Achalasia is a rare disease of the esophagus with impaired relaxation of the lower esophageal sphincter and aperistalsis. The etiology is unknown but speculations include a viral or autoimmune etiology. All specialists dealing with swallowing and esophageal diseases should recognize the classic symptoms of dysphagia for solids/liquids, regurgitation, and choking, especially at night. High-resolution manometry is critical for the diagnosis with endoscopy and barium esophagram having a supportive role. The disease cannot be cured but most can return to near normal swallowing and a regular diet with appropriate therapy. Treatment includes smooth muscle relaxants, botulinum toxin injections to the lower sphincter, pneumatic dilation, Heller myotomy, and peroral endoscopic myotomy. One treatment does not fit all and a tailored approach through a multidiscipline team will give the best long-term outcomes.

Achalasia cardia (AC) is a frequently encountered motility disorder of the esophagus resulting from an irreversible degeneration of neurons. Treatment modalities are palliative in nature, and there is no curative treatment available for AC as of now. Significant advancements have been made in the management of AC over last decade. The introduction of high resolution manometry and per-oral endoscopic myotomy (POEM) has strengthened the diagnostic and therapeutic armamentarium of AC. High resolution manometry allows for the characterization of the type of achalasia, which in turn has important therapeutic implications. The endoscopic management of AC has been reinforced with the introduction of POEM that has been found to be highly effective and safe in palliating the symptoms in short-term to mid-term follow-up studies. POEM is less invasive than Heller's myotomy and provides the endoscopist with the opportunity of adjusting the length and orientation of esophageal myotomy according to the type of AC. The management of achalasia needs to be tailored for each patient, and the role of pneumatic balloon dilatation, POEM, or Heller's myotomy needs to be revisited. In this review, we discuss the important aspects of diagnosis as well as management of AC. The statements presented in the manuscript reflect the cumulative efforts of an expert consensus group.

The treatment options in achalasia patients aim to improve symptoms by reducing the functional obstruction at the level of the gastroesophageal junction. Available treatment modalities are endoscopic botulinum toxin injection (EBTI), pneumatic dilatation (PD), laparoscopic Heller myotomy (LHM), and peroral endoscopic myotomy (POEM). We provide an evidence-based review of current indications, limitations, and future perspectives of these options for the treatment of achalasia.

The PubMed/Medline electronic databases and the Cochrane Library were searched. Quality of evidence was assessed according to the GRADE system.

Functional outcomes after EBTI are significantly worse than those after PD or LHM. LHM with partial fundoplication is associated with low complication rates and provides excellent long-term results with lower need for additional treatment of recurrent dysphagia than PD. POEM is a new promising treatment option with good short-term outcomes and low morbidity in experienced hands.

LHM should be considered the procedure of choice for the treatment of achalasia in patients who are fit for surgery. Large randomized controlled trials with long follow-up are needed to validate the role of POEM.

High-resolution manometry enables the categorization of patients with oesophageal dysphagia caused by oesophageal motility disorders into diagnostic categories according to the Chicago Classification. This Review provides an overview of the literature concerning treatment options for patients with dysphagia associated with achalasia, hypercontractile disorders and hypocontractility disorders of the oesophagus. In achalasia, pharmacotherapy and botulinum toxin are less effective than pneumatic dilation or surgical Heller myotomy, which had comparable efficacy in the largest controlled trial to date. Peroral endoscopic myotomy is a novel therapeutic modality that is currently being evaluated in controlled trials versus pneumatic dilation or surgical myotomy. A variety of medical treatments have been evaluated in hypermotility disorders, but only botulinum toxin injection yielded favourable results in a single controlled trial. Few studies have addressed the treatment of dysphagia in patients with oesophageal hypomotility. A variety of prokinetic agents have been studied, but there is no evidence of clinically relevant efficacy from controlled trials.

Comparison of short- and long-term effects after laparoscopic Heller myotomy (LHM) and endoscopic balloon dilation (EBD) considering the need for retreatment.

