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The normal-IRP side of the Chicago algorithm covers everything that is not achalasia or EGJOO. The sphincter relaxes; the question shifts to what the body is doing. Distal esophageal spasm is a timing problem (premature contraction). Hypercontractile esophagus is a vigor problem (DCI above 8000). Ineffective motility and absent contractility sit at the failed end. Scleroderma is the one disorder that breaks the rule, presenting as absent contractility plus a hypotensive LES that lets reflux through. And EGJOO is the elevated-IRP-with-preserved-peristalsis bucket that v4.0 made harder to call on purpose, because v3.0 was overcalling it.
The case. A patient with chest pain and intermittent dysphagia. Cardiac workup is negative. Manometry shows a normal IRP, 30% of swallows with a distal latency of 3.8 seconds, and a normal DCI on the rest. What is the diagnosis, and what is the first treatment move?
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Welcome to Board Pearls. This is episode two of two of the Esophageal Motility chapter, in the Esophageal Disorders module. In this episode we cover the non-achalasia motility disorders: spasm and hypercontractile patterns with a normal integrated relaxation pressure, the failed-peristalsis-plus-hypotensive-LES dual hit of scleroderma, and the EGJOO bucket that FLIP and timed barium often rescue.
The organizing principle for this whole set is that the first episode already used the integrated relaxation pressure to identify achalasia. Once achalasia is off the table, the algorithm splits cleanly. A normal IRP funnels the patient into a disorder of the esophageal body, and the body either contracts too early, too hard, or not enough. An elevated IRP with some preserved peristalsis funnels the patient into the EGJOO bucket, which v4 treats as a starting point for further workup rather than as a diagnosis. Everything in this episode lives on one of those two paths.
Start with the normal-IRP path and the disorders that come from a hyperexcitable distal esophageal body. Distal esophageal spasm and hypercontractile esophagus sit together because they share a clinical phenotype of dysphagia and non-cardiac chest pain in a patient whose cardiac workup is clean. They also share the IRP. The lower esophageal sphincter relaxes normally on every swallow, which is what lets you separate these patients from type three achalasia at the top of the algorithm.
Distal esophageal spasm in Chicago version four is a normal IRP with at least twenty percent of swallows showing a distal latency under four and a half seconds, in a symptomatic patient. The distal latency is the time from upper sphincter relaxation to the contractile deceleration point in the distal esophagus. A short latency means the contraction reached the distal segment too early relative to swallow initiation. The bolus is being squeezed by a wave that has not yet propagated. The reason version four uses distal latency instead of the older contractile front velocity is empirical. A study of more than a thousand high-resolution tracings showed that rapid contractile front velocity captured a heterogeneous group that did not behave clinically like spasm. A short distal latency picked out a phenotype in which nearly all patients reported dysphagia. Contractile front velocity was dropped from version four entirely.
Hypercontractile esophagus, the diagnosis you will hear called jackhammer, is a normal IRP with at least twenty percent of swallows showing a distal contractile integral over eight thousand, in a symptomatic patient. The threshold is set high deliberately. The upper end of the normal distribution in healthy controls is around five to six thousand. The cutoff at eight thousand is meant to capture truly abnormal contractile vigor and not the upper tail of normal. The older term nutcracker esophagus used a mean amplitude two standard deviations above normal on conventional manometry. It was dropped because many patients labeled with it did not have hypercontractility on high-resolution manometry, and the clinical relevance was debated. Recognizing that distinction matters. A patient carrying a fifteen-year-old nutcracker diagnosis from water-perfused manometry should be restudied on high-resolution manometry before any of it is treated as primary.
Both of these diagnoses require symptoms in version four, and that change is doing real work. Asymptomatic short-distal-latency swallows and asymptomatic high-DCI swallows are common findings and do not constitute disease. The version four wording explicitly calls these patterns clinically inconclusive when symptoms are absent. The recognition point is that the manometric pattern is necessary but not sufficient. A patient with twenty-five percent premature swallows and no dysphagia and no chest pain does not have spasm.
