Esophageal Motility: Esophageal Motility: Spasm and Jackhammer

0:00Welcome to Board Pearls. This is episode three of four of the Esophageal Motility chapter, in the Esophageal Disorders module. This episode is chest pain and dysphagia from an esophagus whose sphincter opens normally but whose body squeezes too early or too hard, through two patients. A forty-nine-year-old woman with distal esophageal spasm is followed from her emergency visits. Her case teaches how chest pain becomes a gastrointestinal, GI, problem, why endoscopy is her first test, which manometry number separates her from type three achalasia, what must be excluded before treatment, and how a disorder with no proven best treatment is treated. A fifty-three-year-old man whose esophagus squeezes on time but far too hard teaches the hypercontractile, or jackhammer, esophagus, and why an obstruction below it has to be excluded first.

0:50Here's the first patient. A forty-nine-year-old woman has had a year of episodes of squeezing chest pain, sometimes with food sticking for a few seconds before it goes down. Hot coffee and ice water both set it off, and so do large bites and stress. She's been to the emergency department twice. Cardiology found nothing: a normal stress test and a normal coronary angiogram. She has occasional heartburn. She takes no opioids.

1:16Her triggers are what move her from cardiology to a GI clinic. Chest pain that isn't cardiac is often esophageal, and two features of hers point to the esophagus specifically. Her pain comes with swallowing problems, and it's provoked by hot and cold liquids and large boluses, which stimulate esophageal muscle. That's the typical way these patients arrive: after a negative cardiac workup, often including catheterization or stress testing, still having pain. The cardiac workup has to come first, because missing a coronary problem is the worse error. Once it's negative, the question is whether her esophagus has a motility disorder, and if so, which one. Her occasional heartburn matters too, because reflux coexists in a meaningful share of these patients, and acid can provoke the very contractions a tracing might find.

2:05The first test for that question is endoscopy with biopsies, before manometry, for two reasons. A stricture or a lesion at the junction can cause dysphagia, and it can also make the esophagus squeeze harder above it to push past it, which a tracing would read as a muscle disease. And eosinophilic esophagitis causes dysphagia, has a known association with spastic motility patterns, and is diagnosed only by biopsy. Skipping the scope risks labeling a structural or inflammatory problem as a primary motility disorder and treating the wrong thing.

2:39Her endoscopy is normal, and so are her biopsies. Because she has heartburn, and because acid exposure can provoke the hyperexcitable response, she also starts a proton pump inhibitor, PPI, trial at this point. It costs little, and if reflux is driving her pain, it treats the cause.

2:56With structure and inflammation excluded, manometry looks for the motility disorder, read in the same order as always: the sphincter first, then the body. Her integrated relaxation pressure, the IRP, is normal, so her sphincter opens. That one fact separates her from type three achalasia. Type three also has premature, spastic contractions in the distal esophagus, but they sit above a sphincter that won't relax. So a normal IRP with spastic contractions isn't achalasia, and an elevated IRP with spastic contractions is. The distinction matters because type three achalasia is treated with peroral endoscopic myotomy, POEM, while spasm starts with pills.

3:37With the sphincter cleared, the body is read for timing. Distal latency is the time from relaxation of the upper esophageal sphincter at the start of the swallow to the contractile deceleration point, the place within three centimeters of the junction where the fast proximal phase of the wave slows into distal emptying. Normally it's four and a half seconds or longer, because the wave needs time to travel down. In her, on more than a fifth of swallows, the contraction arrives in under four and a half seconds. Those are premature contractions: the smooth muscle of the distal esophagus is hyperexcitable and clamps down before the wave has propagated, so the bolus is squeezed by a segment that isn't ready for it. Her other swallows are normal, and that's allowed; spasm can be interspersed with normal peristalsis.

4:25So she meets the definition of distal esophageal spasm in version four of the Chicago classification: a normal IRP, premature contractions with a distal latency under four and a half seconds on at least twenty percent of swallows, and dysphagia or chest pain.

4:41That definition has two features the boards test, and both exist to avoid overcalling the disease. Symptoms are part of it, because short-latency swallows on the tracing of someone without symptoms are common and aren't a disease, so a tracing alone isn't treated. And the timing measure replaced speed. The old conventional definition used simultaneous contractions, a contraction front velocity over six to nine centimeters per second. That turned out to be nonspecific: in a review of more than a thousand high-resolution tracings, the patients with rapid contractions had a mixture of diagnoses, including weak peristalsis, normal peristalsis, and outflow obstruction. Short latency picked out a distinct group, ninety-six percent of whom had dysphagia. So contraction front velocity has been removed from the current classification entirely, and latency is what counts.

5:34If she had a barium swallow, it might show a corkscrew or rosary-bead esophagus from tertiary, uncoordinated contractions. But the diagnosis is made on manometry, and it doesn't require the radiographic finding.

5:47Before her spasm is treated, its contributors are excluded, because each is treatable and fixing it may be all she needs. Opioids matter most in any patient who takes them, because they cause premature and overly strong contractions and raise the contractile integral, producing a tracing that looks exactly like spasm. In a patient on opioids, stopping them comes before any spasm treatment, or the drug's effect is what gets treated. She takes none. Her reflux is covered by the PPI trial she's already on, and eosinophilic esophagitis has been excluded by her biopsies.

6:20Her treatment starts with an honest conversation. High-quality evidence is limited for every option. And the link between the tracing and the symptoms is looser than in achalasia, so who will respond is harder to predict. She's told that up front, because a patient who expects a cure from the first pill will feel the treatment failed when it only helps. The approach still has a logic: relax the smooth muscle first, then dampen the nerves' pain signaling, and keep procedures for disabling disease that hasn't responded to either.