Previously published studies have indicated that LHM is the most effective treatment for Achalasia. In contrast to that a recent randomized trial found EBD equivalent to LHM 2 years after initial treatment.

A search in Medline, PubMed, and Cochrane Central Register of Controlled Trials was conducted for prospective studies on interventional achalasia therapy with predefined exclusion criteria. Data on success rates after the initial and repeated treatment were extracted. An adjusted network meta-analysis and meta-regression analysis was used, combined with a head-to-head comparison, for follow-up at 12, 24, and 60 months.

Sixteen studies including results of 590 LHM and EBD patients were identified. Odds ratio (OR) was 2.20 at 12 months (95% confidence interval: 1.18-4.09; P = 0.01); 5.06 at 24 months (2.61-9.80; P < 0.00001) and 29.83 at 60 months (3.96-224.68; P = 0.001). LHM was also significantly superior for all time points when therapy included re-treatments [OR = 4.83 (1.87-12.50), 19.61 (5.34-71.95), and 17.90 (2.17-147.98); P ≤ 0.01 for all comparisons) Complication rates were not significantly different. Meta-regression analysis showed that amount of dilations had a significant impact on treatment effects (P = 0.009). Every dilation (up to 3) improved treatment effect by 11.9% (2.8%-21.8%).

In this network meta-analysis, LHM demonstrated superior short- and long-term efficacy and should be considered first-line treatment of esophageal achalasia.

Pneumatic dilation has re-emerged as a first line treatment for achalasia, but conclusions are limited by the relatively small numbers of patients studied and the lack of long term follow-up.

To summarise and analyse 29 available studies evaluating pneumatic dilation for achalasia with focus on efficacy, rate or perforation and dilation technique.

A literature search for all studies, in which pneumatic dilation was performed for treatment of achalasia, was conducted. Studies, in which clear endpoints of efficacy of single dilation sessions over a period of years, were chosen.

The response for a single dilation session was 66% at 1 year and 59, 53, 50 and 25% at 2, 3, 5 and 10 years respectively. Use of a Rigiflex dilator and multiple dilations during the initial treatment improved efficacy. Overall perforation rate was only 2% (24/1358) of which only 1% required surgery. Use of multiple dilations led to increased perforation risk. The method of dilation used with regard to balloon size, pressure used, dilation times and single or multiple dilations varied in almost every study.

Pneumatic dilation is safer than commonly thought and efficacious, although multiple dilations will be needed over a lifetime in most patients. Standardisation of the technique should be attempted.

Botulinum toxin injection into the lower esophageal sphincter (LES) treats dysphagia syndromes with preserved peristalsis and incomplete LES relaxation (LESR). We evaluated clinical and esophageal motor characteristics predicting response, and compared duration of efficacy to similarly treated achalasia patients.

Thirty-six subjects (59 ± 2.2 years, 19F/17M) with incomplete LESR on high resolution manometry (HRM) treated with botulinum toxin injection were identified. Individual and composite symptom indices were calculated, and HRM characteristics extracted. Symptom resolution for 6 months was a primary outcome measure, and repeat botulinum toxin injection, dysphagia recurrence or employment of alternate therapeutic approaches were secondary outcome measures. Duration of response was compared using Kaplan-Meier survival curves to a historical cohort of similarly treated achalasia subjects.

Response lasted a mean of 12.8 ± 2.3 months. Symptom relief for >6 months was seen in 58.3%; short (<6 months) response was associated with younger age, higher chest pain index, and esophageal body spastic features (P ≤ 0.04). On multivariate logistic regression, chest pain, younger age and contraction amplitudes >180 mmHg independently predicted <6 months relief (P < 0.05 for each). On survival analysis, relief with a single injection extended to 1 year in 54.8% and 1.5 years in 49.8%, statistically equivalent to that reported by 42 similarly treated achalasia subjects (59 ± 3.2 years, 24F/18M). Symptom relief was more prolonged compared to achalasia when repeat injections were performed on demand (P = 0.003).

