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Management of Esophageal Motility Disorders: A Practical Guide
Abstract
The diagnosis of esophageal motility disorders can be difficult and complex. Patients generally present with dysphagia and chest pain and may have associated gastroesophageal reflux disease (GERD). Imaging and endoscopy are the first steps in determining the accurate diagnosis and help to rule out other etiologies, such as esophageal cancer. The esophageal motility disorder is defined by the findings on high-resolution manometry. Recent changes to the Chicago Classification of esophageal motility disorders highlight new terminology, which includes distal esophageal spasm, absent esophageal contractility, and jackhammer esophagus. Treatment is then tailored for the specific classification. Achalasia, which falls under the category of esophageal motility disorders, will be discussed in a separate chapter.
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The best-defined primary esophageal motor disorder is achalasia. However, symptoms such as dysphagia, regurgitation and chest pain can be caused by other esophageal motility disorders. The Chicago classification introduced new manometric parameters and better defined esophageal motility disorders. Motility disorders beyond achalasia with the current classification are: esophagogastric junction outflow obstruction, major disorders of peristalsis (distal esophageal spasm, hypercontractile esophagus, absent contractility) and minor disorders of peristalsis (ineffective esophageal motility, fragmented peristalsis). The aim of this study was to review the current diagnosis and management of esophageal motility disorders other than achalasia.
With the development of high-resolution manometry and specific metrics to characterize esophageal motility, the Chicago Classification has become the gold standard for the diagnosis of esophageal motor disorders. Major and significant disorders, that is, never observed in healthy subjects, are achalasia, esophagogastric junction outflow obstruction, distal esophageal spasm, absent peristalsis, and hypercontractile (Jackhammer) esophagus. Achalasia subtyping is relevant to predict the response to endoscopic and surgical therapies as several studies suggest that, pneumatic dilation is less effective than Heller myotomy, in type III achalasia. Peroral endoscopic myotomy, initially developed in expert centers, is a promising technique for the treatment of achalasia. The medical therapeutic options for distal esophageal spasm and hypercontractile esophagus are smooth muscle relaxants and pain modulators. Intraesophageal injection of botulinum toxin might be an interesting option for treatment of these disorders but further studies are required to determine the optimal injection protocol and the best candidates based on manometric patterns. The treatment of hypotensive motility disorders is disappointing and relies mainly on dietary and lifestyle changes as no effective esophageal prokinetic is currently available.
The Chicago Classification (CC) of esophageal motility disorders, utilizing an algorithmic scheme to analyze clinical high-resolution manometry (HRM) studies, has gained acceptance worldwide.
This 2014 update, CC v3.0, developed by the International HRM Working Group, incorporated the extensive clinical experience and interval publications since the prior (2011) version.
Chicago Classification v3.0 utilizes a hierarchical approach, sequentially prioritizing: (i) disorders of esophagogastric junction (EGJ) outflow (achalasia subtypes I-III and EGJ outflow obstruction), (ii) major disorders of peristalsis (absent contractility, distal esophageal spasm, hypercontractile esophagus), and (iii) minor disorders of peristalsis characterized by impaired bolus transit. EGJ morphology, characterized by the degree of overlap between the lower esophageal sphincter and the crural diaphragm and baseline EGJ contractility are also part of CC v3.0. Compared to the previous CC version, the key metrics of interpretation, the integrated relaxation pressure (IRP), the distal contractile integral (DCI), and the distal latency (DL) remain unchanged, albeit with much more emphasis on DCI for defining both hypo- and hypercontractility. New in CC v3.0 are: (i) the evaluation of the EGJ at rest defined in terms of morphology and contractility, (ii) 'fragmented' contractions (large breaks in the 20-mmHg isobaric contour), (iii) ineffective esophageal motility (IEM), and (iv) several minor adjustments in nomenclature and defining criteria. Absent in CC v3.0 are contractile front velocity and small breaks in the 20-mmHg isobaric contour as defining characteristics.
