11 Scimitar Syndrome

Clinical Presentation

Asymptomatic 29-year-old woman

Radiologic Findings

PA (Fig. 11.1) chest X-ray demonstrates a small right lung with ipsilateral mediastinal shift and arcuate tubular opacities that course toward the right cardiophrenic angle. Note that the right bronchus is hyparterial (Fig. 11.1). Contrast-enhanced chest CT (lung window) (Fig. 11.2) demonstrates the small volume of the bilobed right lung. Contrast-enhanced chest CT (mediastinal window) (Figs. 11.3, 11.4) demonstrates paired anomalous pulmonary veins (Fig. 11.3) that drain into the inferior vena cava (Fig. 11.4).

Diagnosis

Scimitar Syndrome

Differential Diagnosis

• Arteriovenous Malformation

• Pulmonary Varix

• Intralobar Sequestration

• Mucoid Impaction/Bronchial Atresia

Fig. 11.1

Fig. 11.2

Fig. 11.3

Fig. 11.4

Discussion

Background

Scimitar syndrome is a complex disorder also known as pulmonary venolobar syndrome and hypogenetic lung syndrome. It characteristically affects the right lung and manifests with a variety of cardiopulmonary anomalies. The term scimitar

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