Case 6860
Pulmonary Sequestration
Author(s)
Papadopoulos M., Voultsinou D., Koutsokosta E., Palladas P.
Patient
female, 35 year(s)
Clinical History
A 40 year old female patient with left thoracic pain, after curettage for an unaccepted gestation, underwent a CT examination.
Imaging Findings
A 40 year old woman with left thoracic pain, after curettage for an unaccepted gestation, underwent a chest radiograph and CT examination. No smoking history and no other relevant medical background.
A retrocardiac lesion depicted at the chest radiograph and a further CT examination was conducted. No other symptoms were present and the laboratory exams were within the normal limits.
At CT examination an air-fluid level was present within this consolidation (Figure 1), with compensatory emphysema (Figure 2). Also a dilated azygos vein (Figure 3) was observed.
Further imaging investigation with chest CT Multiplanar Imaging Reconstruction, confirmed the presence anomalous arterial supply of the left lower lobe mass (Figure 4). Findings were compatible with pulmonary sequestration, in accordance with the typical morphology and location.
A retrocardiac lesion depicted at the chest radiograph and a further CT examination was conducted. No other symptoms were present and the laboratory exams were within the normal limits.
At CT examination an air-fluid level was present within this consolidation (Figure 1), with compensatory emphysema (Figure 2). Also a dilated azygos vein (Figure 3) was observed.
Further imaging investigation with chest CT Multiplanar Imaging Reconstruction, confirmed the presence anomalous arterial supply of the left lower lobe mass (Figure 4). Findings were compatible with pulmonary sequestration, in accordance with the typical morphology and location.
Discussion
Pulmonary sequestration (PS) is a mass of non-functioning pulmonary tissue, disconnected or abnormally communicating to the tracheobronchial tree with a normal or anomalous arterial supply and venous drainage. PS occurs in two forms of which the more frequent type is intralobar (ILS, 75-85%), located within normal pulmonary parenchyma, pleura and pulmonary venous drainage. The extralobar form is less frequent (ELS, 15-25%) which usually drains via the systemic venous system. The most frequent location of PS is in the lower left lobe. ELS may also be subdiaphragmatic or even intraperitoneal.
Especially the intralobar form remains often clinically latent. The major clinical manifestations are recurrent pulmonary infections, associated with fever, cough and hemoptysis. In rare cases heart failure due to the massive blood supply of the anomalous artery and hemothorax due to hemorrhage have been described. It may also be discovered incidentally. ELS have earlier manifestations, usually during the neonatal period such as respiratory distress and recurrent infections. During the fetal period pulmonary sequestration can be complicated with fetal hydrops and hydrothorax.
The plain radiograph typically shows a consolidation usually in the left lower lobe. The most usual presentation is a single homogeneous opacity but recurrent infections may lead to the formation of cystic areas within it and focal bronchiectatic changes.
Identifying the anomalous arterial blood supply of a suspected pulmonary mass is the cardinal aspect of the radiographic diagnosis. Angiography is consequently considered the most suitable method to make the definitive diagnosis. The classic findings are the abnormal blood supply, directly from the lower thoracic or upper abdominal aorta in most cases. Anomalous venous drainage to the systemic venous system can also be identified with this method.
CT shows the abnormal lung parenchyma and aberrant vessels supplying and draining the sequestred tissue. Within the parenchymal abnormalities cavities or cystic elements have been described, ocassionally with air-fluid levels. In some cases the alveoli are hyperinflated, forming areas of low attenuation, indicating air trapping in the sequestred mass. Calcifications can be also identified better with CT rather than with any other imaging method. Three dimensional reconstruction can depict the location and the relationship of these pathological elements.
MR imaging may have advantages over CT in identifying blood vessels. Nevertheless, it cannot accuratelly evaluate the lung parenchyma with the disadvantage of considerably slower imaging.
Sonography shows a mass homogeneously echogenic but may have cystic or complex character. Aberrant arterial and venous drainage can also be seen. The diagnosis can be made in utero or in infants. In these cases it can show the lung mass in fetus, particullary if hydramnios is present. Duplex Doppler is also useful in identifying the anomalous vessels.
