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Congenital Anomalies of the Upper Gastrointestinal Tract¹

¹ From the Servicio de Radiodiagnóstico, Hospital Infantil "La Paz," Paseo de la Castellana 261, 28046 Madrid, Spain. Presented as a scientific exhibit at the 1997 RSNA scientific assembly. Received March 16, 1998; revision requested April 29 and final revision received September 15; accepted September 16. Address reprint requests to T.B.

View larger version (10K): [in a new window]   Figure 1.  Drawings illustrate a classification scheme for esophageal atresia and tracheoesophageal fistula. A = atresia without fistula, B = atresia with upper fistula, C = atresia with lower fistula, D = atresia with both lower and upper fistulas, E = tracheoesophageal fistula with no atresia. e = esophagus, s = stomach. Types A and B are similar radiographically, as are types C and D. Prevalence of the different types of atresia in 333 cases seen at the authors' institution over the past 18 years is as follows: A, 10%; B, 0.9%; C, 53%; D, 2.1%; and E, 10%. (Reprinted, with permission, from reference 5.)

View larger version (118K): [in a new window]   Figure 2a.  Esophageal atresia with fistula (type D). (a) Frontal radiograph shows air within the distended upper esophageal pouch (arrows). The presence of gas in the bowel indicates fistulous communication between the lower esophageal segment and the trachea. (b) Lateral radiograph more clearly demonstrates the distended upper esophageal pouch with resulting pressure deformity of the trachea. An upper fistula was also found at surgery.

View larger version (99K): [in a new window]   Figure 2b.  Esophageal atresia with fistula (type D). (a) Frontal radiograph shows air within the distended upper esophageal pouch (arrows). The presence of gas in the bowel indicates fistulous communication between the lower esophageal segment and the trachea. (b) Lateral radiograph more clearly demonstrates the distended upper esophageal pouch with resulting pressure deformity of the trachea. An upper fistula was also found at surgery.

View larger version (101K): [in a new window]   Figures 3-5.  (3) Esophageal atresia without fistula (type A). Frontal radiograph shows a radiopaque tube curling into the upper esophageal pouch (arrows). No air is present in the gastrointestinal tract; therefore, there is no distal fistula. (4) Esophageal atresia with lower fistula (type C). On a frontal radiograph, a catheter is seen coiled within the upper esophageal pouch (arrows). Air is also present in the gastrointestinal tract, indicating communication between the lower esophageal segment and the respiratory tree. The bowel dilatation is the result of an imperforate anus associated with esophageal atresia. (5) Tracheoesophageal fistula without atresia (type E). Esophagogram shows a fistula (arrow) arising from the anterior portion of the esophagus (e) and passing cephalad to the posterior portion of the trachea (t).

View larger version (107K): [in a new window]   Figures 3-5.  (3) Esophageal atresia without fistula (type A). Frontal radiograph shows a radiopaque tube curling into the upper esophageal pouch (arrows). No air is present in the gastrointestinal tract; therefore, there is no distal fistula. (4) Esophageal atresia with lower fistula (type C). On a frontal radiograph, a catheter is seen coiled within the upper esophageal pouch (arrows). Air is also present in the gastrointestinal tract, indicating communication between the lower esophageal segment and the respiratory tree. The bowel dilatation is the result of an imperforate anus associated with esophageal atresia. (5) Tracheoesophageal fistula without atresia (type E). Esophagogram shows a fistula (arrow) arising from the anterior portion of the esophagus (e) and passing cephalad to the posterior portion of the trachea (t).

View larger version (87K): [in a new window]   Figures 3-5.  (3) Esophageal atresia without fistula (type A). Frontal radiograph shows a radiopaque tube curling into the upper esophageal pouch (arrows). No air is present in the gastrointestinal tract; therefore, there is no distal fistula. (4) Esophageal atresia with lower fistula (type C). On a frontal radiograph, a catheter is seen coiled within the upper esophageal pouch (arrows). Air is also present in the gastrointestinal tract, indicating communication between the lower esophageal segment and the respiratory tree. The bowel dilatation is the result of an imperforate anus associated with esophageal atresia. (5) Tracheoesophageal fistula without atresia (type E). Esophagogram shows a fistula (arrow) arising from the anterior portion of the esophagus (e) and passing cephalad to the posterior portion of the trachea (t).

