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Rings, Slings, and Other Things: Vascular Compression of the Infant Trachea Updated from the Midcentury to the Millennium-The Legacy of Robert E. Gross, MD, and Edward B. D. Neuhauser, MD¹

¹ From the Department of Radiology, Babies & Children’s Hospital of New York, 3959 Broadway, BHN 3-318, New York, NY 10032. Received August 9, 1999; revision requested September 14; final revision received March 7, 2000; accepted March 24. Address correspondence to the author (e-mail: berdonw@cpmc3.cpmc.columbia.edu).

View larger version (124K): [in a new window]   Figure 1. Photograph of the physicians who were active at Children’s Hospital Boston, obtained in 1950. Front row, left to right: F. Ingraham, MD, neurosurgery; S. Farber, MD, pathology; and W. Green, MD, orthopedics. Back row, left to right: C. Janeway, MD, pediatrics; G. Brugler, hospital director; Edward B. D. Neuhauser, MD, pediatric radiology; and R. Gross, MD, pediatric surgery. (Photograph courtesy of N. Thorne Griscom, MD, Children’s Hospital Boston, Mass.)

View larger version (89K): [in a new window]   Figure 2. Diagram of a typical double aortic arch vascular ring. The ductus arteriosus (DA) is the ductal remnant, which also must be divided. The right posterior arch may be dominant. In some patients, the left anterior arch is dominant. Ao = aorta, LC = left common carotid artery, LS = left subclavian artery, O = esophagus, PA = main pulmonary artery, RC = right common carotid artery, RS = right subclavian artery, T = trachea. (Reprinted, with permission, from reference 5.)

View larger version (68K): [in a new window]   Figure 3. Imaging in the late 1950s. A, Lateral barium esophagram shows posterior indentation in the esophagus. B, Left ventricular angiogram shows a dominant right arch (arrow) and smaller left arch. The catheter was passed from the inferior vena cava through the foramen ovale into the left atrium and then into the left ventricle. (Photographs courtesy of John Kirkpatrick, MD, St Christopher’s Hospital, Philadelphia, Pa.)

View larger version (83K): [in a new window]   Figure 4a. (a, b) Electron-beam CT images of a double aortic arch. (a) The dominant right arch (arrow) and smaller left arch (arrowhead) are diagnostic of a double aortic arch vascular ring. (b) Image obtained slightly inferior to a shows the uniting descending aortas (arrow) slightly to the left of the midline. (Images courtesy of J. Kuhn, MD, Buffalo Children’s Hospital, New York.) (c-e) Three-dimensional spiral CT reconstructions of a double aortic arch with a dominant right arch. (c) Anterior and (d) posterior views, and (e) view from above are shown. (Images courtesy of Ronald Cohen, MD, Children’s Hospital, Oakland, Calif.)

View larger version (93K): [in a new window]   Figure 4b. (a, b) Electron-beam CT images of a double aortic arch. (a) The dominant right arch (arrow) and smaller left arch (arrowhead) are diagnostic of a double aortic arch vascular ring. (b) Image obtained slightly inferior to a shows the uniting descending aortas (arrow) slightly to the left of the midline. (Images courtesy of J. Kuhn, MD, Buffalo Children’s Hospital, New York.) (c-e) Three-dimensional spiral CT reconstructions of a double aortic arch with a dominant right arch. (c) Anterior and (d) posterior views, and (e) view from above are shown. (Images courtesy of Ronald Cohen, MD, Children’s Hospital, Oakland, Calif.)

View larger version (68K): [in a new window]   Figure 4c. (a, b) Electron-beam CT images of a double aortic arch. (a) The dominant right arch (arrow) and smaller left arch (arrowhead) are diagnostic of a double aortic arch vascular ring. (b) Image obtained slightly inferior to a shows the uniting descending aortas (arrow) slightly to the left of the midline. (Images courtesy of J. Kuhn, MD, Buffalo Children’s Hospital, New York.) (c-e) Three-dimensional spiral CT reconstructions of a double aortic arch with a dominant right arch. (c) Anterior and (d) posterior views, and (e) view from above are shown. (Images courtesy of Ronald Cohen, MD, Children’s Hospital, Oakland, Calif.)

