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Online Mendelian Inheritance in Man
2007-10-07-05 Left lung sequestration, extralobar, intrathoracic © Manson
Left lung sequestration, extralobar, intrathoracic
This is a 28-year-old woman (G3P2) with unremarkable medical history except of spontaneously regressed fetal choroid plexus cyst during her second pregnancy.
The first trimester scan and the triple test screen were normal. At 22nd week the following findings were seen at ultrasound:
Images 1 and 2
. 2D gray scale ultrasound images at 22nd week of pregnancy.
- transverse scan through the fetal chest showing an echogenic mass of the left hemithorax with dextroposition of the heart.
- transverse scan through the fetal chest showing
the compressed right lung (RL) and dextroposition of the heart.
Images 3 and 4
. 2D gray-scale ultrasound images at 22nd week of pregnancy.
ree vessel view showing the posterior lung sequestration and the normal appearance of the superior lobe of the left lung.
ypical appearance of triangle shaped echogenic lung sequestration.
Images 5 and 6
. 2D color Doppler images at 22nd week of pregnancy.
oronal plane showing an aberrant artery originating from the descending aorta
- another coronal plane showing the renal arteries and the aberrant arterial vascularization of the sequestrated lobe of the lung
Images 7 and 8
. 2D color Doppler images at 22nd week of pregnancy. Transverse planes showing the arterial vascularization of the sequestrated lung lobe and also the venous blood return toward the vena cava superior (the visualization of the venous blood return is usually difficult in the case of lung sequestration).
: 32 weeks; the axial scan shows the normalization of the heart displacement and decrease of the hyperechogenicity of the sequestered part of the lungs.
The patient gave birth to a healthy neonate (3690 g) at 39 weeks. A computed tomography was done at 6 weeks of age (images not available) and the presence of the left inferior lobe sequestration without any features of an associated cystic adenomatoid malformation was found. The sequestered part of the lungs wasn"t aerated and was fed by a single arterial vessel.
The embolization of the malformed part of the lungs was proposed and preferred before the
surgical lobectomy. The procedure will be done at six months of life.
: Postnatal chest X-ray shows a discreet opacity of the inferior left lung lobe (*).
A computed tomography was done at 6 weeks of age:
: An axial section of the thorax. The lung sequestration is marked by the (*). The two structures on both side of the heart are the domes of the diaphragm.
Images 13, 14
: The images show parasagittal sections of the thorax. Note the "triangular shape" of the lung sequestration (*) and the normal aspect of the diaphragm (arrows).
: The image shows a coronal section of the thorax.
Images 16, 17
: These two scans show the vascularization of the lung sequestration with a single aberrant artery originate from the descending aorta.
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