Anamolous pulmonary artery

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Anomalous systemic arterial supply to the lungs has been extensively described in association with other congenital abnormalities such as bronchopulmonary sequestration and hypogenetic lung syndrome (scimitar syndrome). In 1946, Pryce introduced the term ‘sequestration’ to describe congenital abnormalities characterised by an anomalous systemic arterial supply to the lung and atresia or hypoplasia of the pulmonary artery. Sequestration was defined by Pryce as a “disconnected (dislocated, ectopic) bronchopulmonary mass or cyst with an anomalous systemic blood supply”.
Since Pryce’s early description of sequestration, a wide spectrum of bronchopulmonary- vascular malformations have been recognised, many of which do not fulfil the original definition. Our case differs from traditional descriptions of sequestration, in that a portion of normal lung receives an anomalous systemic blood supply. Sade et al. proposed the concept of a sequestration spectrum, in order to encompass and describe the various combinations of abnormal bronchial connection, arterial supply and venous drainage. As a similar spectrum concept, Clements and Warner introduced a simple descriptive anatomical approach to such complex bronchopulmonary-vascular malformations named ‘pulmonary malinosculation spectrum’.
Systemic arterialization of the lung without pulmonary sequestration is the rarest form of anomalous systemic arterial supply to the lung . This condition is characterised by an aberrant systemic arterial branch, most commonly arising from the thoracic portion of the descending aorta or the abdominal aorta, supplying an area of lung parenchyma with normal bronchopulmonary system. The left lower lobe is most often involved, and there is commonly an atr...

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... Various treatment modalities have been described, including lobectomy or segmentectomy, systemic artery to pulmonary artery anastomosis and endovascular treatment. Differentiation between this condition from classic sequestration has important treatment implications as it may allow non-operative management (angiographic embolization) of the aberrant systemic artery.

TEACHING POINT
Systemic arterialization of the lung without sequestration is a rare condition often diagnosed following investigation of an incidental cardiac murmur or based on abnormal chest X ray or CT of the thorax, as most patients are asymptomatic. Thoracic CT is the most useful diagnostic test as it demonstrates both the bronchial and vascular anatomy of the lung while CT angiography can clearly depict the origin of the aberrant systemic artery, avoiding invasive techniques for the diagnosis.

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