Chicago Classification v3.0 is an updated analysis scheme for clinical esophageal HRM recordings developed by the International HRM Working Group.
© 2014 John Wiley & Sons Ltd.
Diffuse esophageal spasm is a primary esophageal motility disorder. The prevalence is 3-10% in patients with dysphagia and treatment options are limited. This review summarizes the treatment of diffuse esophageal spasm, including pharmacotherapy, endoscopic treatment, and surgical treatment with a special focus on botulinum toxin injection. A PubMed search was performed to identify the literature using the search items diffuse esophageal spasm and treatment. Pharmacotherapy with smooth muscle relaxants, proton pump inhibitors, and antidepressants was suggested from small case series and uncontrolled clinical trials. Endoscopic injection of botulinum toxin is a well-studied treatment option and results in good symptomatic benefit in patients with diffuse esophageal spasm. Surgical treatment was reported in patients with very severe symptoms refractory to pharmacologic treatment. This article summarizes the present knowledge on the treatment of diffuse esophageal spasm with a special emphasis on botulinum toxin injection. Endoscopic injection of botulinum toxin is presently the best studied treatment option but many questions remain unanswered.
Gastroesophageal reflux disease (GERD) produces a spectrum of symptoms ranging from mild to severe. While the role of the lower esophageal sphincter in the pathogenesis of GERD has been studied extensively, less attention has been paid to esophageal peristalsis, even though peristalsis governs esophageal acid clearance. The aim of this study was to evaluate the following in patients with GERD: (1) the nature of esophageal peristalsis and (2) the relationship between esophageal peristalsis and gastroesophageal reflux, mucosal injury, and symptoms. One thousand six consecutive patients with GERD confirmed by 24-hour pH monitoring were divided into three groups based on the character of esophageal peristalsis as shown by esophageal manometry: (1) normal peristalsis (normal amplitude, duration, and velocity of peristaltic waves); (2) ineffective esophageal motility (IEM; distal esophageal amplitude < 30 mm Hg or >30% simultaneous waves); and (3) nonspecific esophageal motility disorder (NSEMD; motor dysfunction intermediate between the other two groups). Peristalsis was classified as normal in 563 patients (56%), IEM in 216 patients (21%), and NSEMD in 227 patients (23%). Patients with abnormal peristalsis had worse reflux and slower esophageal acid clearance. Heartburn, respiratory symptoms, and mucosal injury were all more severe in patients with IEM. These data show that esophageal peristalsis was severely impaired (IEM) in 21% of patients with GERD, and this group had more severe reflux, slower acid clearance, worse mucosal injury, and more frequent respiratory symptoms. We conclude that esophageal manometry and pH monitoring can be used to stage the severity of GERD, and this, in turn, should help identify those who would benefit most from surgical treatment.
The role of surgery in the management of patients with diffuse oesophageal spasm (DOS) remains controversial. The aim of this study was to assess functional results after extended myotomy for DOS.
This prospective study evaluated 20 patients who had extended myotomy (14 cm on the oesophagus and 2 cm below the oesophagogastric junction) with anterior fundoplication via a laparotomy for severe DOS. Median follow-up was 50 (range 6-84) months. Functional data were assessed by means of dysphagia (range 0-3), chest pain (range 0-3) and overall clinical (range 0-12, including dysphagia, chest pain, regurgitation, gastro-oesophageal reflux) scores.
All patients had severe DOS. The median preoperative overall clinical score was 6 (range 3-8) with a dysphagia score of at least 2. Median postoperative functional scores were significantly lower than preoperative values (overall clinical score 1 versus 6, dysphagia score 0 versus 3, chest pain score 0 versus 2). At final follow-up, good or excellent results were obtained for overall clinical score in 16 patients, for dysphagia score in 18 and for chest pain score in all 20 patients. Postoperative gastro-oesophageal reflux was noted in two of the 20 patients.
Extended myotomy with anterior fundoplication is an effective treatment for severe DOS. Medium-term postoperative functional results were excellent, especially in terms of dysphagia and chest pain.