The look-alike that misleads is type three achalasia. Type three also has premature spastic contractions in the distal body, but the IRP is elevated and the sphincter is not relaxing. Reading the body pattern first and forgetting to check the IRP is the place careful reasoning leads to the wrong answer. A premature contraction with an elevated IRP is type three achalasia and the answer is a tailored myotomy, usually POEM with proximal extension. A premature contraction with a normal IRP is distal esophageal spasm and the answer is the medical ladder. The two patients can look identical on the body tracing alone.
Before treating spasm or jackhammer as primary, work through the things that produce the same pattern secondarily. Opioids elevate the DCI and shorten the distal latency by acting on enteric inhibitory neurons. Chronic oxycodone can produce a manometric phenotype indistinguishable from spasm, jackhammer, or type three achalasia, depending on which parameter dominates. An opioid history is the cue that opioid withdrawal is the first therapeutic test before any procedure. GERD coexists in a meaningful subset and acid exposure can drive the hyperexcitable pattern, so a PPI trial is part of the workup when reflux symptoms are present. Eosinophilic esophagitis can present with a spastic or hypercontractile pattern, and biopsies belong in the initial EGD. A mechanical narrowing distal to the sensor can produce a compensatory high-DCI pattern, which is another reason EGD comes before declaring the motility primary.
The treatment ladder for both disorders is built on smooth muscle relaxation and dampening visceral hypersensitivity, and the evidence is limited at every step. After ruling out the secondary contributors, start with calcium channel blockers. Diltiazem at sixty to ninety milligrams four times daily, or a sustained-release preparation, is the standard. Short-acting nifedipine is avoided for cardiovascular reasons. Nitrates such as isosorbide dinitrate sublingually before meals are an alternative. Sildenafil at fifty milligrams works off-label by prolonging nitric-oxide-mediated smooth muscle relaxation. Peppermint oil contains menthol, which acts as a calcium channel blocker on smooth muscle, and small trials support it. When the smooth muscle relaxant approach fails, move to a neuromodulator. A low-dose tricyclic such as imipramine or amitriptyline at twenty-five to fifty milligrams at bedtime, or trazodone, or an SSRI, dampens visceral afferent signaling centrally rather than altering contraction. Botulinum toxin injected along the distal esophagus is reserved for refractory cases. POEM with a long myotomy is for refractory disabling disease with well-documented spasm or jackhammer. Response rates are reasonable. The mismatch between manometric findings and symptoms in this population makes outcomes harder to predict than in achalasia.
Now to the other side of the normal-IRP path, where the body is hypocontractile rather than hyperexcitable. Ineffective esophageal motility, absent contractility, and scleroderma esophagus all sit on a normal IRP and they sort by two questions. How much of the peristalsis is gone, and what is the sphincter pressure doing.
Ineffective esophageal motility in version four is a normal IRP with greater than seventy percent ineffective swallows or fifty percent or more failed swallows. An ineffective swallow includes a failed contraction with DCI under one hundred, a weak contraction with DCI between one hundred and four hundred fifty, or a fragmented contraction. Fragmented means a peristaltic break greater than five centimeters in the twenty-millimeter isobaric contour. The version four definition is stricter than version three, which only required fifty percent failed or weak swallows, and it now incorporates fragmentation. The diagnosis rate dropped meaningfully when the new criteria were adopted. The clinical relevance is debated because a substantial fraction of patients with the manometric pattern are asymptomatic or have non-specific symptoms.
Where ineffective esophageal motility actually changes management is the preoperative fundoplication workup. The reasoning is mechanical. A complete three-hundred-sixty-degree Nissen requires a peristaltic pump that can push a bolus past a tight wrap. Weak peristalsis cannot do that, and a Nissen placed on top of severe ineffective motility converts mild dysphagia into severe dysphagia. The way you decide whether the muscle has reserve is the multiple rapid swallow maneuver. Five small wet swallows two to three seconds apart should produce a single robust post-swallow contraction whose DCI exceeds the mean of the standard wet swallows. A robust post-MRS contraction means the muscle has latent reserve and the patient is likely to tolerate a complete wrap. Absent contraction reserve in a patient with ineffective motility predicts post-fundoplication dysphagia and points toward a partial wrap. A Toupet at two hundred seventy degrees posterior or a Dor at one hundred eighty degrees anterior is preferred in that setting.
