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I'm Dr. Joseph Kumka, Gastroenterology Fellow, educator, and creator of this podcasts. Whether you're a resident gearing up for the boards, a fellow diving deep into subspecialty topics, or a practicing clinician hungry for high-yield updates—you’re in the right place.
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Welcome to the Deep Dive.
Today, we're tackling something pretty complex in Gosesophagalility disorders.
Yeah, it's a field full of technical jargon, you know, lots of acronyms.
It can definitely feel overwhelming if you don't have a clear map.
Absolutely.
So our mission today is to take the latest diagnostic standard, the Chicago Classification version 4.0, and really burn it down for you.
We want to give you a clear step by step guide.
Show how cllamissions actually think through these cases from a patient's symptoms right through to interpreting those tricky monometries readouts.
Exactly.
We're aiming for that high yield understanding.
By the end of this, you should grasp the, what is it, three core measure met?
Yeah, three key metrics, yeah.
IRP, DL, and DCI.
Get those, and you can really understand that the classification and crucially how it guides treatment.
Okay, so we'll hit the big ones.
Accalasia, all three types, EGJ, outflow obstruction , distalosophagal spasm.
Hypercontractile or Jack Hammer's office.ophagus, inffective motility and absent contractility, the old range.
But let's start where it always starts with the patient.
Right.
So someone comes in, maybe they have dysphagia, trouble swalling, could be a liquid solids or both, or maybe it's chest pain that isn't heart related or really stubborn reflex.
Regurgitation, too, sometimes.
Yeah, regurgitation.
Now, the gut reaction might be, let's test how the esophagus is squeezing.
So why don't we jump straight to monometry?
Ah, that's the absolute crucial first point.
And honestly, a common pitfall.
If someone has dysphagia, especially if they have alarm symptoms?
Like losing weight unexpectedly, anemia being over 50.
Exactly.
You have to rule out a structural problem first, an arm tree comes later .
Always.
So step one is always a an upper endoscopy, an EGD.
Correct.
You need that scope first.
And what are we hunting for with the scope?
I mean, besides an obvious blockage, like a stricture or a web.
The main thing, the critical thing, is ruling out pseoacqualasia.
That's basically a malignancy, usually an adocarcinoma right down in the GE junction.
And it mimics true acylasia.
Perfectly.
It causes outflow obstruction, looks the same onarium swallows sometimes .
If you skip that EGD, you could mistake a cancer for a benign motility disorder.
The consequences, well, they're obviously severe.
Okay, message received loud and clear.
EGED first.
So if the scope is clean, no tumor, no major blockage, what's typically the next step before monometry?
Often, we'll go for a bum mesophogram, sometimes a timed one, it adds functional information, the scope sees the lining, the barium shows how things look when swallowing happens.
Right.
This is where we get those textbook pictures.
Precisely.
You might see that classic, smooth, narrowing at the bottom of the bird's beak, which screams accolasia.
Or the twisty look.
Yeah, or the corkscrew or rosary bead pattern, if it's more of a spastic thing.
But, you know, these are suggestive.
To really classify the disorder accurately, you need the pressure readings, the numbers.
Which brings us finally to the gold standard .
High resolution Monometry, HRM.
That's the one.
Everything in Chicago 4.0 hinges on the HRM data.
Okay, let's dig into into those numbers.
You said three key metrics.
Can you break them down for us?
Make it less technical.
Let's try.
Think of it like this.
The esophagus has a gate at the bottom, a signal controlling the muscle squeeze, and the power of that squeeze itself.
Gate, signal power.
Okay, First, the gate.
That's the IRP integrated relaxation pressure.
It measures how well the lower esophagal sphter, the LS actually opens up when you swallow.
So if the IRP number is high...
The gate's stuck.
It's not relaxing properly .
Impaired LES relaxation.
Got it.
High IRP points towards an outflow problem, like acalia or maybe EGOO.
Exactly.
Second, the signal.
That's DL Dal latency.
This is all about timing.
Does the wave of contraction travel down the esophia smoothly or does it fire off too early?
And there's a specific cutoff time.
Yes.
The magic number is 4.5 seconds.
If the DL is short, less than 4.5 seconds, it means the contraction started prematurely.
That's spasm territory..
Okay, gate, IRP.
Signal timing, DL.
That's the third one, Power.
Right, the punch.
That's the DCI, distal contractile integral.
This measures the strength or vigor of the squings in the main part of the assultesophagus.
So this tells us if it's squeezing too hard or too weakly?
Precisely.
If the DCI is really high, over 8,000, that's a hypercontractile, super strong squeeze in a jackhammer.