View larger version (83K): [in a new window]   Figure 4d. (a, b) Electron-beam CT images of a double aortic arch. (a) The dominant right arch (arrow) and smaller left arch (arrowhead) are diagnostic of a double aortic arch vascular ring. (b) Image obtained slightly inferior to a shows the uniting descending aortas (arrow) slightly to the left of the midline. (Images courtesy of J. Kuhn, MD, Buffalo Children’s Hospital, New York.) (c-e) Three-dimensional spiral CT reconstructions of a double aortic arch with a dominant right arch. (c) Anterior and (d) posterior views, and (e) view from above are shown. (Images courtesy of Ronald Cohen, MD, Children’s Hospital, Oakland, Calif.)

View larger version (71K): [in a new window]   Figure 4e. (a, b) Electron-beam CT images of a double aortic arch. (a) The dominant right arch (arrow) and smaller left arch (arrowhead) are diagnostic of a double aortic arch vascular ring. (b) Image obtained slightly inferior to a shows the uniting descending aortas (arrow) slightly to the left of the midline. (Images courtesy of J. Kuhn, MD, Buffalo Children’s Hospital, New York.) (c-e) Three-dimensional spiral CT reconstructions of a double aortic arch with a dominant right arch. (c) Anterior and (d) posterior views, and (e) view from above are shown. (Images courtesy of Ronald Cohen, MD, Children’s Hospital, Oakland, Calif.)

