Interactive Transcript
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The first of the congenital lung malformation
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that we will discuss is congenital lobar over inflation.
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It's thought that this occurs as a result
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of early bronchial obstruction, eventually leading
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to a ball valve mechanism
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where in utero fluid can get into the distal airways
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but cannot get out.
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And so distal lung airspace overinflate
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ultimately on imaging postnatally.
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When that fluid is replaced by air, we end up
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with a hyper lucent or hyper expanded lobe.
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Note that this entity used to be called congenital low bar
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emphysema, but now is termed congenital
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lobar over inflation.
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I also mentioned bronch atresia here
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because it's probably on the same spectrum as CLO.
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Ultimately what we can see is a tubular atretic bronchus
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with mucus plugging.
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Sometimes we also see an air-filled bronchae
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or fluid-filled mucus seal.
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And as I mentioned in the introduction,
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these congenital lung malformations probably
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have a common origin.
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So it's not unusual
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that bronchi Atresia is seen in conjunction
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with some other malformations including CPAM
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and VPS, which I'll talk about a little bit later.
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The first case I'll show you has multiple images.
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We will start with the baby on the day that they were born.
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We can see that in the left upper lobe there is
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an opacity that could be mistaken for normal thymus
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because frequently normal thymus does live in that area.
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So it would not be unusual
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that this would be just called normal thymus
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and not distinctly something else.
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However, in this particular patient,
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we'll keep our eye on this area.
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Two days later, a follow-up radiograph shows
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that it's no longer pacified in that region,
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so it's not just thymus.
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In fact, it looks fairly normal.
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However, a couple weeks
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after that, at 18 days of age, we noticed
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that left upper lobe is looking hyper expanded hyper
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lucent compared to other parts of the lung.
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And there's midline shift
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of the mediastinal structures to the right.
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The next day, the same patient underwent ct, which confirms,
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as we can see on this coronal ct, hyper lucency
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hyper expansion of the left upper lobe.
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And this was in fact due to congenital lobar over inflation.
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So it makes sense when we think about it
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because the lung lesion originally is opacified
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because it's not normal lung
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and so it was not replaced with air at the same rate
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as the rest of the lungs.
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But over time, eventually that fluid is replaced by air,
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so it normalizes on the chest radiograph,
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but over time, even further,
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that ball valve mechanism is still occurring now with air.
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And so we get the hyper expanded look
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that we see both on the chest radiograph and on the ct.
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The next case is another patient with multiple images.
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The first image that I'll show you is when they were born
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and we can see this patient is rotated to their right
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and we see sort of vague opacity
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in the left upper lobe region.
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Hard to know what to do with prospectively,
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but something to keep our eye on.
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However, a month later when there were 31 days of age,
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we can see similar to our first case,
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there is hyper expansion, hyper lucency of
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that left upper lobe.
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There's also probably some ectasis of the left lower lobe.
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And on that same day, that patient also underwent a CT scan
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confirming we have hyper expansion of that left upper lobe.
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Notice that this still looks like lung parenchyma,
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but it just looks hyper expanded.
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It still has normal pulmonary vessels
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and bronchials traveling throughout.
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It just looks hyper expanded.
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The final case I'll show is another baby
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with multiple images.
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We'll start with this chest radiograph on the day
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that they were born and we can see again
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increased opacity in the left upper lobe.
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There's normal thymus over here on the right side,
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so it would be difficult
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to distinguish normal thymus from an actual opacity
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if you were reading this prospectively.
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But since we are talking about congenital lung disorders,
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we'll keep our eye on that left upper lobe.
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This patient also actually had a prenatal diagnosis
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of a congenital lung lesion,
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and this was their first postnatal image.
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This patient ended up undergoing surgery
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and it was found that this was a bronchial atresia.
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I'll show you on this CT scan coronal images that
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as we scroll through here,
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I'm gonna change my windows a little bit,
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so the contrast is not quite so bright.
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We can see that there's a soft tissue density
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in the left hilum.
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And then if we change to lung windows, we'll also notice
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that there's hyperinflation of that left upper lobe.
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And if we follow the airway from the distal trachea
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to the left main bronchus,
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we don't see a communicating bronchus going to
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that hyper area area.
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So this turned out to be bronchial.
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Atresia, like I said, is probably on a continuum
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with congenital lobar over inflation,
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but in this case we have that hyper expanded lung.
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We do not see a communicating bronchial since it's a retic.
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And then we do see this soft tissue density,
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which turned out to be a mucus seal, which can be associated
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with bronch regia.
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And then this is a follow-up radiograph when the patient was
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eight years old just to show the hyper aeration
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and slight mass effect on the mediastinum.