Chicago Classification Update (v4.0): Technical review on diagnostic criteria for distal esophageal spasm.

Distal esophageal spasm (DES) is defined as a manometric pattern of at least 20% of premature contractions in a context of normal esophago-gastric junction relaxation in a patient with dysphagia or non-cardiac chest pain. The definition of premature contraction requires the measurement of the distal latency and identification of the contractile deceleration point (CDP). The CDP can be difficult to localize, and alternative methods are proposed. Further, it is important to differentiate contractile activity and intrabolus pressure. Multiple rapid swallows are a useful adjunctive test to perform during high-resolution manometry to search for a lack of inhibition that is encountered in DES. The clinical relevance of the DES-manometric pattern was raised as it can be secondary to treatment with opioids or observed in patients referred for esophageal manometry before antireflux surgery in absence of dysphagia and non-cardiac chest pain. Further idiopathic DES is rare, and one can argue that when encountered, it could be part of type III achalasia spectrum. Medical treatment of DES can be challenging. Recently, endoscopic treatments with botulinum toxin and peroral endoscopic myotomy have been evaluated, with conflicting results while rigorously controlled studies are lacking. Future research is required to determine the role of contractile vigor and lower esophageal sphincter hypercontractility in the occurrence of symptoms in patients with DES. The role of impedance-combined high-resolution manometry also needs to be evaluated.

Esophageal Motility Disorders.

Esophageal motility disorders can cause chest pain, heartburn, or dysphagia. They are diagnosed based on specific patterns seen on esophageal manometry, ranging from the complete absence of contractility in patients with achalasia to unusually forceful or disordered contractions in those with hypercontractile motility disorders. Achalasia has objective diagnostic criteria, and effective treatments are available. Timely diagnosis results in better outcomes. Recent research suggests that hypercontractile motility disorders may be overdiagnosed, leading to unnecessary and irreversible interventions. Many symptoms ascribed to these disorders are actually due to unrecognized functional esophageal disorders. Hypercontractile motility disorders and functional esophageal disorders are generally self-limited, and there is considerable overlap among their clinical features. Endoscopy is warranted in all patients with dysphagia, but testing to evaluate for less common conditions should be deferred until common conditions have been optimally managed. Opioid-induced esophageal dysmotility is increasingly prevalent and can mimic symptoms of other motility disorders or even early achalasia. Dysphagia of liquids in a patient with normal esophagogastroduodenoscopy findings may suggest achalasia, but high-resolution esophageal manometry is required to confirm the diagnosis. Surgery and advanced endoscopic therapies have proven benefit in achalasia. However, invasive interventions are rarely indicated for hypercontractile motility disorders, which are typically benign and usually respond to lifestyle modifications, although pharmacotherapy may occasionally be needed.

Distal esophageal spasm.

PURPOSE OF REVIEW: Distal esophageal spasm (DES) is a rare esophageal motility disorder associated with dysphagia and chest pain. In 2011, the diagnosis of DES was refined based on the occurrence of premature (rather than rapid) contractions by high-resolution manometry. New therapeutic options have also been recently proposed. Thus, a review on DES incorporating publications since 2012 is timely because of these revisions in definition and management. RECENT FINDINGS: DES remains a heterogeneous clinical disorder. Its pathophysiology is still debated and DES might be related to achalasia. Alternatively, it might be secondary to medications, especially opiates. Endoscopic ultrasound might be informative diagnostically by demonstrating muscularis propria hypertrophy and thickening. Botulinum toxin injection in the esophageal body has been shown superior to placebo to relieve symptoms associated with DES. Finally, per oral endoscopic myotomy is a promising therapeutic approach, but may be less effective in DES than in achalasia. SUMMARY: The diagnosis of DES should lead to a systematic search for medication that might promote the occurrence of esophageal dysmotility. Endoscopic treatment of DES (botulinum toxin injection or per oral endoscopic myotomy) should be further evaluated in controlled studies using current diagnostic criteria by high-resolution manometry.

International multicenter experience with peroral endoscopic myotomy for the treatment of spastic esophageal disorders refractory to medical therapy (with video).