Absent contractility is a normal IRP with one hundred percent failed peristalsis, every swallow producing a DCI under one hundred. The IRP is what separates this from type one achalasia. Same body pattern of universal failed swallows, but a normal IRP means the sphincter is not the problem, and an elevated IRP means it is. The differential for absent contractility is broad. Scleroderma and other connective tissue disease lead the list. Long-standing severe GERD, diabetes mellitus, amyloidosis, alcoholism, myxedema, and multiple sclerosis all produce the same manometric endpoint. The workup is directed at the underlying systemic disease. The history asks about Raynaud, skin tightening, sicca symptoms, neuropathy, alcohol exposure, and paraproteinemia. The serologies include ANA, anti-centromere, anti-Scl-70, and immunofixation when amyloid is suspected. Treatment of absent contractility itself is symptomatic. The motility cannot be repaired, so management is aimed at the downstream consequence, which is reflux.
Scleroderma esophagus is the canonical disorder of failed peristalsis combined with a hypotensive lower esophageal sphincter, and the dual hit is what makes it distinctive. Fibrosis and vascular obliteration take out the smooth muscle of the distal two thirds of the esophagus while sparing the striated upper third. The same fibrotic process drops the resting LES pressure below ten millimeters of mercury. The pump fails and the antireflux barrier opens up in the same disease. The clinical phenotype follows directly. Acid pours back across an open sphincter and there is no peristaltic wave to clear it. The downstream consequences are severe erosive esophagitis, peptic stricture, and Barrett esophagus in five to thirty-five percent of patients. Esophageal adenocarcinoma is rare despite the Barrett rate, but Barrett surveillance at standard intervals is appropriate.
The recognition pattern on manometry is the combination of absent or near-absent contractility and a hypotensive LES. About eighty percent of scleroderma patients have hypocontraction at diagnosis, and many progress to absent contractility over time. The combination separates scleroderma from primary absent contractility, in which the LES pressure may be normal. It also separates it from achalasia, in which the IRP is elevated. The systemic disease is confirmed serologically. ANA is positive in over ninety percent. Anti-centromere antibody is the marker of the limited cutaneous form, the CREST phenotype standing for calcinosis cutis, Raynaud, esophageal dysfunction, sclerodactyly, and telangiectasias. Anti-Scl-70 marks the diffuse cutaneous form with greater visceral organ involvement.
Management of scleroderma esophagus is aggressive reflux control because the motility cannot be repaired. Twice-daily PPI is the standard of care and is continued indefinitely, with over ninety percent symptom response. Head-of-bed elevation of six to eight inches augments gravity-driven nocturnal clearance when peristalsis is least active. Vonoprazan or another potassium-competitive acid blocker is an option for refractory cases. Peptic strictures develop frequently and are treated with serial endoscopic dilation. Coexistent gastroparesis is common and can worsen reflux by raising gastric residual volume, which is part of why prokinetic therapy gets considered. Barrett surveillance follows the standard intervals for non-dysplastic disease.
The piece of careful reasoning that misleads here is the temptation to offer an antireflux operation for severe refractory reflux, because the reflux looks like the dominant problem. Fundoplication fails in scleroderma because the pump that has to push a bolus past a wrap has failed first. A Nissen placed on absent peristalsis produces severe postoperative dysphagia. Partial wraps in carefully selected scleroderma patients are sometimes considered but are not the default, and magnetic sphincter augmentation and transoral incisionless fundoplication are not appropriate in this population. The recurring vignette is the woman with Raynaud, sclerodactyly, and severe reflux whose manometry shows absent peristalsis and a low LES pressure. The answer is twice-daily PPI, not Nissen.
