on the right side are minimal. Increased arterial flow is readily apparent. (b) Left lateral radiograph shows a large high aortic arch, enlargement of the right cardiac chambers, and overfilled pulmonary arteries.

View larger version (146K): [in a new window]   Figure 10b.  Total anomalous pulmonary venous return in a young woman with a patent ductus arteriosus. (a) Anteroposterior radiograph shows that the mediastinum is widened, particularly on the left side, where there is a communication with the left brachiocephalic vein. The changes on the right side are minimal. Increased arterial flow is readily apparent. (b) Left lateral radiograph shows a large high aortic arch, enlargement of the right cardiac chambers, and overfilled pulmonary arteries.

View larger version (51K): [in a new window]   Figure 11.  Diagram shows types of total anomalous pulmonary venous return. CPV = common pulmonary vein, CS = coronary sinus, IVC = inferior vena cava, LA = left atrium, LPV = left pulmonary vein, LV = left ventricle, PVS = portal venous system, RA = right atrium, RPV = right pulmonary vein, RV = right ventricle. Permission to reprint this figure electronically was denied by the publisher. Please see print version.

View larger version (126K): [in a new window]   Figure 12a.  Anomalous pulmonary vein from the left upper lobe in a 54-year-old man. (a-c) CT scans show drainage of an anomalous left upper lobe vein (arrow) into the left brachiocephalic vein. Distinction from a left superior vena cava is made with CT (see text). (d) Coronal MR angiogram of another patient shows anomalous drainage of a left upper lobe vein into the left brachiocephalic vein (right arrow) and partial drainage of the right upper lobe into the superior vena cava (left arrow). (Fig 12d courtesy of C. Higgins, MD, University of California, San Francisco.)

View larger version (124K): [in a new window]   Figure 12b.  Anomalous pulmonary vein from the left upper lobe in a 54-year-old man. (a-c) CT scans show drainage of an anomalous left upper lobe vein (arrow) into the left brachiocephalic vein. Distinction from a left superior vena cava is made with CT (see text). (d) Coronal MR angiogram of another patient shows anomalous drainage of a left upper lobe vein into the left brachiocephalic vein (right arrow) and partial drainage of the right upper lobe into the superior vena cava (left arrow). (Fig 12d courtesy of C. Higgins, MD, University of California, San Francisco.)

View larger version (137K): [in a new window]   Figure 12c.  Anomalous pulmonary vein from the left upper lobe in a 54-year-old man. (a-c) CT scans show drainage of an anomalous left upper lobe vein (arrow) into the left brachiocephalic vein. Distinction from a left superior vena cava is made with CT (see text). (d) Coronal MR angiogram of another patient shows anomalous drainage of a left upper lobe vein into the left brachiocephalic vein (right arrow) and partial drainage of the right upper lobe into the superior vena cava (left arrow). (Fig 12d courtesy of C. Higgins, MD, University of California, San Francisco.)

View larger version (133K): [in a new window]   Figure 12d.  Anomalous pulmonary vein from the left upper lobe in a 54-year-old man. (a-c) CT scans show drainage of an anomalous left upper lobe vein (arrow) into the left brachiocephalic vein. Distinction from a left superior vena cava is made with CT (see text). (d) Coronal MR angiogram of another patient shows anomalous drainage of a left upper lobe vein into the left brachiocephalic vein (right arrow) and partial drainage of the right upper lobe into the superior vena cava (left arrow). (Fig 12d courtesy of C. Higgins, MD, University of California, San Francisco.)

View larger version (116K): [in a new window]   Figure 13a.  Anomalous pulmonary vein from the right upper lobe in a 69-year-old man. (a) CT scan shows a connection from an anomalous right upper lobe vein (arrow) to the superior vena cava. (b) Coronal MR angiogram of another patient shows anomalous drainage of a right upper lobe vein into the superior vena cava (arrow). (Fig 13b courtesy of C. Higgins, MD.)

View larger version (141K): [in a new window]   Figure 13b.  Anomalous pulmonary vein from the right upper lobe in a 69-year-old man. (a) CT scan shows a connection from an anomalous right upper lobe vein (arrow) to the superior vena cava. (b) Coronal MR angiogram of another patient shows anomalous drainage of a right upper lobe vein into the superior vena cava (arrow). (Fig 13b courtesy of C. Higgins, MD.)

View larger version (131K): [in a new window]   Figure 14a.  Arteriovenous malformation. (a) Frontal radiograph shows a nodular area of increased opacity in the right middle lobe. (b) CT scans show an arteriovenous malformation. The supplying and draining vessels are clearly seen. The lateral segment of the right middle lobe and the lingula are the most common locations for a solitary arteriovenous malformation. (Case courtesy of J. Wandtke, MD, University of Rochester, Rochester, NY.)

View larger version (60K): [in a new window]   Figure 14b.  Arteriovenous malformation. (a) Frontal radiograph shows a nodular area of increased opacity in the right middle lobe. (b) CT scans show an arteriovenous malformation. The supplying and draining vessels are clearly seen. The lateral segment of the right middle lobe and the lingula are the most common locations for a solitary arteriovenous malformation. (Case courtesy of J. Wandtke, MD, University of Rochester, Rochester, NY.)

View larger version (143K): [in a new window]   Figure 15a.  Hypogenetic lung (scimitar) syndrome in a 68-year-old asymptomatic woman who underwent a routine preoperative study. (a) Frontal radiograph shows shift of the mediastinum to the right secondary to volume loss. The patient does not have a right upper lobe. The right hilum appears small; however, the pulmonary artery was of normal size on CT scans. An oblong area of increased opacity (arrow) parallels the right border of the heart. (b) Volume-rendered CT image shows the oblong area of increased attenuation (arrow) parallel to the right border of the heart. This finding represents an anomalous vein that drains into the inferior vena cava (arrowhead).