BACKGROUND: Limited data exist on the use of peroral endoscopic myotomy (POEM) for therapy of spastic esophageal disorders (SEDs). OBJECTIVE: To study the efficacy and safety of POEM for the treatment of patients with diffuse esophageal spasm, jackhammer esophagus, or type III (spastic) achalasia. DESIGN: Retrospective study. SETTING: International, multicenter, academic institutions. PATIENTS: All patients who underwent POEM for treatment of SEDs refractory to medical therapy at 11 centers were included. INTERVENTIONS: POEM. MAIN OUTCOME MEASUREMENTS: Eckardt score and adverse events. RESULTS: A total of 73 patients underwent POEM for treatment of SEDs (diffuse esophageal spasm 9, jackhammer esophagus 10, spastic achalasia 54). POEM was successfully completed in all patients, with a mean procedural time of 118 minutes. The mean length of the submucosal tunnel was 19 cm, and the mean myotomy length was 16 cm. A total of 8 adverse events (11%) occurred, with 5 rated as mild, 3 moderate, and 0 severe. The mean length of hospital stay was 3.4 days. There was a significant decrease in Eckardt scores after POEM (6.71 vs 1.13; P = .0001). Overall, clinical response was observed in 93% of patients during a mean follow-up of 234 days. Chest pain significantly improved in 87% of patients who reported chest pain before POEM. Repeat manometry after POEM was available in 44 patients and showed resolution of initial manometric abnormalities in all cases. LIMITATIONS: Retrospective design and selection bias. CONCLUSION: POEM offers a logical therapeutic modality for patients with SEDs refractory to medical therapy. Results from this international study suggest POEM as an effective and safe platform for these patients.

Esophageal hypomotility and spastic motor disorders: current diagnosis and treatment.

Esophageal hypomotility (EH) is characterized by abnormal esophageal peristalsis, either from a reduction or absence of contractions, whereas spastic motor disorders (SMD) are characterized by an increase in the vigor and/or propagation velocity of esophageal body contractions. Their pathophysiology is not clearly known. The reduced excitation of the smooth muscle contraction mediated by cholinergic neurons and the impairment of inhibitory ganglion neuronal function mediated by nitric oxide are likely mechanisms of the peristaltic abnormalities seen in EH and SMD, respectively. Dysphagia and chest pain are the most frequent clinical manifestations for both of these dysfunctions, and gastroesophageal reflux disease (GERD) is commonly associated with these motor disorders. The introduction of high-resolution manometry (HRM) and esophageal pressure topography (EPT) has significantly enhanced the ability to diagnose EH and SMD. Novel EPT metrics in particular the development of the Chicago Classification of esophageal motor disorders has enabled improved characterization of these abnormalities. The first step in the management of EH and SMD is to treat GERD, especially when esophageal testing shows pathologic reflux. Smooth muscle relaxants (nitrates, calcium channel blockers, 5-phosphodiesterase inhibitors) and pain modulators may be useful in the management of dysphagia or pain in SMD. Endoscopic Botox injection and pneumatic dilation are the second-line therapies. Extended myotomy of the esophageal body or peroral endoscopic myotomy (POEM) may be considered in highly selected cases but lack evidence.

Management of spastic disorders of the esophagus.

The concept of esophageal spastic disorders encompasses spastic achalasia, distal esophageal spasm, and jackhammer esophagus. These are conceptually distinct in that spastic achalasia and distal esophageal spasm are characterized by a loss of neural inhibition, whereas jackhammer esophagus is associated with hypercontractility. Hypercontractility may also occur as a result of esophagogastric junction outflow obstruction or inflammation. The diagnosis of jackhammer esophagus as a primary motility disorder is based on the characteristic manometric findings after ruling out mechanical obstruction and eosinophilic esophagitis. Despite the differences in pathophysiology among the esophageal spastic disorders, their management is similar.

Esophageal spasm: demographic, clinical, radiographic, and manometric features in 108 patients.