6:51Smooth muscle relaxants come first, because the problem is muscle contracting at the wrong time. Diltiazem, sixty to ninety milligrams four times a day or a sustained-release form, is the usual calcium channel blocker. Short-acting nifedipine is avoided because of its cardiovascular effects. Nitrates are an alternative: isosorbide dinitrate, five to ten milligrams under the tongue before meals, or oral isosorbide mononitrate. Sildenafil, fifty milligrams, has been used off label, because inhibiting phosphodiesterase five prolongs the nitric oxide signal that relaxes smooth muscle, the same signal that's missing in achalasia. Peppermint oil has support from small trials, because the menthol in it acts as a calcium channel blocker on smooth muscle.

7:36Neuromodulators follow when relaxants fail or aren't tolerated, and they're the same drugs used for functional chest pain: a low-dose tricyclic such as imipramine or amitriptyline, twenty-five to fifty milligrams at bedtime, trazodone, or a selective serotonin reuptake inhibitor, SSRI. They don't change the contractions. They act centrally, dampening the visceral pain signaling from the esophagus rather than altering the muscle.

8:02Procedures are kept for refractory disease. Botulinum toxin injected along the distal esophagus has been used in patients who haven't responded to medicine. And for refractory, disabling symptoms with clear spasm on manometry, POEM with a long myotomy over the spastic segment is an option, with short-term response in roughly eighty to ninety percent in dedicated series. But the best treatment for spasm isn't well defined, and the mismatch between tracing and symptoms makes success harder to predict, so a myotomy isn't offered to someone whose symptoms are tolerable.

8:34She starts diltiazem and continues her PPI. Over two months, her episodes become less frequent and less severe. If they return, amitriptyline at bedtime is the next step, and a procedure is far down the list.

8:48The second patient has the other disorder of this group, and the boards pair them because they look alike at the bedside. A fifty-three-year-old man has the same story: chest pain and dysphagia after a normal cardiac workup. His IRP is normal, and his contractions arrive on time, with a normal latency. But they're enormously strong. The distal contractile integral, the DCI, which measures the vigor of the contraction as pressure times length times duration, is over eight thousand on more than a fifth of his swallows.

9:19That's a hypercontractile esophagus, often called jackhammer esophagus: a normal IRP with a DCI over eight thousand on at least twenty percent of swallows, with dysphagia or chest pain. It's rare. The cutoff of eight thousand is deliberately high, because healthy people top out around five to six thousand, and the goal is to count only truly abnormal vigor. The mechanism is presumed hyperexcitability of the smooth muscle, possibly with longitudinal muscle contraction magnifying the pressure signal. The term replaced the older nutcracker esophagus, defined on conventional manometry by a mean amplitude more than two standard deviations above normal; its relevance was debated, and many patients labeled with it didn't have hypercontractility on high-resolution testing, which is why the classification abandoned the term.

10:07His workup has one extra concern, and it's the same one that made endoscopy first for her. An obstructive lesion just below the manometry sensors, at the junction, can make the esophagus contract harder to push past it, producing a high contractile integral that's a compensation, not a disease. So endoscopy and a careful look at the junction come before calling his disorder primary. Opioids also raise the contractile integral. And reflux coexists in a substantial fraction and may drive the high-vigor swallows.

10:36His treatment follows the same sequence as hers, because at the bedside the two disorders often can't be told apart: stop opioids, treat reflux, exclude eosinophilic esophagitis, then calcium channel blockers, nitrates, sildenafil, or peppermint oil, then a tricyclic or another neuromodulator, then botulinum toxin, and POEM only for selected refractory, disabling disease. His response is just as variable and inconsistent, so counseling him that no specific regimen has been proven is part of his management.

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

11:14On the tracing, calling it spasm when the IRP is elevated mistakes type three achalasia, which is treated with POEM. Diagnosing a motility disorder from a tracing in someone without symptoms treats a finding that's common in healthy people. And relying on contraction speed instead of latency uses a criterion that's been dropped for being nonspecific.

11:36In the workup, skipping endoscopy misses eosinophilic esophagitis and an obstruction that makes the esophagus squeeze harder above it. And treating a patient on opioids before stopping them treats the drug's effect.

11:48In treatment, using short-acting nifedipine adds cardiovascular risk. Reaching for POEM before medicines have been tried skips the safer steps. And promising a patient that a specific drug will work ignores how unpredictable response is.

12:04So, start to finish.

12:07Chest pain and dysphagia after a negative cardiac workup point to the esophagus. Endoscopy with biopsies comes first, to exclude eosinophilic esophagitis and obstruction, with a PPI trial if there's heartburn.

12:20On manometry, a normal IRP separates these disorders from type three achalasia. A distal latency under four and a half seconds on at least a fifth of swallows is distal esophageal spasm. A contractile integral over eight thousand on at least a fifth is a hypercontractile esophagus. Both require symptoms.

12:41Stop opioids and treat reflux. Then treat in order: smooth muscle relaxants such as diltiazem or nitrates, then a neuromodulator, then botulinum toxin, and POEM only for disabling disease that hasn't responded. And tell the patient that no regimen is proven.

13:00Episode four is the opposite problem: an esophagus that squeezes too weakly or not at all, and why that matters most for reflux and before a fundoplication.

13:09For 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 three of four of chapter two, and I'll see you in the next one.