Differential diagnosis includes mainly bronchiectasis, lung abscess, arterio-venous shunt, Bochdalek hernia, pulmonary infarction, cystic adenomatoid malformation, bronchogenic cyst, congenital lobar emphysema, bronchial atresia, Swyer-James syndrome, pneumonia, systemic arterial supply to normal lung and mediastinal tumors such as teratomas and neuroblastomas.
Treatment strategy depends on the form and presence of symptoms.
Especially the intralobar form remains often clinically latent. The major clinical manifestations are recurrent pulmonary infections, associated with fever, cough and hemoptysis. In rare cases heart failure due to the massive blood supply of the anomalous artery and hemothorax due to hemorrhage have been described. It may also be discovered incidentally. ELS have earlier manifestations, usually during the neonatal period such as respiratory distress and recurrent infections. During the fetal period pulmonary sequestration can be complicated with fetal hydrops and hydrothorax.
The plain radiograph typically shows a consolidation usually in the left lower lobe. The most usual presentation is a single homogeneous opacity but recurrent infections may lead to the formation of cystic areas within it and focal bronchiectatic changes.
Identifying the anomalous arterial blood supply of a suspected pulmonary mass is the cardinal aspect of the radiographic diagnosis. Angiography is consequently considered the most suitable method to make the definitive diagnosis. The classic findings are the abnormal blood supply, directly from the lower thoracic or upper abdominal aorta in most cases. Anomalous venous drainage to the systemic venous system can also be identified with this method.
CT shows the abnormal lung parenchyma and aberrant vessels supplying and draining the sequestred tissue. Within the parenchymal abnormalities cavities or cystic elements have been described, ocassionally with air-fluid levels. In some cases the alveoli are hyperinflated, forming areas of low attenuation, indicating air trapping in the sequestred mass. Calcifications can be also identified better with CT rather than with any other imaging method. Three dimensional reconstruction can depict the location and the relationship of these pathological elements.
MR imaging may have advantages over CT in identifying blood vessels. Nevertheless, it cannot accuratelly evaluate the lung parenchyma with the disadvantage of considerably slower imaging.
Sonography shows a mass homogeneously echogenic but may have cystic or complex character. Aberrant arterial and venous drainage can also be seen. The diagnosis can be made in utero or in infants. In these cases it can show the lung mass in fetus, particullary if hydramnios is present. Duplex Doppler is also useful in identifying the anomalous vessels.
Differential diagnosis includes mainly bronchiectasis, lung abscess, arterio-venous shunt, Bochdalek hernia, pulmonary infarction, cystic adenomatoid malformation, bronchogenic cyst, congenital lobar emphysema, bronchial atresia, Swyer-James syndrome, pneumonia, systemic arterial supply to normal lung and mediastinal tumors such as teratomas and neuroblastomas.
Treatment strategy depends on the form and presence of symptoms.
Final Diagnosis
Pulmonary Sequestration
MeSH
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Congenital, Hereditary, and Neonatal Diseases and Abnormalities
[C16]
Diseases existing at birth and often before birth, or that develop during the first month of life (INFANT, NEWBORN, DISEASES), regardless of causation. Of these diseases, those characterized by structural deformities are termed ABNORMALITIES. -
Bronchopulmonary Sequestration
[C16.131.740.214]
A developmental anomaly in which a mass of nonfunctioning lung tissue lacks normal connection with the tracheobroncheal tree and receives an anomalous blood supply originating from the descending thoracic or abdominal aorta. The mass may be extralobar, i.e., completely separated from normally connected lung, or intralobar, i.e., partly surrounded by normal lung.
References
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[1]Michalakeas C, Tsekes G, Demopoulos G, Pappas J, Grammelis V, Tserpe P, Lazanas MC (2006) Intralobar bronchopulmonary sequestration in a young adult. Pneumon 19(3):252-257 .
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[5]
Citation
Papadopoulos M., Voultsinou D., Koutsokosta E., Palladas P. (2008, Jul 28).
Pulmonary Sequestration, {Online}.
URL: http://www.eurorad.org/case.php?id=6860