Diffuse esophageal spasm (DES) remains insufficiently understood. Here we aimed to summarize the demographic, clinical, radiographic, and manometric features in a large cohort of patients with DES. We identified all consecutive patients diagnosed with DES from 2000 to 2006 at Mayo Clinic Florida. The computerized records of these patients were reviewed to extract relevant information. We performed 2654 esophageal motilities during that period. There were 108 patients with esophageal spasm, and 55% were female. Median age was 71 years. The most common leading symptom was dysphagia in 55, followed by chest pain in 31. Weight loss occurred in 28 patients. The median of time from onset of symptoms to diagnosis was 48 months (range 0-480), with a median of time from the first medical consultation to diagnosis of 8 months (range 0-300). The most frequent comorbidities were hypertension and psychiatric problems. At presentation, 81 patients were taking acid-reducing medications, and 49 patients were taking psychotropic drugs. An abnormal esophagogram was noted in 46 of 76 patients with this test available, but most radiographic findings were nonspecific with the typical 'corkscrew' appearance seen in only three patients. Gastroesophageal reflux disease (GERD) was diagnosed by pH testing or endoscopy in 41 patients. We did not find any difference between the rate of simultaneous contractions or esophageal amplitude between patients with a leading symptom of dysphagia and those with chest pain. DES is an uncommon motility disorder that often goes unrecognized for years. Physicians should be aware of the clinical heterogeneity of DES and consider motility testing early in the course of unexplained esophageal symptoms. Given the high prevalence of GERD in DES, the role of GERD and the impact of acid-reducing therapy in DES deserve further study.

Diffuse esophageal spasm: the surgical approach.

Diffuse esophageal spasm (DES) is a rare primary motility disorder of unknown cause, that can be found in patients complaining of chest pain and dysphagia and in whom ischemic heart disease and GERD have been excluded. The manometric hallmark of DES is the presence of simultaneous contractions in the distal esophagus alternating with a normal peristalsis. Even at specialized esophageal motility laboratories, DES is considered an uncommon diagnosis. In this review, the authors discuss the clinical and diagnostic aspects of this disease, as well as the possible therapeutic options (medical, endoscopic or surgical therapy). Surgery (esophageal myotomy performed through a thoracotomy or with a thoracoscopic access) seems to have a better outcome than medical or endoscopic treatment, and it is considered "the last resource" in these patients. However, satisfactory results are reported, from highly skilled centers, in only about 70% of treated cases, certainly inferior to those achieved in other esophageal disorders. The role of surgery in this disease requires therefore further study, even if controlled trials are probably difficult to perform, due to the rarity of the disease.

[Benign esophageal disorders. Gastroesophageal reflux disease, diffuse esophageal spasm, achalasia]. / Benigne Erkrankungen des Ösophagus. Refluxkrankheit, diffuser Ösophagusspasmus, Achalasie.

Gastroesophageal reflux disease (GERD) is the most frequent benign disorder of the upper gastrointestinal (GI) tract and other defined disease entities, such as achalasia and diffuse esophageal spasm, also belong to this group. In addition to surgical therapy, medicinal therapy also has an important role in all 3 of these disorders. Therefore, it is very important to follow precise indication criteria based on diagnostic evaluation and patient selection as well as to use an optimal operative technique.The therapeutic spectrum for achalasia varies from Botox injections and endoscopic dilatation to laparoscopic myotomy which achieves a success rate up to 90%.Patients with diffuse spasm suffer from severe dysphagia, thoracic pain and burning sensations and even respiratory problems. Surgical therapy consists of thoracoscopic long myotomy and in selective cases with persisting pain even esophagectomy and gastric pull-up.Therapeutic options for GERD predominantly involve conservative medicinal therapy with proton pump inhibitors and selective laparoscopic antireflux procedures. Minimally invasive techniques have led to a higher acceptance of surgical therapy. The two major procedures most frequently used are total Nissen fundoplication and posterior partial Toupet fundoplication.

Botulinum toxin in the treatment of diffuse esophageal spasm.

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.

Use of computerized manometry for the detection of pharyngoesophageal spasm in tracheoesophageal speech.