View larger version (77K): [in a new window]   Figures 6, 7.  (6) Congenital stenosis in a 9-month-old male infant with persistent regurgitation. Esophagogram demonstrates a small, narrowed segment in the middle third of the esophagus (arrows). (7) Congenital esophageal webs. (a) Esophagogram shows a concentric filling defect (arrows). At endoscopy, a thin diaphragm with a central opening was noted. (b) Esophagogram shows a tracheoesophageal fistula (arrow) located at the same level as a congenital stricture (arrowhead).

View larger version (78K): [in a new window]   Figures 6, 7.  (6) Congenital stenosis in a 9-month-old male infant with persistent regurgitation. Esophagogram demonstrates a small, narrowed segment in the middle third of the esophagus (arrows). (7) Congenital esophageal webs. (a) Esophagogram shows a concentric filling defect (arrows). At endoscopy, a thin diaphragm with a central opening was noted. (b) Esophagogram shows a tracheoesophageal fistula (arrow) located at the same level as a congenital stricture (arrowhead).

View larger version (78K): [in a new window]   Figures 6, 7.  (6) Congenital stenosis in a 9-month-old male infant with persistent regurgitation. Esophagogram demonstrates a small, narrowed segment in the middle third of the esophagus (arrows). (7) Congenital esophageal webs. (a) Esophagogram shows a concentric filling defect (arrows). At endoscopy, a thin diaphragm with a central opening was noted. (b) Esophagogram shows a tracheoesophageal fistula (arrow) located at the same level as a congenital stricture (arrowhead).

View larger version (86K): [in a new window]   Figures 8, 9.  (8) Complete esophageal duplication in a 27-year-old man with a history of dysphagia and choking. Esophagogram shows the duplicated esophagus (d) located posterior to the normal esophagus. (9) Duplication cyst in a 2-year-old girl with recurrent episodes of vomiting. (a) Esophagogram shows extrinsic compression of the left wall of the esophagus (arrows). (b) On a coronal T1-weighted MR image, a sharply defined low-signal-intensity mass (arrows) is seen adjacent to the left side of the esophagus. The mass had very high signal intensity on a T2-weighted MR image (not shown).

View larger version (89K): [in a new window]   Figures 8, 9.  (8) Complete esophageal duplication in a 27-year-old man with a history of dysphagia and choking. Esophagogram shows the duplicated esophagus (d) located posterior to the normal esophagus. (9) Duplication cyst in a 2-year-old girl with recurrent episodes of vomiting. (a) Esophagogram shows extrinsic compression of the left wall of the esophagus (arrows). (b) On a coronal T1-weighted MR image, a sharply defined low-signal-intensity mass (arrows) is seen adjacent to the left side of the esophagus. The mass had very high signal intensity on a T2-weighted MR image (not shown).

View larger version (112K): [in a new window]   Figures 8, 9.  (8) Complete esophageal duplication in a 27-year-old man with a history of dysphagia and choking. Esophagogram shows the duplicated esophagus (d) located posterior to the normal esophagus. (9) Duplication cyst in a 2-year-old girl with recurrent episodes of vomiting. (a) Esophagogram shows extrinsic compression of the left wall of the esophagus (arrows). (b) On a coronal T1-weighted MR image, a sharply defined low-signal-intensity mass (arrows) is seen adjacent to the left side of the esophagus. The mass had very high signal intensity on a T2-weighted MR image (not shown).

View larger version (104K): [in a new window]   Figure 10a.  Double aortic arch. Anteroposterior (a) and lateral (b) barium esophagograms show posterior indentation of the upper esophagus bilaterally (arrows).

View larger version (109K): [in a new window]   Figure 10b.  Double aortic arch. Anteroposterior (a) and lateral (b) barium esophagograms show posterior indentation of the upper esophagus bilaterally (arrows).

View larger version (93K): [in a new window]   Figure 11a.  Aberrant right subclavian artery. (a) Anteroposterior esophagogram shows an oblique filling defect passing cephalad from left to right (pathognomonic indentation) (arrows). (b) Lateral esophagogram shows compression of the posterior wall of the esophagus (arrow).

View larger version (108K): [in a new window]   Figure 11b.  Aberrant right subclavian artery. (a) Anteroposterior esophagogram shows an oblique filling defect passing cephalad from left to right (pathognomonic indentation) (arrows). (b) Lateral esophagogram shows compression of the posterior wall of the esophagus (arrow).

View larger version (126K): [in a new window]   Figure 12.  Pyloric atresia in a male neonate. Frontal radiograph shows distention of the stomach and absence of gas in the small bowel (single bubble appearance).