If it's really low, under 450, the squeeze is weak, ineffective.
IRP DL DCI.
Gate signal punch .
Okay, that framework really helps.
So let's follow the logic.
First question.
Is the IRP elevated?
Is the gate stuck?
Okay, if the IRP is elevated, yes, we've got an outflow problem.
We're dealing with something in the Accalagia family, or E.G. JOO , how the muscle above the blockage behaves tells us which one it is.
Let's start with Te Ialia, the classic kind.
Type I is straightforward, high IRP, and basically absent peristalsis.
The muscle in the esophial body is just given up.
It's flaccid deb above the tight LS.
No squeeze at all.
Okay, type two.
This one sounds weird.
Panisophagal pressurization.
What's that look like?
Yeah, type 2 is elevated IRP plus, in at least 20% of swallows, you see this widespread simultaneous pressure build up throughout the esophagus.
Like the whole tube is squeezing at once instead of in a wave.
Exactly.
It's trying to force things through the blocked LS, generating pressure everywhere.
Interestingly, this residual muscle activity means type two actually has the best prognosis.
We'll get to that.
Okay.
And type three, the spastic type.
When Type 3 is often the most painful.
You've got the elevated IRP, the stuck gate, plus those premature contractsions.
Remember the short deal .
Less than 4.5 seconds and 20% are Morse walls.
So, it's blocked at the bottom and spasming above it.
Ouch.
Yeah, it's a double whammy.
Blockage and spasm.
That covers the three accolias.
What about the last one in the high IRP category?
EGJ outflow Obstruction or AGJOO?
Ah, EGJO-O. This is a tricky one.
It has the elevated IRP, likeylia, but crucially, the peristis, the muscle wave above it , is normal, or at least somewhat preser.
So the muscle itself is working, but the pressure reading at the junction is high.
Why is that tricky?
Because it's often not a true fixed obstruction.
It can be an artifact.
An artifact, you mean like the test wasn't quite right.
Could be.
Maybe the monometry catheter got kicked slightly, or the patient has a big hiatal hernia pressing on the area .
Or very commonly it's related to medications, especially opioids, they can tighten up the LES.
So you can't just treat based on that number alone?
Absolutely not.
With EGOO, you must get corroborating evidence.
Do a time bum swallow, does it empty slowly or maybe an endo flip test to measure the actual opening diameter and distens?
You need proof of a real mechanical issue before you consider treatment.
Right.
Confirm before intervening.
Makes sense.
Okay, let's shift gears.
What if the IRP is normal ?
The gate is opening?
Okay.
If the IRP is normal, the LES isn't the primary problem, so now we look at the timing, the DL.
The signal speed.
Right.
If the IRP is normal, but the DL is short under that 4.5 second threshold in at least 20% of the swallows, the musc is firing too early.
That's Dal lesophagal spasm, DE.
Creature uncoordinated squeezes.
Exactly.
And that's the motility disorder that typically correlates with that corkscrew appearance you might see on a barium study , defined by the timing abnormality.
Okay.
Now, what if the gate is fine, normal IRP, and the timing is fine?
Normalormal deal?
Then we look at the last piece.
The power, the DCI.
The punch.
Let's start with too much power.
If IRP and DL are normal, but the DCI is huge over 8, are more swallows, that's hypercontraction.ile esophagus, better known as jackhammer esophagus.
Slamming contractions, right?
I imagine that causes significant pain.
Often intense chest pain.
Yeah.
It's just pure excessive force.
And no, this replaced the old term nutcracker esophagus.
Jackhammer is the current term for DCI 8000.
Good clarification.
Now, the other extreme, normal gait, normal timing, but weak power.
That's ineffective esophical motility, IEM .ed by a low DCI under 450 in the majority, usually over 50% or even 70%, depending on criteria, of swallows.
The squeezes are just too feeble to properly clear food or refluxate.
And you see this a lot in people with GERD.
Very commonly.
It's thought that the poor clearance from the weak contractions likely worsens their refux symptoms, because stuff that comes up doesn't get pushed back down effectively.
Makes sense.
And if the weakness is total , like zero power.
If you have 100% failed swallows, DCI basically is zero, that's classified as absent contracts..
Yeah.
The esophagal body muscle just isn't contracting at all.
Now, speaking of absent contractility, there's a really important condition to differentiate here, isn't there?
Scleroderma.
Yes, absolutely critical.
Scleroderma esophagus has a very specific monometry signature.
What is it?
It's absent contractility, just like the idiopathic form , but it also has a hypotensive LS.