View larger version (139K): [in a new window]   Figure 5a. MR imaging in the late 1980s. (a, b) T1-weighted spin-echo images (repetition time msec/echo time msec, 500/30) of a double aortic arch. (a) Posterior coronal image shows two arches meeting to form the descending aorta; the left arch is slightly bigger. (b) The more anterior image shows the two aortic arches on either side of the trachea, which is slightly narrowed. (c) Anterior and (d) posterior T1-weighted, spin-echo, three-dimensional MR reconstructions (750/30) of the double aortic arch in a and b. In c and d, AAo = ascending aorta, DA = descending aorta, DAo = descending aorta, ES = esophagus (yellow), LAA = left aortic arch, LCCA = left common carotid artery, LSA = left subclavian artery, RAA = right aortic arch, RCCA = right common carotid artery, RSA = right subclavian artery, TR = trachea (white). (Images in c and d courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (131K): [in a new window]   Figure 5b. MR imaging in the late 1980s. (a, b) T1-weighted spin-echo images (repetition time msec/echo time msec, 500/30) of a double aortic arch. (a) Posterior coronal image shows two arches meeting to form the descending aorta; the left arch is slightly bigger. (b) The more anterior image shows the two aortic arches on either side of the trachea, which is slightly narrowed. (c) Anterior and (d) posterior T1-weighted, spin-echo, three-dimensional MR reconstructions (750/30) of the double aortic arch in a and b. In c and d, AAo = ascending aorta, DA = descending aorta, DAo = descending aorta, ES = esophagus (yellow), LAA = left aortic arch, LCCA = left common carotid artery, LSA = left subclavian artery, RAA = right aortic arch, RCCA = right common carotid artery, RSA = right subclavian artery, TR = trachea (white). (Images in c and d courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (121K): [in a new window]   Figure 5c. MR imaging in the late 1980s. (a, b) T1-weighted spin-echo images (repetition time msec/echo time msec, 500/30) of a double aortic arch. (a) Posterior coronal image shows two arches meeting to form the descending aorta; the left arch is slightly bigger. (b) The more anterior image shows the two aortic arches on either side of the trachea, which is slightly narrowed. (c) Anterior and (d) posterior T1-weighted, spin-echo, three-dimensional MR reconstructions (750/30) of the double aortic arch in a and b. In c and d, AAo = ascending aorta, DA = descending aorta, DAo = descending aorta, ES = esophagus (yellow), LAA = left aortic arch, LCCA = left common carotid artery, LSA = left subclavian artery, RAA = right aortic arch, RCCA = right common carotid artery, RSA = right subclavian artery, TR = trachea (white). (Images in c and d courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (113K): [in a new window]   Figure 5d. MR imaging in the late 1980s. (a, b) T1-weighted spin-echo images (repetition time msec/echo time msec, 500/30) of a double aortic arch. (a) Posterior coronal image shows two arches meeting to form the descending aorta; the left arch is slightly bigger. (b) The more anterior image shows the two aortic arches on either side of the trachea, which is slightly narrowed. (c) Anterior and (d) posterior T1-weighted, spin-echo, three-dimensional MR reconstructions (750/30) of the double aortic arch in a and b. In c and d, AAo = ascending aorta, DA = descending aorta, DAo = descending aorta, ES = esophagus (yellow), LAA = left aortic arch, LCCA = left common carotid artery, LSA = left subclavian artery, RAA = right aortic arch, RCCA = right common carotid artery, RSA = right subclavian artery, TR = trachea (white). (Images in c and d courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (92K): [in a new window]   Figure 6a. (a) Anteroposterior view of necropsy specimen shows tracheal indentation (arrow) by innominate artery. The infant died of unrelated causes. (Photograph courtesy of W. Blanc, MD, Babies & Children’s Hospital of New York.) (b) Sagittal (left) and coronal (right) T1-weighted MR images (750/30). Left: The innominate artery (a) is anterior to and indenting the trachea (t). The vein (v) and thymus (th) are noted. Note the cervical extension of the thymus. Right: The innominate artery (arrows) passes in front and to the right of the trachea (t). (Images courtesy of G. Mandell, MD, DuPont Institute, Wilmington, Del.) (c) Tracheomalacia, or innominate artery syndrome. Histologic section of the midthoracic esophagus (E) and trachea (T) obtained at autopsy shows the tracheal cartilage is reduced in length and the membranous trachea is increased. The overall tracheal lumen is flattened. (Hematoxylin-eosin stain; original magnification, x30.) (Photograph of specimen courtesy of J. L. Emery, MD, Sheffield, England.) (d) Electron-beam CT images of tracheomalacia, or innominate artery syndrome. The same image level is noted to change the tracheal caliber from narrowed to normal during the respiratory cycle. The trachea is outlined in white. A = innominate artery. (Images courtesy of Wilbur Smith, MD, University of Iowa Hospitals & Clinics, Iowa City, Iowa.)

View larger version (89K): [in a new window]   Figure 6b. (a) Anteroposterior view of necropsy specimen shows tracheal indentation (arrow) by innominate artery. The infant died of unrelated causes. (Photograph courtesy of W. Blanc, MD, Babies & Children’s Hospital of New York.) (b) Sagittal (left) and coronal (right) T1-weighted MR images (750/30). Left: The innominate artery (a) is anterior to and indenting the trachea (t). The vein (v) and thymus (th) are noted. Note the cervical extension of the thymus. Right: The innominate artery (arrows) passes in front and to the right of the trachea (t). (Images courtesy of G. Mandell, MD, DuPont Institute, Wilmington, Del.) (c) Tracheomalacia, or innominate artery syndrome. Histologic section of the midthoracic esophagus (E) and trachea (T) obtained at autopsy shows the tracheal cartilage is reduced in length and the membranous trachea is increased. The overall tracheal lumen is flattened. (Hematoxylin-eosin stain; original magnification, x30.) (Photograph of specimen courtesy of J. L. Emery, MD, Sheffield, England.) (d) Electron-beam CT images of tracheomalacia, or innominate artery syndrome. The same image level is noted to change the tracheal caliber from narrowed to normal during the respiratory cycle. The trachea is outlined in white. A = innominate artery. (Images courtesy of Wilbur Smith, MD, University of Iowa Hospitals & Clinics, Iowa City, Iowa.)

