Esophageal Motility: Esophageal Motility: The Weak Esophagus

0:00Welcome to Board Pearls. This is episode four of four of the Esophageal Motility chapter, in the Esophageal Disorders module. This episode is the weak esophagus, through two patients. A forty-seven-year-old woman who arrives for a fundoplication and leaves with a diagnosis of scleroderma esophagus teaches why every wrap needs manometry first, how weak and failed swallows are counted into a diagnosis, what a very low sphincter pressure adds, how the systemic disease behind the tracing is found, and how her esophagus is protected once surgery is off the table. A sixty-one-year-old man with ineffective esophageal motility and proven reflux teaches the single manometric maneuver that decides whether he can have his wrap, and which wrap.

0:41Here's the first patient. A forty-seven-year-old woman has had severe heartburn and regurgitation for years, and over the last year solids and liquids both go down slowly. A twice-daily proton pump inhibitor, PPI, helps only partly. Her endoscopy showed erosive esophagitis and a short peptic stricture at the bottom of the esophagus. She's been referred to a surgeon for a Nissen fundoplication, and the surgeon has ordered high-resolution manometry before booking the operation.

1:11Manometry comes before every fundoplication because of what a wrap does. A wrap tightens the junction on purpose, so acid can't get back up. A normal esophagus can push a bolus through that tighter junction. An esophagus that can't generate a strong wave may not, and a wrap on top of weak peristalsis can turn mild dysphagia into severe dysphagia. So the manometry isn't a formality. It answers whether her esophagus has enough strength to push food past the wrap the surgeon plans to build. And her dysphagia to liquids as well as solids is already a hint that the problem is her push rather than a narrowing, because a structural narrowing holds up solids first.

1:47Her tracing is read in the order the Chicago classification, version four, reads every tracing: the sphincter first, then the body. The catheter carries thirty-six pressure sensors a centimeter apart, and the standard protocol is ten five-milliliter water swallows lying down and five sitting up. Her integrated relaxation pressure, the IRP, measures whether the lower sphincter relaxes enough for a bolus to pass; on the most widely used system, normal is under fifteen millimeters of mercury lying down and under twelve sitting up. Hers is normal. That rules out achalasia and outflow obstruction, the disorders of an elevated IRP. A weak esophagus has a normal IRP, because outflow isn't the obstacle: even a sphincter that relaxes normally doesn't help if nothing is pushing the bolus toward it.

2:36With a normal IRP, the reading moves to the body, and the body is assessed by counting swallows. The distal contractile integral, the DCI, measures how hard each swallow squeezes in the smooth muscle of the lower two thirds of the esophagus, as pressure times length times duration. Under one hundred is a failed swallow. One hundred to four hundred fifty is weak. Four hundred fifty to eight thousand is normal. A swallow can also be fragmented, meaning a break of more than five centimeters in the contraction wave at the twenty-millimeter pressure contour, even when the overall squeeze is above four hundred fifty. Failed, weak, and fragmented swallows all count as ineffective.

3:17Two diagnoses come from that count. Ineffective esophageal motility is a normal IRP with more than seventy percent of swallows ineffective, or at least half of them failed. That definition is stricter than the previous version, which required only half to be failed or weak, and it now counts fragmentation, which is why the diagnosis is made less often than it used to be. Absent contractility is a normal IRP with every single swallow failed. Every one of her swallows fails. She has absent contractility.

3:48There's an important look-alike here. Every swallow failing with an elevated IRP is type one achalasia. So the IRP decides between two silent esophagi, and they're treated in opposite ways. Achalasia needs the sphincter weakened so food can get out. Absent contractility needs its reflux treated, because acid that gets in isn't cleared. Misreading the IRP would mean cutting the sphincter of a patient whose problem is already too much reflux.

4:17Absent contractility describes the tracing, not the cause, and her resting sphincter pressure points to the cause. Hers is very low, under ten millimeters of mercury. That second finding is what turns a description into a likely diagnosis. Failed peristalsis plus a very low sphincter pressure is scleroderma until proven otherwise. Failed peristalsis with a normal sphincter pressure points instead to another cause of absent contractility, or to ineffective motility when some contraction remains.