View larger version (136K): [in a new window]   Figure 13.  Congenital antral stenosis in a 1-month-old male infant with vomiting attacks from birth. Frontal radiograph shows distention of the stomach and decreased gas in the small bowel. Findings at pylorus US were normal.

View larger version (148K): [in a new window]   Figure 14.  Incomplete antral web. Radiograph from a barium study (anterior oblique view) shows a concentric radiolucent band (arrows) producing discrete antral lumen reduction. Arrowhead indicates pylorus.

View larger version (115K): [in a new window]   Figure 15.  Ectopic pancreas in the gastric antrum. Image from an upper gastrointestinal series shows a rounded nodular defect in the gastric antrum with central umbilication identified by a fleck of barium (arrow). The diagnosis was confirmed at surgery.

View larger version (139K): [in a new window]   Figure 16a.  Gastric duplication cyst. (a) Image from a contrast-enhanced upper gastrointestinal series demonstrates an intraabdominal mass displacing the stomach and bowel to the right. (b) CT scan through the gastric body shows a near-water-attenuation spheric mass (arrows) in close contact with the greater curvature of the stomach. There is no communication between the cystic lumen and the true stomach lumen. This is the least common of the enteric duplications.

View larger version (169K): [in a new window]   Figure 16b.  Gastric duplication cyst. (a) Image from a contrast-enhanced upper gastrointestinal series demonstrates an intraabdominal mass displacing the stomach and bowel to the right. (b) CT scan through the gastric body shows a near-water-attenuation spheric mass (arrows) in close contact with the greater curvature of the stomach. There is no communication between the cystic lumen and the true stomach lumen. This is the least common of the enteric duplications.

View larger version (135K): [in a new window]   Figure 17a.  Complete pyloric duplication in a 32-year-old man with epigastric pain. (a) Image from an upper gastrointestinal series demonstrates principal (p) and duplicated (d) pyloric canals between the stomach and duodenum. Ulceration (arrow) caused the patient's symptoms. (b) Endoscopic images of the gastric outlet show two pyloric orifices.

View larger version (63K): [in a new window]   Figure 17b.  Complete pyloric duplication in a 32-year-old man with epigastric pain. (a) Image from an upper gastrointestinal series demonstrates principal (p) and duplicated (d) pyloric canals between the stomach and duodenum. Ulceration (arrow) caused the patient's symptoms. (b) Endoscopic images of the gastric outlet show two pyloric orifices.

View larger version (126K): [in a new window]   Figures 18, 19.  (18) Duodenal atresia in an 8-hour-old female neonate who presented with bilious vomiting. Abdominal scout radiograph shows a markedly distended stomach and duodenum with no gas in the rest of the intestinal tract (double bubble sign). (19) Annular pancreas in a male neonate. Lateral radiograph shows complete duodenal obstruction with the double bubble sign, a finding that is indistinguishable from duodenal atresia.

View larger version (110K): [in a new window]   Figures 18, 19.  (18) Duodenal atresia in an 8-hour-old female neonate who presented with bilious vomiting. Abdominal scout radiograph shows a markedly distended stomach and duodenum with no gas in the rest of the intestinal tract (double bubble sign). (19) Annular pancreas in a male neonate. Lateral radiograph shows complete duodenal obstruction with the double bubble sign, a finding that is indistinguishable from duodenal atresia.

View larger version (140K): [in a new window]   Figure 20.  Duodenal web in a 1-year-old boy with a history of intermittent vomiting. Radiograph from a barium study shows dilatation of the stomach and proximal duodenum. An incomplete diaphragm was found at surgery.

View larger version (117K): [in a new window]   Figure 21.  Nonobstructing duodenal diaphragm in a 32-year-old man. Radiograph from a barium study shows a smooth, transverse filling defect in the second portion of the duodenum (arrows), allowing free passage of contrast material to the small bowel.

View larger version (116K): [in a new window]   Figure 22a.  Duodenal duplication cyst. (a) Radiograph from a barium study shows a large filling defect on the medial border of the duodenum displacing and indenting the lumen. (b) Sonogram demonstrates a large, sonolucent cyst with a characteristic echogenic inner mucosal layer and an anechoic underlying muscular layer. Q = cyst, V = gallbladder.

View larger version (181K): [in a new window]   Figure 22b.  Duodenal duplication cyst. (a) Radiograph from a barium study shows a large filling defect on the medial border of the duodenum displacing and indenting the lumen. (b) Sonogram demonstrates a large, sonolucent cyst with a characteristic echogenic inner mucosal layer and an anechoic underlying muscular layer. Q = cyst, V = gallbladder.