View larger version (141K): [in a new window]   Figure 6c. (a) Anteroposterior view of necropsy specimen shows tracheal indentation (arrow) by innominate artery. The infant died of unrelated causes. (Photograph courtesy of W. Blanc, MD, Babies & Children’s Hospital of New York.) (b) Sagittal (left) and coronal (right) T1-weighted MR images (750/30). Left: The innominate artery (a) is anterior to and indenting the trachea (t). The vein (v) and thymus (th) are noted. Note the cervical extension of the thymus. Right: The innominate artery (arrows) passes in front and to the right of the trachea (t). (Images courtesy of G. Mandell, MD, DuPont Institute, Wilmington, Del.) (c) Tracheomalacia, or innominate artery syndrome. Histologic section of the midthoracic esophagus (E) and trachea (T) obtained at autopsy shows the tracheal cartilage is reduced in length and the membranous trachea is increased. The overall tracheal lumen is flattened. (Hematoxylin-eosin stain; original magnification, x30.) (Photograph of specimen courtesy of J. L. Emery, MD, Sheffield, England.) (d) Electron-beam CT images of tracheomalacia, or innominate artery syndrome. The same image level is noted to change the tracheal caliber from narrowed to normal during the respiratory cycle. The trachea is outlined in white. A = innominate artery. (Images courtesy of Wilbur Smith, MD, University of Iowa Hospitals & Clinics, Iowa City, Iowa.)

View larger version (62K): [in a new window]   Figure 6d. (a) Anteroposterior view of necropsy specimen shows tracheal indentation (arrow) by innominate artery. The infant died of unrelated causes. (Photograph courtesy of W. Blanc, MD, Babies & Children’s Hospital of New York.) (b) Sagittal (left) and coronal (right) T1-weighted MR images (750/30). Left: The innominate artery (a) is anterior to and indenting the trachea (t). The vein (v) and thymus (th) are noted. Note the cervical extension of the thymus. Right: The innominate artery (arrows) passes in front and to the right of the trachea (t). (Images courtesy of G. Mandell, MD, DuPont Institute, Wilmington, Del.) (c) Tracheomalacia, or innominate artery syndrome. Histologic section of the midthoracic esophagus (E) and trachea (T) obtained at autopsy shows the tracheal cartilage is reduced in length and the membranous trachea is increased. The overall tracheal lumen is flattened. (Hematoxylin-eosin stain; original magnification, x30.) (Photograph of specimen courtesy of J. L. Emery, MD, Sheffield, England.) (d) Electron-beam CT images of tracheomalacia, or innominate artery syndrome. The same image level is noted to change the tracheal caliber from narrowed to normal during the respiratory cycle. The trachea is outlined in white. A = innominate artery. (Images courtesy of Wilbur Smith, MD, University of Iowa Hospitals & Clinics, Iowa City, Iowa.)

