CTICasesScimitar Syndrome Variant
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Imaging findings
The patient was a 47-year-old female with known bowel malrotation and a twin sister with similar findings. Chest imaging revealed dextrocardia and bronchial atresia of the right upper lobe, with a small right hemithorax and volume loss. CT demonstrated a venous anomaly consistent with a Scimitar variant, despite the absence of a classic Scimitar vein, and an inferior pulmonary vein draining into the IVC.
Key takeaways
Scimitar syndrome can present as a constellation of abnormalities including bronchial atresia, lobar hypoplasia, and anomalous venous return, even when a well-defined Scimitar vein is absent. Always look for associated bronchial and lobar developmental abnormalities in patients with dextrocardia or suspected Scimitar variants.
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