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Didelphys Uterus – Pediatric

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Moving on to our next case, this is a 12-year-old

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who came into the ED with acute pelvic pain.

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Generally, you don't start with a CT scan, but

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in this particular case, this is what we have.

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So starting from the bottom up, we can

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already see this IV contrast-enhanced CT

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that something is going on in the pelvis.

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We have this large fluid collection

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surrounded by different structures right here.

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It's certainly quite a bit lower than you'd

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expect to see an ovarian cyst, for example.

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We do have a little bit of free fluid

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back here as well, anterior to the rectum.

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And then it's hard to figure out

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exactly what's going on right here.

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We have this structure leading out to the right

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adnexa, the structure leading to the left adnexa.

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So again, whenever you're looking at

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female pelvic imaging on a CT, CT is not

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the best modality to show you everything

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going on with the female pelvis.

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However, it's worthwhile trying to figure out

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what you're seeing because you can, a lot of

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times, at least lead to what should happen next.

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I would argue this is probably the ovary

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right here is a little follicle right there.

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You can follow it all the

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way up if you're not sure.

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All the way up.

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And you can follow it all the way

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up to the ovarian vein as it dumps

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into the left renal vein there.

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But going back to what's going on down deeper

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in the pelvis, let's move to our coronal.

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In this case, starting anteriorly with the

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bladder right here and moving posteriorly,

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we have this big fluid-filled, slightly

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heterogeneous structure right here.

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And if you stop right here, we can see those

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two structures that were sort of heading

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out towards the adnexa bilaterally.

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They sort of meet right here.

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So these are not fallopian tubes.

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These are way too thick to be fallopian tubes.

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To have this amount of fluid in them

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and then have this degree of thickness

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symmetrically, that would just be unusual.

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So what this is, are these are

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two divergent uterine horns.

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And then you have this

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process going on down here.

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So what this is, is a didelphys

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uterus with hematocolpos, most

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likely due to a vaginal septum.

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Here's our transverse uterus picture from her.

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So we have our bladder right here.

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This is transabdominal.

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She's 12 years old.

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So we were not going to do

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transvaginal in this particular case.

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As we scroll through.

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And the uterus cervix is going to be

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down here in this particular area.

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This is post-operative, I should let you know.

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There's no specific structure

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that you're going to see.

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And you can nicely see here the two horns,

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and they're very, very widely divergent

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as we head out into the adnexa there.

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Very widely divergent.

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So let's go back here again, you can see as

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we're coming from the fundus down, down here.

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You never actually see them meet if there's

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two separate cavities this entire time

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until we get down to the vaginal area.

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So in this particular case, she was

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diagnosed with Herlyn-Werner-Wunderlich syndrome, which is

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obstructed hemivagina with renal agenesis.

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And if we go back to the CT scan now, you can

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see there's one large hypertrophied kidney.

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And there is no right-sided kidney.

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Another way you can tell if there was ever a

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right kidney is to try and look at the adrenal

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gland, which she's so skinny, it's hard to see.

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But a normal adrenal gland

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will form a V or a Y shape.

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And if there is never a kidney in this

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position, you'll have a pancake or a

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flat adrenal gland, which is right here.

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There was never a normal kidney

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in that right renal bed.

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Instead, you have a single left kidney,

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which has compensatory hypertrophy,

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which is why it's so large right there.

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So didelphys, that is a failure of Mullerian

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duct fusion, so both of them form, but

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they don't fuse together, and they don't

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fuse at all in any particular area.

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So you will have two horns that are widely

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divergent, and you'll have two cervices.

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About 75% of the time, these are

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associated with a proximal vaginal septum.

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And that may be how these

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patients end up coming in.

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They don't necessarily get a period or

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they start their periods, but part of

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it's going to be obstructed right here.

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And they come in with these

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heterogeneous fluid collections.

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So if you have an MRI on a patient,

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you should always look for that vaginal

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septum because that changes management.

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It's again, other things you're looking

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for besides that deep fundal cleft.

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You can see how widely spaced these

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two horns are very widely spaced.

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And if you have a transvaginal ultrasound,

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you're going to look for two cervixes.

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And again, MRI always looks for a hemivagina.

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If you think you have a didelphys uterus.

Report

Faculty

Kathryn McGillen, MD

Assistant Professor of Radiology, Medical Director of Ultrasound

Penn State University Milton S Hershey Medical Center

Tags

Uterus

Ultrasound

MRI

Gynecologic (GYN)

CT

Body

Acquired/Developmental

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