J Korean Soc Radiol.  2014 Oct;71(4):191-195. 10.3348/jksr.2014.71.4.191.

A Case of Scimitar Syndrome with H-Type Tracheoesophageal Fistula and Multiple Anomalies: Diagnosis Using Electrocardiography-Gated Chest CT

• Lim KJ
• Kang EJ
• Lee KN
• Jo JH

• KMID: 2098005
• DOI: http://doi.org/10.3348/jksr.2014.71.4.191

Abstract

Scimitar syndrome is a rare, combined abnormality of bronchopulmonary development and pulmonary vascular development characterized by an anomalous pulmonary venous return to the inferior vena cava. Although the scimitar syndrome has been associated with many anomalies, a tracheoesophageal fistula (TEF), especially from H-type, is extremely rare and only a few cases have been reported without detailed descriptions. Herein we report a rare case of scimitar syndrome with H-type TEF and multiple anomalies in a newborn infant, with a special emphasis on the imaging features associated with the radiologic diagnosis using an electrocardiography-gated computed tomography.

MeSH Terms

Diagnosis*
Humans
Infant, Newborn
Multidetector Computed Tomography
Scimitar Syndrome*
Tomography, X-Ray Computed*
Tracheoesophageal Fistula*
Vena Cava, Inferior

Figure

• 

  Fig. 1 A newborn male infant shows tachypnea and chest wall retraction while breathing. A. A chest radiograph demonstrates an almost completely opacified right hemithorax with a mediastinal shift to the right side. Additionally, hemivertebra of T10 (black arrow), left 9-10th rib fusion (arrowhead), and a gaseous distended stomach and intestine are also seen. The tip of the nasogastric tube was seen within the stomach (nasogastric tube; white arrows). B. A minimal intensity projection image showing the hypoplastic right lung with a single right lobar bronchus. C. An axial electrocardiography (ECG)-gated volume chest CT showing a hypoplastic right pulmonary artery measuring 2.27 mm in diameter (main pulmonary artery: 9.5 mm, left pulmonary artery: 4.94 mm). D. A coronal reformatted ECG-gated volume chest CT showing an aberrant systemic artery to the hypoplastic right lung from the abdominal aorta (arrow). E. A coronal reformatted ECG-gated volume chest CT showing the curved anomalous pulmonary veins (scimitar vein, arrow) descending vertically, draining the right lung, and entering into the inferior vena cava. F. An axial ECG-gated volume chest CT showing the cardiac dextroposition and the focal out-pouching wall at the tracheal posterior wall (arrow). G. A three-dimensional tracheobronchial tree image demonstrates the focal retracted posterior wall of the trachea, 1 cm above the level of the carina (arrow). H. Barium esophagography revealed a slit like fistula tract between the trachea and the esophagus (arrow). A final spot view visualized the barium-coated trachea and single right bronchus. The distal esophagus is intact and shows normal passage of contrast media into the stomach, suggesting an H-type tracheoesophageal fistula. Note.-A = abdominal aorta, AA = ascending aorta, C = celiac trunk, DA = descending aorta, E = esophagus, IVC = inferior vena cava, LLB = left lower lobar bronchus, LMB = left main bronchus, LUB = left upper lobar bronchus, LUL = left upper lung, MPA = main pulmonary artery, RB = right lobar bronchus, RPA = right pulmonary artery, SVC = superior vena cava, T = trachea

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