Now to the other path, the elevated-IRP-with-preserved-peristalsis path that opens onto EGJ outflow obstruction. EGJOO is the heterogeneous bucket. The version four changes to the criteria reflect the recognition that the manometric pattern alone captures a large fraction of false positives and that the diagnosis on its own does not justify treatment.
The manometric criteria in version four have three pieces. First, an elevated median IRP in both supine and upright positions, with the Medtronic upright cutoff at twelve millimeters of mercury. Second, some preserved peristalsis with intact or weak swallows. Third, increased intrabolus pressure on at least twenty percent of swallows. The supine-plus-upright requirement is new and is doing real work. Supine IRP alone has a high false positive rate. In a representative study of radiographically confirmed EGJOO, an isolated upright IRP elevation had ninety-eight percent sensitivity but only sixteen percent specificity. A supine elevation that resolves upright is most often a positional artifact, often driven by intra-abdominal pressure in obesity, rather than true outflow obstruction. The intrabolus pressure requirement captures the compartmentalized pressure that real obstruction generates when the bolus is squeezed against an unrelaxing sphincter.
Beyond the manometric criteria, version four declared that an EGJOO diagnosis is always clinically inconclusive on its own. A clinically relevant EGJOO requires the manometric pattern plus symptoms, meaning dysphagia or chest pain, plus a confirmatory test demonstrating actual EGJ obstruction. There are two confirmatory tests. A timed barium esophagram with a barium tablet shows a retained barium column at one, two, and five minutes and impaired tablet passage when obstruction is real. The functional luminal imaging probe, FLIP, demonstrates reduced EGJ distensibility directly. The three-part requirement of manometry plus symptoms plus confirmation is the single most important practical change in version four for this diagnosis. It removes a large fraction of asymptomatic incidental EGJOO from the disease pool.
The differential for EGJOO is broad and every entry has to be considered before settling on a primary motor disorder. The opioid patient is the canonical mimic. Chronic opioid use raises the IRP, raises the DCI, and shortens the distal latency, producing a manometric phenotype that looks like EGJOO or type three achalasia or spasm or jackhammer depending on which parameter dominates. An opioid history in a patient with manometric EGJOO is the cue that opioid cessation is the first therapeutic test before any myotomy. A paraesophageal hiatal hernia produces mechanical narrowing at the EGJ and is identified on EGD and cross-sectional imaging. Eosinophilic esophagitis can produce fibrostenotic disease that impairs distensibility and elevates the IRP, and biopsies belong in every unexplained EGJOO workup. Infiltrative cancer at the EGJ produces pseudoachalasia or EGJOO depending on the degree of involvement, and the older patient with rapid weight loss should trigger endoscopic ultrasound and cross-sectional imaging. Obesity with intra-abdominal fat raises baseline IRP and is a known confounder. Post-fundoplication anatomy reliably elevates the IRP. An achalasia variant in evolution accounts for some cases, particularly type three patterns in which peristalsis is fragmenting but not yet absent.
The workup follows a specific sequence. EGD evaluates for mechanical narrowing, a mass at the EGJ, hiatal hernia anatomy, retained material suggestive of an achalasia variant, and takes biopsies for eosinophilic esophagitis. Cross-sectional imaging evaluates for extrinsic compression and submucosal pathology. EUS is added when suspicion for infiltrative malignancy is high. Timed barium esophagram with a tablet evaluates emptying and passage. FLIP evaluates distensibility directly. The opioid history triggers a trial of discontinuation. The rapid drink challenge during manometry itself, two hundred milliliters of water through a straw, can support the diagnosis. An abnormal result is an IRP over twelve millimeters of mercury with panesophageal pressurization in the first thirty seconds.
