CTICasesScimitar Syndrome Variant with Azygos Drainage and Systemic Arterial Supply
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Imaging findings
The chest radiograph shows a dramatic reduction in right-sided lung volume. CT demonstrates a right pulmonary artery. However, the right superior and inferior pulmonary veins do not connect to the left atrium. Instead, they sweep back in the aortoesophageal recess, join a tiny azygos vein, and complete the azygos arch, draining into the superior vena cava. Airway anomalies include a hyperarterial bronchus on the right, a bizarre branching pattern, a small rudimentary segment/lobe, and only a couple of segments in the lower lobe. A small systemic arterial branch, likely an inferior phrenic branch, provides additional supply to the right lung. There is also a hemi-vertebral body and scoliosis.
Key takeaways
This case represents a complex congenital anomaly, a variant of Scimitar syndrome, fitting a pattern of lung disorganization. The atypical right pulmonary venous drainage into the azygos/SVC system (often termed 'Scimitar without the Scimitar' when the classic curvilinear vein is absent), combined with bronchial and vertebral anomalies and systemic arterial supply, highlights the multifaceted nature of these developmental disorders. Recognizing the presence of multiple abnormalities increases the likelihood of finding even more complex variants.
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