View larger version (66K): [in a new window]   Figure 7a. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (114K): [in a new window]   Figure 7b. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (133K): [in a new window]   Figure 7c. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (119K): [in a new window]   Figure 7d. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (134K): [in a new window]   Figure 7e. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (134K): [in a new window]   Figure 7f. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (164K): [in a new window]   Figure 7g. (a) Diagram of anomalous left pulmonary artery, or pulmonary sling. Br. = bronchus, L.A. = ligamentous remnant of ductus arteriosus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery (in anomalous retrotracheal course), L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b) Lateral airway esophagrams show an anomalous left pulmonary artery (arrow) passing between the esophagus behind it and trachea in front of it. (c) Transverse T1-weighted MR image (750/30) obtained in another patient shows an anomalous left pulmonary artery (arrow) passing posteriorly and to the left between the trachea and the esophagus. Note the emphysematous right lung. The patient survived following surgical correction. (Image courtesy of S. Sane, MD, Minneapolis, Minn.) (d) Autopsy findings in an anomalous left artery, or pulmonary sling, with long-segment tracheal stenosis. Patient had an incidental coexistent anomalous right subclavian artery passing behind the esophagus. The tracheal stenosis, as viewed externally, is reflected in the change in diameter of the trachea involving approximately the last eight tracheal rings above the carina. Note the characteristic inverted "T" appearance of the splayed right and left main bronchi. L = left pulmonary artery, R = right pulmonary artery, S = anomalous subclavian artery. (Reprinted, with permission, from reference 11.) (e) Anteroposterior tracheobronchogram shows severe complete O-ring tracheal stenosis. Note the inverted trachea (T). The distal trachea is narrower than the bronchi. The patient, who also had a tracheal bronchus (arrow) to the right upper lobe, died after recurrent respiratory decompensations that seemed to be bronchiolitis. An anomalous left pulmonary artery was detected only at autopsy. (Image courtesy of B. Benjamin, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (f) Histologic trachea specimen obtained at autopsy in the patient in e shows complete O-ring tracheal stenosis. (Image courtesy of P. Bale, MD, Royal Alexandra Children’s Hospital, Sydney, Australia.) (g) Three-dimensional reconstructed MR image (750/30) obtained in another patient, as viewed from above, shows an anomalous left pulmonary artery, or pulmonary sling. This patient also had an anomalous right subclavian artery as an incidental finding and tracheal stenosis from complete rings. The left pulmonary artery (L) arises from the right pulmonary artery (R) and passes to the left behind the trachea and left main bronchus (white). M = main pulmonary artery. (Image courtesy of Beverley Newman, MD, Children’s Hospital, Pittsburgh, Pa.)

View larger version (73K): [in a new window]   Figure 8a. (a) Typical lateral barium esophagram shows an anomalous right subclavian artery with a normal trachea. The indentation (arrow) in the esophagus is oblique and shallow. Solids are lodged in this area in an 8-year-old child who grew up consuming liquids only. The family refused surgery. (b) An illustration of dysphagia lusoria in a 62-year-old woman with lifelong obstructed deglutition, from the 1794 article by Bayford. Note the careful placement of the anomalous vessel (arrows) between the trachea and the esophagus on the anterior (top) and lateral (bottom) views. This is the always quoted so-called first reference on anomalous right subclavian artery. (Reprinted from reference 15.)

View larger version (63K): [in a new window]   Figure 8b. (a) Typical lateral barium esophagram shows an anomalous right subclavian artery with a normal trachea. The indentation (arrow) in the esophagus is oblique and shallow. Solids are lodged in this area in an 8-year-old child who grew up consuming liquids only. The family refused surgery. (b) An illustration of dysphagia lusoria in a 62-year-old woman with lifelong obstructed deglutition, from the 1794 article by Bayford. Note the careful placement of the anomalous vessel (arrows) between the trachea and the esophagus on the anterior (top) and lateral (bottom) views. This is the always quoted so-called first reference on anomalous right subclavian artery. (Reprinted from reference 15.)

View larger version (98K): [in a new window]   Figure 9a. (a) Lateral esophagram shows unusually prominent posterior indentation (arrow), from an anomalous right subclavian artery arising from a diverticulum, in the esophagus of an infant who had feeding problems. The airway is normal. (b) Original image of the Kommerell diverticulum, with an anomalous right subclavian artery arising from the diverticulum off the left aortic arch (posterior view). (Reprinted, with permission, from reference 17.) (c, d) T1-weighted MR images (750/30) obtained in the patient in a. (c) Coronal image shows a normal trachea. (d) More posterior coronal image shows a diverticulum (arrow) arising from the right side of the left descending aorta. The diverticulum gave rise to an anomalous right subclavian artery.

