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Systemic arterial supply to the lung

Case contributed by Hemali Kumari Lanka
Diagnosis almost certain

Presentation

Known patient with COPD presented with low volume hemoptysis

Patient Data

Age: 70 years
Gender: Male

The posterior basal segment of the left lower lobe is hyperlucent and shows distal air trapping compared to the rest of the lung. A broncholith is seen within. The overall appearance is suggestive of segmental bronchial atresia.

This segment of the lung is supplied by an aberrant systemic artery arising from the celiac axis at T12 vertebral level. Its diameter is 7 mm. In addition to that, normal pulmonary arterial supply, as well as normal pulmonary venous drainage of the left lower lobe is demonstrated.

The rest of both lungs show paraseptal, panacinar emphysematous changes with few apical subpleural bullae.

No evidence of pulmonary hypertension or cardiomegaly.

Case Discussion

Systemic arterialization of the lung without sequestration (Pryce I sequestration) is the rarest form of bronchopulmonary vascular malformation. If this is severe enough, may lead to the left to left cardiac shunt in some patients. In contrast, pulmonary sequestration is a cystic/solid mass of non-functioning lung tissue that is devoid of communication with the bronchopulmonary tree but has a systemic arterial supply. Another possibility of systemic arterial supply is a hybrid lesion that represents congenital pulmonary airway malformation with pulmonary sequestration.

Treatment options

Symptomatic patients are treated with either surgical ligation of the anomalous vessel with or without resection of the involved lung segment or endovascular treatment.

This patient responded well to medical treatment.

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