The functional luminal imaging probe deserves attention as the tool that version four leans on for several of these decisions. The FLIP catheter is passed transorally with the endoscope and positioned across the EGJ. A sixteen-centimeter cylindrical balloon with sixteen paired impedance planimetry electrodes is filled with conductive saline in stepwise volumes, typically up to sixty or seventy milliliters. The impedance between electrodes is converted to cross-sectional area at sixteen axial locations along the segment. The narrowest cross-sectional area is then divided by the simultaneous intraballoon pressure. That yields the distensibility index in millimeters squared per millimeter of mercury. The output is a real-time three-dimensional image of EGJ behavior during distension.
The Dallas Consensus thresholds are the numbers to carry. Normal EGJ opening requires a maximum EGJ diameter of at least sixteen millimeters and an EGJ distensibility index of at least two point zero millimeters squared per millimeter of mercury at sixty milliliters of fill. Reduced EGJ opening is a maximum diameter under twelve millimeters and a distensibility index under two point zero. Intermediate values are labeled inconclusive. Reduced opening supports outflow obstruction and supports achalasia in patients whose IRP did not quite cross the manometric threshold. The number to anchor on is two point zero for the distensibility index. The older literature sometimes referenced higher cutoffs, but the Dallas Consensus that informs current practice places the line at two point zero.
FLIP also delivers panometry, which reads the contractile response of the esophageal body during balloon distension. A normal contractile response is multiple distinct antegrade contractions, typically repetitive antegrade contractions occurring six plus or minus three times per minute over at least six centimeters of axial length. Abnormal contractile responses are categorized as absent, spastic, disordered, or diminished. Normal EGJ opening combined with normal contractile response makes a major motility disorder highly unlikely. Reduced EGJ opening combined with spastic or non-spastic obstruction patterns supports achalasia or clinically relevant EGJOO.
FLIP rescues several specific diagnostic problems. A patient with classic achalasia symptoms whose IRP comes back at fourteen on supine swallows, just under the threshold, can be confirmed as achalasia by reduced EGJ opening on FLIP. A patient who meets version four manometric criteria for EGJOO can be confirmed or refuted by FLIP distensibility, with reduced opening supporting clinically relevant obstruction and normal opening pointing back toward observation. A high-resolution manometry study that fails because of artifact or intolerance can be addressed with FLIP performed during the same EGD without a separate visit. FLIP is also used intraoperatively during POEM and Heller myotomy to titrate the myotomy length, with normalization of the distensibility index correlating with better post-procedure symptom outcomes.
Treatment of confirmed clinically relevant EGJOO depends on the suspected mechanism. EGJOO with hypercontractile or spastic body features, confirmed by abnormal FLIP and abnormal barium emptying, is managed as a type three achalasia variant, and POEM with a longer myotomy is generally preferred. EGJOO with normal or weak peristalsis and mild symptoms can be observed with PPI and dietary modification. Botulinum toxin or pneumatic dilation is reserved for disabling symptoms. Mechanical causes are treated for the cause. Opioid-induced EGJOO resolves with opioid cessation, and if cessation is not feasible, management is symptomatic rather than procedural.
So the through-line for this episode is that once the integrated relaxation pressure has excluded achalasia, the manometry reads cleanly on two questions. What is the body doing on a normal IRP, and what is the body doing on an elevated IRP. On a normal IRP, premature contractions with a short distal latency are distal esophageal spasm, hypercontractile swallows with a DCI over eight thousand are jackhammer, and the medical ladder treats both. Failed peristalsis on a normal IRP sorts on the LES. A normal LES with weak or failed swallows is ineffective motility or absent contractility, where the management is about reflux and about whether a fundoplication is safe. A hypotensive LES with failed peristalsis is scleroderma, where twice-daily PPI is the answer and Nissen is the wrong answer. On an elevated IRP with preserved peristalsis, EGJOO is a manometric starting point that needs symptoms and a confirmatory test. FLIP and timed barium do the confirming, and they also rescue the borderline achalasia case in which the IRP did not quite cross.
That hands us off to chapter three. The reflux that scleroderma drives is the severe end of a disease that starts as a barrier failure long before it becomes an acid problem. The LA grading on EGD stratifies treatment intensity and Barrett risk, with PPI timing and vonoprazan closing out the acid suppression ladder.