View larger version (85K): [in a new window]   Figure 9b. (a) Lateral esophagram shows unusually prominent posterior indentation (arrow), from an anomalous right subclavian artery arising from a diverticulum, in the esophagus of an infant who had feeding problems. The airway is normal. (b) Original image of the Kommerell diverticulum, with an anomalous right subclavian artery arising from the diverticulum off the left aortic arch (posterior view). (Reprinted, with permission, from reference 17.) (c, d) T1-weighted MR images (750/30) obtained in the patient in a. (c) Coronal image shows a normal trachea. (d) More posterior coronal image shows a diverticulum (arrow) arising from the right side of the left descending aorta. The diverticulum gave rise to an anomalous right subclavian artery.

View larger version (94K): [in a new window]   Figure 9c. (a) Lateral esophagram shows unusually prominent posterior indentation (arrow), from an anomalous right subclavian artery arising from a diverticulum, in the esophagus of an infant who had feeding problems. The airway is normal. (b) Original image of the Kommerell diverticulum, with an anomalous right subclavian artery arising from the diverticulum off the left aortic arch (posterior view). (Reprinted, with permission, from reference 17.) (c, d) T1-weighted MR images (750/30) obtained in the patient in a. (c) Coronal image shows a normal trachea. (d) More posterior coronal image shows a diverticulum (arrow) arising from the right side of the left descending aorta. The diverticulum gave rise to an anomalous right subclavian artery.

View larger version (98K): [in a new window]   Figure 9d. (a) Lateral esophagram shows unusually prominent posterior indentation (arrow), from an anomalous right subclavian artery arising from a diverticulum, in the esophagus of an infant who had feeding problems. The airway is normal. (b) Original image of the Kommerell diverticulum, with an anomalous right subclavian artery arising from the diverticulum off the left aortic arch (posterior view). (Reprinted, with permission, from reference 17.) (c, d) T1-weighted MR images (750/30) obtained in the patient in a. (c) Coronal image shows a normal trachea. (d) More posterior coronal image shows a diverticulum (arrow) arising from the right side of the left descending aorta. The diverticulum gave rise to an anomalous right subclavian artery.

View larger version (67K): [in a new window]   Figure 10a. (a) Diagram illustrates absence of the pulmonary valve, with huge central pulmonary arteries compressing the main bronchi. Br. = bronchus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery, L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b, c) Radiographs show absent pulmonary valve with retained fetal lung fluid from airway obstruction. (b) Anteroposterior radiograph shows an opaque right lung. (c) Anteroposterior radiograph obtained 8 hours later shows hyperinflation. Note the right hilar mass on the dilated right pulmonary artery (arrow). (d) Echocardiogram in the subxiphoid long-axis projection shows a huge main pulmonary artery (MPA). Only dysplastic valve tissue is seen on real-time studies with massive pulmonary insufficiency. (e) Echocardiogram in the subxiphoid ventricular short-axis projection, obtained in the patient in e, shows outflow annulus narrowing (arrow) below the huge main pulmonary artery (MPA). The patient survived after undergoing valve replacement and reduction of the central aneurysm of pulmonary arteries. (Images in d and e courtesy of F. Bierman, MD, New York, NY.)

View larger version (137K): [in a new window]   Figure 10b. (a) Diagram illustrates absence of the pulmonary valve, with huge central pulmonary arteries compressing the main bronchi. Br. = bronchus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery, L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b, c) Radiographs show absent pulmonary valve with retained fetal lung fluid from airway obstruction. (b) Anteroposterior radiograph shows an opaque right lung. (c) Anteroposterior radiograph obtained 8 hours later shows hyperinflation. Note the right hilar mass on the dilated right pulmonary artery (arrow). (d) Echocardiogram in the subxiphoid long-axis projection shows a huge main pulmonary artery (MPA). Only dysplastic valve tissue is seen on real-time studies with massive pulmonary insufficiency. (e) Echocardiogram in the subxiphoid ventricular short-axis projection, obtained in the patient in e, shows outflow annulus narrowing (arrow) below the huge main pulmonary artery (MPA). The patient survived after undergoing valve replacement and reduction of the central aneurysm of pulmonary arteries. (Images in d and e courtesy of F. Bierman, MD, New York, NY.)

