Abstract
Intralobular pulmonary sequestration may present in adulthood as massive haemoptysis http://ow.ly/EQNsf
A 50-year-old female with no prior respiratory disease or symptoms presented with massive haemoptysis and respiratory failure. Multidetector computed tomographic angiography demonstrated an aberrant artery supplying a lobulated mass occupying two-thirds of the right chest (fig. 1a and b). Aortography confirmed a large aberrant systemic artery originating from the supra-diaphragmatic aorta (fig. 1c), with drainage into the pulmonary veins (fig. 1d). Emergent transcatheter arterial embolisation of the feeding artery was performed (fig. 1e). 2 weeks later, after recovering from respiratory failure, lobectomy was performed because of persistent bleeding and mild fever despite antibiotics. A massive haemorrhage within the intralobar sequestra with a thrombosed feeding artery as a result of the intravascular coil was confirmed (fig. 1f).
Pulmonary sequestration is a rare sporadic developmental abnormality in which a region of the lung parenchyma has abnormal connection with the airways and is supplied by an aberrant artery arising from the aorta or one of its branches. Most sequestration are intralobar (75–85%), with incomplete communication with the adjacent lung and venous drainage via the pulmonary veins. They are generally observed in the medial or posterobasal segments of the left (60%) and right (40%) lower lobes [1]. It presents most commonly in childhood with recurrent infections, although massive haemoptysis in young adulthood has been reported [2, 3]. Conversely, extralobar sequestrations are characterised by the absence of connection with the airway, venous drainage via systemic veins, left lower lobe predominance, male predilection and frequent concomitant congenital abnormalities. They generally present in the neonatal period as left-to-right cardiac shunting, respiratory distress and cyanosis (large sequestration), or are found as an incidental finding later in life.
Footnotes
Conflict of interest: None declared.
Provenance: Submitted article, peer reviewed.
- Received July 23, 2014.
- Accepted August 19, 2014.
- Copyright ©ERS 2015.
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