View larger version (143K): [in a new window]   Figure 15b.  Hypogenetic lung (scimitar) syndrome in a 68-year-old asymptomatic woman who underwent a routine preoperative study. (a) Frontal radiograph shows shift of the mediastinum to the right secondary to volume loss. The patient does not have a right upper lobe. The right hilum appears small; however, the pulmonary artery was of normal size on CT scans. An oblong area of increased opacity (arrow) parallels the right border of the heart. (b) Volume-rendered CT image shows the oblong area of increased attenuation (arrow) parallel to the right border of the heart. This finding represents an anomalous vein that drains into the inferior vena cava (arrowhead).

View larger version (129K): [in a new window]   Figure 16a.  Sequestration in a 37-year-old woman with a productive cough. (a, b) Conventional radiographs show an area of increased opacity in the left lower lobe. (c) CT scan shows a vessel that arises from the aorta and supplies the sequestered lung tissue. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) shows a dilated bronchiole with a mucous plug (straight arrow); the injury pattern of bronchiolitis obliterans-organizing pneumonia and fibrosis are seen in the surrounding lung parenchyma (curved arrow). (e) Photomicrograph (original magnification, x20; hematoxylin-eosin stain) shows the wall of the systemic artery (straight arrow) with an adjacent bronchus (curved arrow).

View larger version (124K): [in a new window]   Figure 16b.  Sequestration in a 37-year-old woman with a productive cough. (a, b) Conventional radiographs show an area of increased opacity in the left lower lobe. (c) CT scan shows a vessel that arises from the aorta and supplies the sequestered lung tissue. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) shows a dilated bronchiole with a mucous plug (straight arrow); the injury pattern of bronchiolitis obliterans-organizing pneumonia and fibrosis are seen in the surrounding lung parenchyma (curved arrow). (e) Photomicrograph (original magnification, x20; hematoxylin-eosin stain) shows the wall of the systemic artery (straight arrow) with an adjacent bronchus (curved arrow).

View larger version (127K): [in a new window]   Figure 16c.  Sequestration in a 37-year-old woman with a productive cough. (a, b) Conventional radiographs show an area of increased opacity in the left lower lobe. (c) CT scan shows a vessel that arises from the aorta and supplies the sequestered lung tissue. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) shows a dilated bronchiole with a mucous plug (straight arrow); the injury pattern of bronchiolitis obliterans-organizing pneumonia and fibrosis are seen in the surrounding lung parenchyma (curved arrow). (e) Photomicrograph (original magnification, x20; hematoxylin-eosin stain) shows the wall of the systemic artery (straight arrow) with an adjacent bronchus (curved arrow).

View larger version (159K): [in a new window]   Figure 16d.  Sequestration in a 37-year-old woman with a productive cough. (a, b) Conventional radiographs show an area of increased opacity in the left lower lobe. (c) CT scan shows a vessel that arises from the aorta and supplies the sequestered lung tissue. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) shows a dilated bronchiole with a mucous plug (straight arrow); the injury pattern of bronchiolitis obliterans-organizing pneumonia and fibrosis are seen in the surrounding lung parenchyma (curved arrow). (e) Photomicrograph (original magnification, x20; hematoxylin-eosin stain) shows the wall of the systemic artery (straight arrow) with an adjacent bronchus (curved arrow).

View larger version (145K): [in a new window]   Figure 16e.  Sequestration in a 37-year-old woman with a productive cough. (a, b) Conventional radiographs show an area of increased opacity in the left lower lobe. (c) CT scan shows a vessel that arises from the aorta and supplies the sequestered lung tissue. (d) Photomicrograph (original magnification, x7; hematoxylin-eosin stain) shows a dilated bronchiole with a mucous plug (straight arrow); the injury pattern of bronchiolitis obliterans-organizing pneumonia and fibrosis are seen in the surrounding lung parenchyma (curved arrow). (e) Photomicrograph (original magnification, x20; hematoxylin-eosin stain) shows the wall of the systemic artery (straight arrow) with an adjacent bronchus (curved arrow).

View larger version (132K): [in a new window]   Figure 17a.  Right intralobar sequestration and left extralobar sequestration in an 18-year-old woman with a history of repeated infections of the right lung. Frontal (a) and lateral (b) radiographs show extensive abnormality in the right lower lobe. The abnormality consists of cavitation, as evidenced by an air-fluid level. There is some increased opacity in the adjacent parenchyma, which may be due to pneumonia. There is abnormal increased opacity behind the heart medially. The left hemidiaphragm appears to join a posteromedial mass: an extralobar sequestration. (Case courtesy of G. Barnes, MD, Tucson, Ariz.)

View larger version (130K): [in a new window]   Figure 17b.  Right intralobar sequestration and left extralobar sequestration in an 18-year-old woman with a history of repeated infections of the right lung. Frontal (a) and lateral (b) radiographs show extensive abnormality in the right lower lobe. The abnormality consists of cavitation, as evidenced by an air-fluid level. There is some increased opacity in the adjacent parenchyma, which may be due to pneumonia. There is abnormal increased opacity behind the heart medially. The left hemidiaphragm appears to join a posteromedial mass: an extralobar sequestration. (Case courtesy of G. Barnes, MD, Tucson, Ariz.)