Scimitar Syndrome With Esophageal Varices
Magnetic Resonance Angiography Detects Anomalous Pulmonary Venous Return
A 17-year-old man was admitted to the emergency department complaining of a mouth full of blood that he had spit out twice since the previous day. A chest radiogram revealed a small right lung and dextroposition of the cardiomediastinal structure (Figure 1). After the man’s condition was stabilized, he underwent an MRI to evaluate a suspected congenital anomaly. After a contrast agent was injected intravenously, 3-dimensional magnetic resonance angiography (MRA) was performed with a 1.5-T scanner while the man held his breath for 10 seconds. Oblique-coronal reconstruction revealed a small, hypoplastic right pulmonary artery. Also noted was a large anomalous pulmonary venous return toward the inferior vena cava just at the level of the diaphragm (Figure 2A). Taken together, these findings indicated scimitar syndrome. Interestingly, the MRA showed that the anomalous pulmonary vein did not drain into the inferior vena cava. Instead, it serpiginously communicated to the azygos esophageal venous complex, causing severe esophageal varices (Figure 2B). Esophagoscopy confirmed the severely engorged varices with cherry red–colored mucosa (Figure 3). Presurgical catheter angiography confirmed the diagnosis of scimitar syndrome with anomalous venous return into esophageal varices, and a blind end to the inferior vena cava (Figure 4). This unusual pathway was identical to what was depicted by MRA. After surgical reimplantation of the pulmonary vein into the left atrium, the esophageal varices diminished, and the patient no longer experiences bleeding symptoms.
This study was completed at Kaohsiung Veterans General Hospital under grant VGHKS93-80.