Botulinum toxin injections can provide lasting symptom relief in dysphagia syndromes with incomplete LESR. Prominent perceptive symptoms and non-specific spastic features may predict shorter relief.

Although rare, esophageal achalasia is the best described primary esophageal motility disorder. Commonly used treatments are endoscopic botulin toxin injection (EBTI), endoscopic balloon dilation (EBD), and surgical myotomy with or without a fundoplication; however, reported outcomes mostly come from cohort studies.

To summarize and compare the safety and efficacy of endoscopic and surgical treatments for esophageal achalasia.

A systematic electronic Medline literature search of articles on esophageal achalasia. Treatment options reviewed included EBTI, EBD, and surgical myotomy using open and minimally invasive techniques. Main outcome measures were frequency of symptom relief, prevalence of post-treatment gastroesophageal reflux (GER), and complications. Outcome probability was estimated using weighted averages of the sample prevalence in each study, with weights equal to the number of patients. Outcomes, within or across studies, were compared using meta-analysis and meta-regression, respectively.

A total of 105 articles reporting on 7855 patients were selected, tabulated and reviewed. Symptom relief after EBD was better than after EBTI (68.2% vs. 40.6%; OR 3.4; 95% CI, 1.2-9.8; P = 0.02), and the need for additional therapy was greater for patients receiving EBTI (46.6% vs. 25%; OR, 2.6; 95% CI, 1.05-6.5; P = 0.04). Laparoscopic myotomy, when combined with an antireflux procedure, provided better symptom relief (90%) than all endoscopic and other surgical approaches and a low complication rate (6.3%). The incidence of postoperative GER was lower when a fundoplication was added to a laparoscopic myotomy (31.5% without a fundoplication vs. 8.8% with; OR, 6.3; 95% CI, 2.0-19.4; P = 0.003).

EBD is superior to EBTI. Laparoscopic myotomy with fundoplication was the most effective surgical technique and can be considered the operative procedure of choice.

To determine the most effective intervention procedure by evaluation of mid and long-term therapeutic efficacy in patients of stricture of the gastrointestinal tract (GIT).

Different intervention procedures were used to treat benign stricture of GIT in 180 patients including pneumatic dilation (group A, n=80), permanent (group B, n=25) and temporary (group C, n=75) placement of expandable metallic stents.

The diameters of the strictured GIT were significantly greater after the treatment of all procedures employed (P<0.01). For the 80 patients in group A, 160 dilations were performed (mean, 2.0 times per patient). Complications in group A included chest pain (n=20), reflux (n=16), and bleeding (n=6). Dysphagia relapse occurred in 24 (30%) and 48 (60%) patients respectively during 6-and-12 month follow-up periods in group A. In group B, 25 uncovered or partially covered or antireflux covered expandable metallic stents were placed permanently, complications included chest pain (n=10), reflux (n=15), bleeding (n=3), and stent migration (n=4), and dysphagia relapse occurred in 5 (20%) and 3 patients (25%) during the 6-and-12 month follow-up periods, respectively. In group C, the partially covered expandable metallic stents were temporarily placed in 75 patients and removed after 3 to 7 days via gastroscope, complications including chest pain (n=30), reflux (n=9), and bleeding (n=12), and dysphagia relapse occurred in 9 (12%) and 8 patients (16%) during the 6-and-12 month follow-up periods, respectively. The placement and withdrawal of stents were all successfully performed. The follow-up of all patients lasted for 6 to 96 months (mean 45.3+/-18.6 months).

The effective procedures for benign GIT stricture are pneumatic dilation and temporary placement of partially-covered expandable metallic stents. Temporary placement of partially-covered expandable metallic stents is one of the best methods for benign GIT strictures in mid and long-term therapeutic efficacy.