OBJECTIVE: To evaluate the utility of computerized manometry (CM) to identify pharyngoesophageal segment (PES) spasm during tracheoesophageal speech. STUDY DESIGN: Prospective clinical, controlled study. SUBJECTS AND METHODS: Intraluminal pressures of the PES were collected in 12 tracheoesophageal speakers without spasm and 8 tracheoesophageal speakers with PES spasm before and after localized injection of botulinum toxin to the PES. All subjects underwent voice analysis and videofluoroscopy in addition to CM before and after treatment. RESULTS: All tracheoesophageal speakers with PES spasm presented with mean intraluminal pressures greater than 16 mmHg (mean, 25.36 mmHg). In contrast, mean intraluminal pressures of subjects without spasm was 11.76 mmHg (P<0.05). The negative predictive value associated with the use of 16 mmHg as a threshold value for spasm was 100%. CONCLUSION: CM is a clinically useful tool to aid in speech rehabilitation for tracheoesophageal speakers. Intraluminal pressures of greater than 16 mmHg was highly predictive for PES spasm.

A comprehensive appraisal of the surgical treatment of diffuse esophageal spasm.

Diffuse esophageal spasm is a motility disorder of undetermined cause. The optimal treatment remains controversial, and evidence-based data are lacking. Several medical treatment modalities have been proposed, but none has emerged as the treatment of choice. Patients who do not respond to medical therapy may be considered for surgical treatment. The surgical treatment of diffuse esophageal spasm is based on similar principles to the treatment of achalasia. A long esophageal myotomy is done to divide the hypertrophied circular muscle that is frequently noted in diffuse esophageal spasm. To protect against postoperative reflux, an antireflux procedure may be added. However, the surgical treatment of diffuse esophageal spasm has not been subjected to randomized clinical trials. The purpose of this article is to provide a review of the available literature regarding the surgical management of the diffuse esophageal spasm. In particular, we offer an appraisal of surgical outcomes, the effects of surgery on manometric and radiologic parameters (when available), complications, and mortality.

Surgical treatment of primary esophageal motility disorders.

Named primary esophageal motility disorders (PEMD) present with specific manometric patterns classified as: (1) hypertensive lower esophageal sphincter, (2) nutcracker esophagus (also hypercontratile, hypertensive, or hypercontracting esophagus), (3) diffuse esophageal spasm, and (4) achalasia. These conditions, with the exception of achalasia, are rare, poorly understood, and inadequately studied. Treatment of these conditions is based on symptoms and aimed at symptomatic improvement. The authors reviewed current literature on surgical treatment of non-achalasia PEMD. The review shows that: (a) surgical therapy may be an attractive alternative in patients with PEMD; (b) proper selection of patients based on symptoms evaluation and esophageal function tests is essential; (c) laparoscopic myotomy with proximal extent tailored to manometric findings seems to be the ideal surgical therapy; and (d) esophagectomy may be necessary as a last resource due to multiple failures of surgical conservative treatment.

Successful surgical treatment for diffuse esophageal spasm.

A 74-year-old man, who had previously received curative distal gastrectomy for gastric cancer, was admitted to our hospital with severe dysphagia and weight loss. Barium swallow examination revealed the esophagus to have the corkscrew appearance characteristic of diffuse esophageal spasm (DES). This diagnosis was confirmed by esophageal manometry, which revealed intermittent, simultaneous, high-amplitude (30-100 mmHg) contractions after 65% of wet swallows. The muscle layer was also found to be thickened throughout the spastic region. Long esophagomyotomy with fundoplication was performed after treatment with medication proved ineffective. Myotomy proceeded superiorly to the area under aortic arch and inferiorly 3 cm into the cardiac portion. Fluoroscopy of the esophagus after the operation showed the spastic changes to be absent, and the patient showed improved clinical signs. We therefore recommend long myotomy of the esophageal wall with antireflux surgery for DES with sever dysphagia that is resistant to conservative treatment.

Long myotomy of the esophagus and gastric cardia with a complete fundic patch procedure for diffuse esophageal spasm.

Under the hypothesis that the surgical management of diffuse esophageal spasm requires the elimination or reduction of episodes of dysphagia and chest pain and prevention of postoperative gastroesophageal reflux, long esophageal myotomy and fundoplication had been performed. However, there have been some cases with unsatisfactory results. We describe herein a new surgical procedure of long myotomy of the esophagus and gastric cardia with a complete fundic patch operation for the patient with diffuse esophageal spasm. The advantages of this procedure are to preserve the separation of each myotomized edge and to reinforce the wall of the surface of the myotomized mucosa in order to avoid the postoperative problems. Postoperative course of the patient with this procedure was satisfactory.