View larger version (135K): [in a new window]   Figure 10c. (a) Diagram illustrates absence of the pulmonary valve, with huge central pulmonary arteries compressing the main bronchi. Br. = bronchus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery, L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b, c) Radiographs show absent pulmonary valve with retained fetal lung fluid from airway obstruction. (b) Anteroposterior radiograph shows an opaque right lung. (c) Anteroposterior radiograph obtained 8 hours later shows hyperinflation. Note the right hilar mass on the dilated right pulmonary artery (arrow). (d) Echocardiogram in the subxiphoid long-axis projection shows a huge main pulmonary artery (MPA). Only dysplastic valve tissue is seen on real-time studies with massive pulmonary insufficiency. (e) Echocardiogram in the subxiphoid ventricular short-axis projection, obtained in the patient in e, shows outflow annulus narrowing (arrow) below the huge main pulmonary artery (MPA). The patient survived after undergoing valve replacement and reduction of the central aneurysm of pulmonary arteries. (Images in d and e courtesy of F. Bierman, MD, New York, NY.)

View larger version (132K): [in a new window]   Figure 10d. (a) Diagram illustrates absence of the pulmonary valve, with huge central pulmonary arteries compressing the main bronchi. Br. = bronchus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery, L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b, c) Radiographs show absent pulmonary valve with retained fetal lung fluid from airway obstruction. (b) Anteroposterior radiograph shows an opaque right lung. (c) Anteroposterior radiograph obtained 8 hours later shows hyperinflation. Note the right hilar mass on the dilated right pulmonary artery (arrow). (d) Echocardiogram in the subxiphoid long-axis projection shows a huge main pulmonary artery (MPA). Only dysplastic valve tissue is seen on real-time studies with massive pulmonary insufficiency. (e) Echocardiogram in the subxiphoid ventricular short-axis projection, obtained in the patient in e, shows outflow annulus narrowing (arrow) below the huge main pulmonary artery (MPA). The patient survived after undergoing valve replacement and reduction of the central aneurysm of pulmonary arteries. (Images in d and e courtesy of F. Bierman, MD, New York, NY.)

View larger version (131K): [in a new window]   Figure 10e. (a) Diagram illustrates absence of the pulmonary valve, with huge central pulmonary arteries compressing the main bronchi. Br. = bronchus, L.L.L. = left lower lobe, L.P.A. = left pulmonary artery, L.U.L. = left upper lobe, P.T. = pulmonary trunk, R.L.L. = right lower lobe, R.M.L. = right middle lobe, R.P.A. = right pulmonary artery, R.U.L. = right upper lobe. (Reprinted, with permission, from reference 18.) (b, c) Radiographs show absent pulmonary valve with retained fetal lung fluid from airway obstruction. (b) Anteroposterior radiograph shows an opaque right lung. (c) Anteroposterior radiograph obtained 8 hours later shows hyperinflation. Note the right hilar mass on the dilated right pulmonary artery (arrow). (d) Echocardiogram in the subxiphoid long-axis projection shows a huge main pulmonary artery (MPA). Only dysplastic valve tissue is seen on real-time studies with massive pulmonary insufficiency. (e) Echocardiogram in the subxiphoid ventricular short-axis projection, obtained in the patient in e, shows outflow annulus narrowing (arrow) below the huge main pulmonary artery (MPA). The patient survived after undergoing valve replacement and reduction of the central aneurysm of pulmonary arteries. (Images in d and e courtesy of F. Bierman, MD, New York, NY.)