Since 1980, botulinum toxin has been employed for the treatment of various voluntary muscle spastic disorders in the fields of neurology and ophthalmology. More recently, botulinum toxin has been proved to be effective in the therapy of dyskinetic smooth muscle disorders of the gastrointestinal tract. Achalasia and anal fissure are the gastrointestinal disorders in which botulinum toxin therapy has been most extensively investigated. Botulinum toxin is the best treatment option for achalasia in patients whose condition makes them unfit for pneumatic dilation or surgery. In anal fissure, botulinum toxin is highly effective and may become the treatment of choice. In the future, botulinum toxin application in the gastrointestinal tract will be extended to many other gastrointestinal disorders, such as non-achalasic motor disorders of the oesophagus, dysfunction of Oddi's sphincter, achalasia of the internal anal sphincter and others. This article describes the mechanism of action, rationale of employment, indications and side-effects of botulinum toxin application in smooth muscle disorders of the gastrointestinal tract, and compares the results of different techniques of botulinum toxin therapeutic application.

the aim of this work was to check literature to assess botulinum toxin injection efficacy in gastrointestinal motor disorders with special emphasis on controlled clinical trials.

literature was carried out with the Medline data bank.

seventy three articles in French and in English including a recent general review were retained. Comparative clinical trials only concerned achalasia and anal fissure. The other gastrointestinal motor disorders only gave rise to open non-controlled trials assessed on clinical end points.

this review of literature helps to determine usefulness and safety of Botulinum toxin injection in the treatment of esophageal achalasia and anal fissure. The main limitation is its brief duration of action. Studies concerning the others spastic motor disorders are based on unknown physiopathology and controlled trials are required to assess its efficacy.

botulinum toxin is increasingly used for gastrointestinal motor disorders with worthwhile results. Its efficacy has been yet established for only two disorders : it has a valuable palliative role in achalasia and may be curative for anal fissure.

Advances in achalasia has led to the development of new therapeutic options. This review will focus on methodology and outcomes of two established techniques; pneumatic dilation and surgical myotomy; and one new technique, LES injection of botulinum A toxin.

Achalasia is an idiopathic neuromuscular disorder of the esophagus which is associated with absence of esophageal peristalsis and incomplete relaxation of a normal or raised lower esophageal sphincter (LES). Dysphagia is the most commonly associated symptom. Conventional therapeutic approaches are directed to reducing LES pressure and include orally-administered smooth muscle relaxants, forceful sphincter dilation with balloon dilators, and open or laparoscopic-assisted myotomy of the LES. Pharmacologic therapies have a low success rate. Forceful dilation has a perforation complication rate of 2% to 5%, and myotomies may precipitate significant gastroesophageal reflux, a complication minimized when a partial fundal wrap is employed simultaneously. In recent years, botulinum toxin, utilized widely as a striated muscle relaxant in managing blepharospasm, anal sphincter spasm, and muscle spasm complicating CVAs, and in smoothening facial wrinkles, has been extended to the management of achalasia on the basis that it impairs smooth muscle responsiveness to acetylcholine. Eighty units of Botox (botulinum toxin) are injected directly into the endoscopically (endoscopic ultrasound techniques may facilitate localization) located LES region (20 units into each of 4 quadrants). Symptom relief lasting 6 months on average is experienced in more than 65% of treated patients, and the complication rate is negligible. This therapeutic option is reserved for patients too ill to undergo any surgical procedure and is most effective when the lower esophageal region is hypertonic.

To define the esophageal motor disorders of achalasia and esophageal spasms and describe their presentation in the clinical setting.

Selected research-based articles, textbooks, and expert opinion. A case study is presented.

The presentation of esophageal motor disorders may not be clear, particularly when the presenting symptom is chest pain. Determining whether the pain is cardiac or digestive in origin is crucial.

Progressive dysphagia for both solids and liquids is the major symptom of achalasia; other symptoms include regurgitation, chest pain, and nocturnal cough. Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids. Dysphagia related to esophageal motility is characterized by a sensation of swallowed food "sticking" in the throat or chest; there is no problem initiating the act of swallowing.