4:46The reason scleroderma produces both is its mechanism. Fibrosis and obliteration of the small vessels destroy the smooth muscle of the lower two thirds of the esophagus and the nerves that drive it, while the striated muscle of the upper third is spared. The same fibrosis drops the resting sphincter pressure. So the barrier against reflux fails just as the pump that clears reflux fails. Acid pours up through an open sphincter and stays there, because there's no wave to strip it back down. That's why she has erosive esophagitis and a stricture, and why her PPI only partly works: it lowers the acid, but it can't clear what refluxes. On manometry, about eighty percent of patients with scleroderma have weak contractions, which over time often progress to absent contractility.

5:30The tracing alone doesn't prove scleroderma, because absent contractility has a broad differential, and the cause decides who else needs to be involved in her care. The same tracing occurs in scleroderma and other connective tissue diseases, in long-standing severe reflux, in diabetes, in amyloidosis, in alcoholism, in myxedema from hypothyroidism, in multiple sclerosis, and in otherwise healthy patients with long-standing reflux. So the workup is a directed history and directed tests. She's asked about Raynaud phenomenon, skin changes, dry eyes and mouth, diabetes, alcohol, and neurologic symptoms. The serologies are an antinuclear antibody, ANA, and antibodies against centromere, Scl-70, and ribonucleic acid, RNA, polymerase three. If amyloid or another paraprotein disorder is possible, serum and urine immunofixation and free light chains are added.

6:29When asked, she says her fingers turn white and then blue in the cold, and she's noticed the skin on her fingers getting tight. Her ANA and anti-centromere antibody are positive. She has scleroderma. The ANA is positive in more than ninety percent of patients with scleroderma, and anti-centromere is characteristic of the limited cutaneous form, also called CREST: calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias. Her esophagus is the E. So she also needs a rheumatologist, because the esophagus is only one organ the disease can affect.

7:07Her diagnosis changes the treatment plan she was referred with, and the first decision is what not to do. She should not have a fundoplication. Her esophagus has no peristalsis to push food past a wrap, and dysphagia after a Nissen in scleroderma is unacceptably common. Partial wraps are sometimes considered in carefully selected patients, but they aren't the default. Magnetic sphincter augmentation and transoral incisionless fundoplication aren't appropriate either, for the same reason. So the manometry did exactly what it was ordered for: it stopped an operation that would have made her worse, and the surgeon is told why.

7:42Since the muscle can't be repaired, everything is aimed at the reflux. A twice-daily PPI is the standard of care, with symptom response reported in more than ninety percent, and it continues indefinitely, because the disease that causes the reflux isn't going away. Raising the head of the bed six to eight inches goes with it, because during sleep peristalsis is least active and gravity is the only thing clearing her esophagus. If her symptoms stay refractory, a potassium-competitive acid blocker such as vonoprazan is an option, because it gives stronger acid suppression.

8:15The complications of that uncleared reflux need their own treatment. Peptic strictures develop frequently in scleroderma, so her stricture is dilated endoscopically, with incremental Savary bougie or balloon dilation, and she may need repeat sessions. Gastroparesis is common in scleroderma and adds to reflux by keeping more volume in the stomach, so it's looked for, and a prokinetic is considered if she has it. And she needs Barrett surveillance. Barrett esophagus develops in five to thirty-five percent of patients with scleroderma esophagus, because of the severe, uncleared reflux. Adenocarcinoma itself is rare despite that rate, but surveillance is still appropriate. In end-stage disease, esophagectomy with reconstruction has been done in selected patients.

9:00She stays on twice-daily PPI with the head of her bed raised, has her stricture dilated, and is scheduled for surveillance endoscopy. Her heartburn improves and solids go down more easily.

