Narrative reviews
Advances in Management of Esophageal Motility Disorders

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The widespread adoption of high-resolution manometry (HRM) has led to a restructuring in the classification of esophageal motility disorder classification summarized in the Chicago Classification, currently in version 3.0. It has become apparent that the cardinal feature of achalasia, impaired lower esophageal sphincter relaxation, can occur in several disease phenotypes: without peristalsis, with premature (spastic) distal esophageal contractions, with panesophageal pressurization, or even with preserved peristalsis. Furthermore, despite these advances in diagnostics, no single manometric pattern is perfectly sensitive or specific for idiopathic achalasia and complimentary assessments with provocative maneuvers during HRM or interrogating the esophagogastric junction with the functional luminal imaging probe during endoscopy can be useful in clarifying equivocal or inexplicable HRM findings. Using these tools, we have come to conceptualize esophageal motility disorders as characterized by obstructive physiology at the esophagogastric junction, smooth muscle esophagus, or both. Recognizing obstructive physiology as a primary target of therapy has become particularly relevant with the development of a minimally invasive technique for performing a calibrated myotomy of the esophageal circular muscle, the POEM procedure. Now and going forward, optimal management is to render treatment in a phenotype-specific manner: e.g. POEM calibrated to patient-specific physiology for spastic achalasia and spastic disorders of the smooth muscle esophagus, more conservative strategies (pneumatic dilation) for the disorders limited to the sphincter.

Section snippets

A New Perspective on Esophageal Motility Disorders: Obstructive Physiology

The CC of esophageal motility disorders was built around 3 key metrics derived from pressure topography plots: the integrated relaxation pressure (IRP), the distal contractile integral (DCI), and the distal latency (DL).1 From the beginning of the CC, it was proposed that the analysis of HRM studies be hierarchical, beginning at the EGJ and proceeding proximally. This was in recognition of the fundamental importance of outflow obstruction, manifest by an IRP greater than the upper limit of

Clarifying the Gray Zones: Provocative Stimuli and Ancillary Tests

Among potential findings in HRM studies, the detection of obstructive physiology at the EGJ is the most fundamental because it is ultimately the best therapeutic target. In the CC this is based on detection of an elevated IRP. However, no metric or technology has perfect sensitivity and specificity for detecting relevant sphincter dysfunction, and in marginal or atypical cases one has to consider all available evidence, including other studies and other metrics. There are clearly cases of

A New Perspective on Management: Phenotype-Directed Treatment

The ideal therapy for an esophageal motility disorder would revert swallow function to normal, render the patient symptom free, and not result in pathologic reflux. No current therapy for any of the esophageal motility disorders achieves all of these objectives, and that is unlikely to change in the foreseeable future. Furthermore, the controlled treatment data currently available lag well behind recent advances in diagnostics. Indeed, although the original description of achalasia phenotypes

Esophagogastric Junction Outflow Obstruction

In addition to the 3 subtypes of achalasia, CC v3.0 recognizes EGJ outflow obstruction as another entity characterized by EGJ obstructive physiology. With this entity, the IRP is greater than the upper limit of normal, but there is fragmented or even normal peristalsis such that criteria for achalasia are not met. From its initial description, EGJ outflow obstruction was reported to be a heterogeneous group, only some of whom benefitted from achalasia treatments.15 Potential etiologies include

Conclusions

High-resolution manometry and the CC have led to a major restructuring in the classification of esophageal motility disorders. Along with this has come the recognition that a defining feature of the major esophageal motility disorders is obstructive physiology, whether the EGJ, the distal esophagus, or both (Figure 1). Although the CC has helped crystallize esophageal motility diagnoses, especially the varied phenotypes of achalasia, it has also led to the realization that there are

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    Conflicts of interest These authors disclose the following: Albert J. Bredenoord received research funding from Medtronic and educational and research funding from MMS. John E. Pandolfino received consulting and educational fees from Medtronic, Sandhill, and Torax and stock options from Crospon. The remaining authors disclose no conflicts.

    Funding Peter J. Kahrilas and John E. Pandolfino were supported by R01 DK079902 (J.E.P.) from the U.S. Public Health Service.

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