Esophageal motor function remains of investigative and clinical interest because of its relevance to symptoms and its relation to the occurrence and management of gastroesophageal reflux disease. Refinement in diagnostic methods continues to occur, and improved tests for identifying the nature or severity of motor disturbances in both the proximal and distal esophageal regions are now well described. Controversy concerning the management of achalasia, the best-understood distal motor disorder, is resolving as the benefits and disadvantages of available treatment options are becoming recognized. The relation of esophageal motor dysfunction to outcomes from antireflux surgery remains incompletely understood.

Motor dysfunction is responsible for symptomatic illnesses both in the proximal skeletal muscle region and in the distal smooth muscle esophagus. Practical methods for diagnosing and treating oropharyngeal dysphagia continue to reach consensus. Achalasia, the most significant of the distal motor disorders, is of investigative interest because of the expanded armamentarium of treatment options. Minimally invasive surgical methods have taken an important foothold as a primary treatment of this disorder. Appreciation is growing for sensory dysfunction that accompanies distal motor disorders. Such dysfunction may help explain the observed discrepancies between symptoms and measurable motility abnormality.

At the close of the 20th century, therapeutic endoscopy in the esophagus has expanded to encompass a broad array of interventions. As the number of procedures grows, emphasis in the medical literature has begun to shift to analyses of which procedures should be performed. Many studies published in 1999 on topics ranging from endoscopic treatment of benign and malignant strictures, to variceal bleeding, to Barrett esophagus have focused on which of several methods provides the best long-term response with the fewest interventions. This is a review of the major published studies of endoscopic interventions in the esophagus as well as selected abstracts. The conclusions of these studies and reports of new endoscopic therapies draw a clear map of where nonoperative esophageal therapeutics are headed in the next several years.

Achalasia is rare in children. Recently, injection of botulinum toxin into the lower esophageal sphincter has been studied as an alternative to esophageal pneumatic dilatation or surgical myotomy as treatment for achalasia. In the current study, the effects of botulinum toxin were investigated in the largest known series of children with achalasia.

Treatment for achalasia was assessed in 23 pediatric patients who received botulinum toxin from June 1995 through November 1998. Those who continued to receive botulinum toxin and did not subsequently undergo pneumatic dilatation or surgery were considered repeat responders. Results were compared with those of published studies evaluating the use of botulinum toxin in adults with achalasia.

Nineteen patients initially responded to botulinum toxin. Mean duration of effect was 4.2 months +/- 4.0 (SD). At the end of the study period, three were repeat responders, three experienced dysphagia but did not receive pneumatic dilatation or surgery, three underwent pneumatic dilatation, eight underwent surgery, three underwent pneumatic dilatation with subsequent surgery, and three awaited surgery. Meta-analysis shows that, in the current study group, the data point expressing time of follow-up evaluation versus percentage of patients needing one injection session without additional procedures (botulinum toxin injection, pneumatic dilatation, or surgery) falls within the curve for those in studies on adult patients receiving botulinum toxin for achalasia.

Botulinum toxin effectively initiates the resolution of symptoms associated with achalasia in children. However, one half of patients are expected to need an additional procedure approximately 7 months after one injection session. The authors recommend that botulinum toxin be used only for children with achalasia who are poor candidates for either pneumatic dilatation or surgery.

Advances in the clinical use of botulinum neurotoxins continue. Of interest to the neurologist is the advanced practice in the treatment of focal dystonia and the new developments on other dyskinesias and on autonomic control of smooth muscle motility. New toxin serotypes are now being tested; their availability will improve clinical practice and will possibly lead to combined treatments. Indications in spasticity and in juvenile cerebral palsy are now under scrutiny. The combination of focal chemodenervation with specific rehabilitation procedures enables new development in this field.

A consolidation of ideas regarding the pathogenesis and management of esophageal motor disorders occurred over the past year. The development and application of diagnostic techniques has stimulated new thinking about the events responsible for peristalsis and has provided novel avenues for studying mechanisms of symptom production. Achalasia remains the most investigated and understood motor disorder, and the year's research focused heavily on management approaches for this important condition. Other topics addressed in this review include proximal esophageal symptoms and disorders, spastic disorders, and esophageal hypomotility.