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Case: VACTERL With TEF

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So this is a, uh, newborn infant with concern for,

0:06

um, imperforate anus and so on this, uh, chest radiograph.

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The important finding is

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that this enteric tube is not extending into the stomach

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where we think it should belong.

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Um, so, uh, we have concern

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for esophageal atresia in this infant.

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Another important finding once,

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once you're considering esophageal atresia, is,

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is there a trache, esophageal fistula versus not?

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And we can say there is concern for fistula

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because we have distal bowel gas in this stomach

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and in this bowel.

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Now, this, this patient, I would also say there's concern

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for hetero ataxia or, um, aus abnormality

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because the stomach looks like it's right sided to me

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as opposed to left sided.

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Um, last but not least, we have a right sided cardiac, um,

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apex as opposed to a left-sided cardiac apex.

0:58

So first we're gonna call our text

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and make sure that this is not mislabeled right to left,

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but, um, if, assuming that it is labeled correctly, this is

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concerning for hetero ataxia.

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There's one important other finding to look

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for when you have a patient with esophageal atresia, um,

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with or without a trache esophageal fistula.

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And that is vertebral body anomalies.

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So when we look super carefully at these

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superior vertebral bodies,

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we definitely have a vertebral anomaly.

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There is a funny kind of partial fusion of, of these, um,

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upper lower cervical

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and upper thoracic vertebral bodies here at

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the C seven T one level.

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And then we have a hemi vertebra at the leftward aspect of,

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uh, probably T two in this infant.

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So now we're dealing with the ral, uh, spectrum.

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So we found the vertebral body anomalies.

1:49

This patient on physical exam has anorectal malformation

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with concern for imperfect anus.

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We have a right-sided cardiac apex, so this patient needs

1:57

to undergo, uh, car echocardio echocardiography.

2:02

Um, so that's the c the renal anomalies.

2:05

This we will recommend a renal ultrasound.

2:07

And then with the vertebral anomalies,

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we'll also recommend a spine ultrasound

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and this patient, um, we don't see all

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of the upper extremity limbs,

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but hopefully on exam they would be able

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to see if there's a limb abnormality, um, associated

2:19

with this patient with vl.

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Um, on exam. So the first uh, uh,

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screening ultrasound that, uh, was presented to us

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with this patient for follow-up was the spine ultrasound.

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So we are starting in the, uh,

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the prone position in the transverse plane.

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Um, and, uh, we know that we should be able

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to see central spinal cord with central penal canal,

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but instead we see this funky looking cystic structure in

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the middle of this, uh, uh, spinal canal.

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Um, as we are going more superiorly,

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the cord does look more normal.

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Um, this patient is prone in position,

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but this cord is not located

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In the, the anterior aspect,

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anterior one third of the spinal canal.

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It looks like it's more located posteriorly.

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Um, hopefully we will see some, um,

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some additional sagittal images later.

3:09

These lungs look okay, what I see

3:12

of adrenal glands looks okay here is probably left adrenal

3:15

gland and right adrenal gland over here.

3:18

Hopefully next we'll have some sagittal images to look at.

3:21

Yeah, so, um, this, this, uh, uh, sacrum looks like it kind

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of abruptly ends here.

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Um, we do see something in the rectum.

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So, um, uh, this patient has in peripheral anus,

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but we see rectum anterior to the sacrum here.

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And then we have some definite abnormalities

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of the lower aspect of the conus meis.

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So a filer cyst should be, um, at the tip

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of the conus meis really not involving the

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conus meis itself.

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So that's, um, the first thing that catches my eye.

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The second abnormality is no matter

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how you're gonna label this vertebral bodies,

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this is too low.

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So if we call this the S two sacral vertebral body

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where the thecal sac ends, this would be S one,

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L five, L four.

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So this, uh, this spinal cord

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and conus MedU go at least

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to the L four vertebral body at a minimum the inferior

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aspect of the L three vertebral body.

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It does not have a normal, uh, triangular shaped,

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uh, conus metis.

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So this is gonna be concerning for tethered cord.

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We'll recommend an MRI of the spine when this patient is,

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uh, well enough to undergo that examination.

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The last, um, ultrasound we have for screening

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for additional anomalies,

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and this patient is a renal ultrasound,

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and so here we're in the sagittal plane, um,

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and we can see part of right kidney

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and it looks like it's sitting right on top

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of a vertebral body.

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That's what this genic sort of rounded structure is here.

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The tech has labeled the, the rightward aspect

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of this right-sided kidney.

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It looks pretty normal.

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We don't see any hydro necrosis or, um, anything.

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We have good profusion with power doppler imaging.

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Um, we'll keep looking at this kidney

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to see if we see anything abnormal Besides,

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it looks like it's a little bit too low in the location, um,

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it looks like as we're going.

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Um, yeah, so here's here we're seeing that the,

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the renal FoST is empty where it's supposed to be.

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We do have a normal abdominal aorta, um, up top

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and we, um, see some part of IBC,

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but this looks more like Aus continuation to me.

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So typically our int hepatic IVC will be int hepatic,

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it'll go into the liver over here.

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So this is probably an interrupted IVC

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with ASUS continuation.

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So this was the patient we were concerned about hetero

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ataxia and this is supporting that,

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that concern that we had earlier.

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Unfortunately, our urinary bladder is completely collapsed.

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Um, so we can't see too much about the urinary

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bladder on this examination.

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Here is our left kidney, which looks, um,

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a little bit more normal in

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Morphology compared to that right kidney,

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although it looks, looks pretty,

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pretty globular in configuration.

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Let's go to Aase to see if we can figure out, um,

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what else is going on with this infant.

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Why do these kidneys kind

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of look like they're abnormal in morphology?

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So that's the urinary bladder.

6:13

I think that this, this might be a horseshoe kidney.

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So, um, if you look at this, uh, right kidney,

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we don't really see the, uh, like the end of it very well.

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Let's see if we can find,

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and here's the, here's the left side, left side kidney.

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So I think we have a low lying.

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Yeah, look at where the sonographer has placed the marker

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of where she's imaging.

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So this is, this is gonna be a horseshoe kidney where both

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of the, um, renal motis are located in the pelvis.

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And, um, there's gonna be a, a tissue connecting,

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connecting the two of them at midline.

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Um, we don't have that nice, um, image showing that, uh, uh,

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isus of tissue between the two renal moty.

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Um, but this is, this is the renal anomaly that goes

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with the ral syndrome in this infant, um, with

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esophageal atresia, tracheal esophageal

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fistula, vertebral anomalies.

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And then we also have the spine abnormalities of the infant.

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Um, here we go. So here's the trans,

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this is the image I was looking for y'all.

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So the, here we're in the transverse plane

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of the right kidney and check out this, um, isus

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of renal tissue extending across the midline.

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So this is the inferior aspect of the left kidney

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as we come superiorly, we have this isus

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of tissue connecting the left kidney and right kidney.

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Um, and then here is the right kidney.

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So this is a, uh, uh,

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a horseshoe pelvic kidney in this infant with bacterial.

Report

Text

Faculty

Judy H. Squires, MD

Associate Professor of Radiology

UPMC Children's Hospital of Pittsburgh

Tags

X-Ray (Plain Films)

Vascular Imaging

Vascular

Ultrasound

Stomach

Spine

Rectal/Anal

Pediatrics

Neuroradiology

Neonatal

Musculoskeletal (MSK)

Kidneys

Inferior vena cava

Genitourinary (GU)

Gastrointestinal (GI)

Esophagus

Congenital

Chest

Cardiac Chambers

Cardiac

Body