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A case of variant scimitar syndrome
Antoine Legras1, Claude Guinet2, Marco Alifano1
1Service de Chirurgie Thoracique, Hôpital Hôtel-Dieu, Paris, France.
Chest
|October 4, 2012
Summary
Scimitar syndrome, a rare congenital lung anomaly, presents unique challenges. This case details a complex variant with anomalous venous return, highlighting advanced imaging for diagnosis.
Area of Science:
- Cardiology
- Thoracic Surgery
- Radiology
Background:
- Scimitar syndrome is a rare congenital pulmonary venolobar syndrome characterized by hypogenetic lung and partial anomalous pulmonary venous return.
- It typically involves anomalous venous drainage to the right atrium or inferior vena cava.
Observation:
- A 25-year-old man presented with a complex and exceptional variant of scimitar syndrome.
- Imaging revealed a hypoplastic right lung, ipsilateral hemidiaphragm agenesis, and a hypogenetic right pulmonary artery.
- A unique feature was a large, sinuous systemic arterial supply and anomalous venous return directly into the left atrium, termed a "meandering" pulmonary vein.
Findings:
- The hyperoxia test indicated no significant intrapulmonary shunt.
- Three-dimensional (3-D) reconstruction via chest CT scan provided precise anatomical detail.
- This variant, with venous return to the left atrium, represents an exceptional presentation of scimitar syndrome.
Implications:
- Complex congenital anomalies like this variant scimitar syndrome pose diagnostic and management difficulties due to their rarity.
- Chest CT with 3-D reconstruction is crucial for accurate, noninvasive diagnosis of these intricate conditions.
- Understanding these variants is vital for appropriate clinical management and surgical planning.