The lower esophel sphinter pressure itself is abnormally low.
So not only does the pump not work, but the valve at the bottom is leaky.
Exactly.
The gate is floppy and wide open, and that combination is devastating clinically.
Because without that LES barrier, you get massive free flowing reflux from the stomach right up the esophagus .
It leads to severe esophagitis, strictures, and a very high risk of aspiration into the lungs.
It's the classic esophageal finding in crest syndrome.
Okay, that low LES pressure is the key differentiator.
Now, quickly, before we hit treatments, what about those behavioral mimics?
Ratesumination and supergastric belching?
How do we spot those?
Right.
These can confuse things.
The clue on monometry, often using impedence monitoring, too, is that the primary event is't in the esophagus muscle itself.
So what do you see for rumination?
With rumination syndrome, the patient effortlessly brings up recently eaten undigested food .
Monometry shows a sudden spike and gastrop pressure just beforeurg.
It's an abdominal muscle contraction, a voluntary or subconscious squeeze pushing the food up.
Not an esophageal problem, but an abdominal action.
Correct.
And with supergastricing, the patient complains of excessive .
But the imped sensors show air, entering the esophagus from above, they're sucking air in, and then immediately expelling.
It's not gas coming up from the stomach.
Oh, again, a behavioral pattern, not a motility disorder, and the treatment reflects that.
Totally different.
Treatment for both is behavioral things like diaphragmatic breathing exercises, cognitive behavioral therapy , not pills or surgery.
Okay, that distinction is vital.
Let's finally talk treatment strategy.
How does our IRPD,LDCI diagnosis guide what we actually do for the patient?
It lines up pretty well.
Yeah.
Fundamentally, if the problem is accalia spectrum or confirmed AGJO, meaning elevated IRP of achanical block.
No, you need to open the gate mechanically.
Exactly.
That means procedures, helleriotomy, surgery, pneum dilation, PD stretching with the balloon, or poem, Ploscopomy, cutting the muscle from inside with the scope.
And if it's one of the spastic disorders, normal IRP, but abnormal timing, D or power, jackhammer?
Then the muscle is overactive, maybe hypersensitive.
So you try medical therapy first.
Smooth muscle relaxants, calcium channel blockers, nitrates, maybe sylophil, sometimes low dose antidepressants, like TCAs, can help with pain signaling, too.
Let's drill down on acylia treatment.
You mentioned type two has the best prognosis.
Why again?
Because in type 2, that panosophagal pressurization shows the esophagal body muscle still has some oom .
Once you relieve the LES of obstruction with Heller, PD, or poem, that residual pressure helps clear the esophagus effectively.
They respond well to pretty much any of his standard treatments.
What about type three, the spastic type?
Why is PM often preferred there?
Ah, Type 3 is tough.
It's got the LS block and that long segment of spastic muscle contracting prematrial above it.
Right, the double problem.
So a standard helleromyotomy or a balloon dilation mainly just targets the LS itself.
It might not adequately address the extensive spasm higher up.
But POM can.
Poem gives the endoscop the flexibility to extend theomy, the muscle cut much further up the esophagus, cutting through that spastic segment along with the L .
That longer cut is often needed for durable relief in type 3.
So poem's advantage is the ability to customize the length of the muscle cut for the specific problem.
What about Botox?
Where does that fit in?
Botox injections into the LS are really reserved for patients who are too frail or elderly for the more definitiveitive procedures like surgery or poem.
It works, but the effect is temporary.
Usually only lasts 6, 12 months.
It's more palliative.
And quickly, if medical therapy fails for severe DES or jackasshammer, can poem help there too?
Yes, it's becoming an option.
If someone has debilitating symptoms from spasm or hypercontraction that don't respond to medication , poem can be used to perform a targeted myomy of just the affected segment of the esophagal body, leaving the LS intact.
Fascinating how the endoscopic tools are being adapted.
So that really walks us through the whole Chicago 4.0 process.
From symptoms, the crucial EGD ruleout.
Through understanding IRP, DLDC.
To classifying the specific disorder and finally matching it to the most logical treatment strategy.
It's a structured approach.
Yeah.
And the key really isn't just knowing the definitions.
It's about constantly applying that framework and remembering the checks and balances.
Like the EGD first rule?
Always.
Even if the monometry looked like textbook type I acia, you have to know you ruled out pseudo-acia first.
True understanding here means knowing the rules, yes, but critically knowing the exceptions and the potential traps along the way.
That's what ensures patient safety.
A fantastic point to end on.
Thinking critically about the whole process, not just the final label, thank you for joining us for this deep dive today.
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