9:12The second patient has the milder and far more common version, and his is the case where the surgical question usually turns on the motility. A sixty-one-year-old man has reflux proven on a pH study off PPI, a small hiatal hernia, and erosive esophagitis. He wants surgery. His IRP is normal, and most of his swallows are weak or failed, but not all, with more than seventy percent ineffective. His resting sphincter pressure is normal. He has ineffective esophageal motility.

9:41It's a common finding, present in roughly twenty to thirty percent of patients tested for non-cardiac chest pain, reflux, or dysphagia, and its clinical relevance is debated, because many people with it have no symptoms or only nonspecific ones. Its usual companion is reflux, because a weak wave doesn't clear acid, and the same smooth muscle weakness can lower sphincter pressure. In most patients, treatment is aimed at the dominant problem, usually reflux; prokinetics such as metoclopramide, or domperidone where it's available, are sometimes tried, with limited evidence.

10:13For him, though, the question is the wrap, and one maneuver during manometry answers it: multiple rapid swallows. He takes five small sips of water, two milliliters each, two to three seconds apart. When the series ends, a healthy esophagus produces one strong contraction whose DCI exceeds the average of its single swallows. That rebound shows contraction reserve: the muscle can find more force when it needs it.

10:40With reserve, a wrap is less likely to cause dysphagia. Without reserve, the absent rebound supports the diagnosis of ineffective motility and predicts dysphagia after a wrap. And for a patient with ineffective motility who does go to surgery, a partial wrap, usually a Toupet, two hundred seventy degrees from behind, is generally preferred over a full Nissen, because a partial wrap obstructs a weak wave much less.

11:05His multiple rapid swallows produce a strong contraction afterward. He has reserve, and he has a laparoscopic Toupet.

11:13Here are the traps the boards set in these patients.

11:17In reading the tracing, doing a fundoplication without manometry risks wrapping an esophagus that can't push past it. Confusing absent contractility with type one achalasia, which only the IRP separates, would lead to cutting a sphincter that already lets too much reflux through. And missing the low sphincter pressure loses the clue that points to scleroderma.

11:37In absent contractility, failing to look for a systemic disease when every swallow fails misses scleroderma, amyloidosis, diabetes, and the others. Offering a fundoplication, magnetic augmentation, or a transoral fundoplication to a patient with scleroderma causes severe dysphagia. Stopping the PPI in scleroderma lets the reflux return, because it continues for life. And forgetting Barrett surveillance ignores a rate of up to about a third.

12:05In ineffective motility, ignoring the finding in a surgical candidate skips the multiple rapid swallows that predict dysphagia after a wrap. And choosing a Nissen when the motility is weak, instead of a partial wrap, invites that dysphagia.

12:20So, start to finish.

12:23Every fundoplication needs manometry first, because a wrap on a weak esophagus causes dysphagia. A weak esophagus has a normal IRP, and the diagnosis comes from counting failed, weak, and fragmented swallows and checking the resting sphincter pressure.

12:38More than seventy percent ineffective swallows, or half failed, is ineffective esophageal motility. It matters mainly before surgery, where contraction reserve after multiple rapid swallows predicts how a wrap will go, and a partial wrap is preferred.

12:54Every swallow failed with a normal IRP is absent contractility, and it calls for a search for systemic disease; with an elevated IRP, it's type one achalasia instead. Failed peristalsis plus a very low sphincter pressure is scleroderma, confirmed by Raynaud, skin changes, ANA, and anti-centromere.

13:16Scleroderma esophagus is treated through its reflux: a lifelong twice-daily PPI, head-of-bed elevation, stricture dilation, a search for gastroparesis, and Barrett surveillance, and never a fundoplication.

13:29The next chapter is reflux itself: how the barrier between the stomach and esophagus works, how endoscopy and pH testing prove reflux, how acid suppression is stepped up, and who should have surgery.

13:40For the full chapter, the practice vignettes, and the topic-tagged question bank, head to board pearls dot com. You'll find the rest of the series on Apple Podcasts, Spotify, or wherever you listen to podcasts. That brings us to the end of episode four of four of chapter two, and I'll see you in the next one.