Primary Esophageal Motor Disorder Research Articles

Clinical Updates in Esophageal Motility Disorders Beyond Achalasia

Although achalasia is arguably the most widely known motility disorder, the diagnostic workup and treatment of other primary esophageal motility disorders are increasingly relevant.1 Esophageal symptoms such as dysphagia, non-cardiac chest pain, and regurgitation can arise from primary esophageal motor disorders beyond achalasia, including disorders with impaired relaxation across the lower esophageal sphincter (LES) that do not meet criteria for achalasia and disorders of esophageal peristalsis (Figure 1).

Esophagogastric junction outflow obstruction.

In the Chicago Classification version 4.0 (CCv4), esophagogastric junction outflow obstruction (EGJOO) is manometrically defined as an elevated median integrated relaxation pressure (IRP) and elevated intrabolus pressure (IBP) during supine wet swallows, and persistently elevated median IRP in the upright position. A clinically relevant conclusive diagnosis of EGJOO requires a manometric diagnosis of EGJOO and associated symptoms such as dysphagia and/or chest pain with at least one of the following supportive investigations (pharmacologic provocation, timed barium esophagogram, and/or endoflip). The Chicago Classification is intended for diagnosis of primary esophageal motor disorders, and thus history and endoscopic evaluation are important to exclude conditions (eg, previous surgery, strictures, or masses) that can secondarily generate the EGJOO pattern on HRM. While a manometric finding of EGJOO is often made and can be an early sign of achalasia, more often it is a manometric finding without clinical implications. The proposed changes in CC4.0 have attempted to make the diagnosis more specific, in order to reduce the number of clinically irrelevant diagnoses and avoid confusion by patients and physicians alike.

Megaesófago como complicación de acalasia: Reporte de caso y revisión narrativa de la literatura

El megaesófago se presenta entre el 5 % y el 20 % de pacientes con acalasia, un trastorno motor esofágico primario reconocido hace más de 300 años, a considerarse en todo paciente con disfagia no explicada por un proceso obstructivo o inflamatorio luego de un estudio endoscópico detallado. Se presenta el caso de un paciente con disfagia progresiva, en quien se documentó megaesófago como complicación de una acalasia de largo tiempo de evolución, no tratada. Se descartó la enfermedad de Chagas mediante enzimoinmunoensayo (ELISA) e inmunofluorescencia indirecta (IFI), tal como recomiendan las guías actuales. Ante la baja frecuencia de esta entidad en nuestro medio y las implicaciones terapéuticas que tiene para los pacientes con acalasia, se realizó una revisión narrativa en la literatura sobre su diagnóstico y alternativas de manejo.

IDIOPATHIC ACHALASIA PRIMARILY DIAGNOSED AS BULIMIA NERVOSA: THE IMPORTANCE OF THE NUTROLOGYST

Idiopathic achalasia is a primary esophageal motor disorder characterized byesophageal aperistalsis and abnormal lower esophageal sphincter relaxation in responseto deglutition. Bulimia nervosa is an eating disorder, which amongst other symptomspresents with self-induced vomiting after repetitive episodes of binge eating in order toprevent weight gain. The present study reports the case of a female patient initiallydiagnosed with bulimia nervosa, after ruling out an etiology of organic cause throughlab tests and endoscopy. The patient was referred to a nutrologyst for treating the eatingdisorder, and after nutritional evaluation, she received nutritional supplementation dueto severe dysphagia, regurgitation and weight loss. As diagnostic criteria of bulimianervosa were not met, a further investigation was conducted, which eventuallyconfirmed idiopathic achalasia through two specific tests: digitalesophagogastroduodenal seriography, and esophageal manometry. Chagas disease, themajor cause of idiopathic achalasia in Brazil, was ruled out by specific lab test. In viewof the severity of the disease, the patient underwent urgent cardiomyotomy andfundoplication. Later, balloon dilators were also used in two occasions. After sixteenmonths of medical and nutrology follow-up, the patient now is in good nutritional statusand was able to go back to her daily activities and healthy lifestyle.

Endoscopic Treatment of Esophageal Achalasia: Experience of the Hepato-Gastroenterology Service of Fez

The achalasia is a rare primary esophageal motor disorder characterized by relaxation disorders of the lower esophageal sphincter and absence of the esophageal body peristalsis. Several studies suggest that the response to the endoscopic treatment depends on several predictors. The aim of our study was to evaluate the endoscopic treatment of esophageal achalasia and identify the predictive factors of endoscopic treatment response. Patients and Methods: This is a retrospective analytical study of 78 patients with achalasia, managed in the gastroenterology department of the university medical center Hassan II-Fez, during a period of 5 years (January 2009 to December 2014). The diagnosis of achalasia was retained on a set of clinical, endoscopic, manometric and radiological arguments. A graded dilation protocol starting with a 35 mm balloon three times for 30 seconds in progressive pressure between 5 and 8 psi was performed. We used the Eckardt score to evaluate the clinical remission. Results: During the study period, 78 patients were included. The average age of our patients was 47 years old [18 - 81] with a sex-ratio M/F of 1.05. The average of Eckardt score before dilation was 5.9 [3 - 9]. An average of 1.41 dilation sessions was performed per patient with 85.9% of the initial success rate (n = 67). Initial success without further dilation sessions was achieved in 55.1% of our patients (n = 43). A clinical recurrence requiring further dilation sessions was observed in 30.8% of the cases (n = 24). The average relapse time after first dilation success was 2.7 years, 75% occurs within the first year. Dilation failure was retained in 14 patients (17.9%) requiring surgery. Only one post-dilation perforation was noted. In multivariate analysis, only odynophagia and the number of dilatation sessions were factors of failure of the endoscopic dilation. Conclusion: Pneumatic dilation is a minimally morbid and effective procedure. Our work showed that odynophagia, and the number of dilation sessions, are two predictive factors of endoscopic treatment failure.

Results of laparoscopic Heller myotomy and Dor fundoplication in treatment of achalasia

Abstract Introduction: Achalasia is a primary esophageal motor disorder, which is relatively rare. Patients usually start silently and detect late for months to years. Therefore, laparoscopic Heller myotomy is now considered the surgical procedure of choice for treating achalasia. Anti-reflux procedure usually performed by surgeons is a Dor procedure because of its many advantages, and it is easy to perform in laparoscopic surgery. Purpose: The objectives of this study is to determine the incidence and complications of laparoscopic Heller myotomy and Dor fundoplication and determine percentage improvement dysphagia after surgery. Material and Methods: Patients diagnosed achalasia and underwent laparoscopic Heller myotomy and Dor fundoplication at the Digestive Surgery Department of Cho Ray hospital from January 2011 to May 2017. Results: 23 patients with achalasia were operated on for laparoscopic Heller myotomy and Dor fundoplication. 95.7% of patient have dysphagia, regurgitation: 39.1%, and weight loss: 46%. The grade of patients with the highest diameter of the esophagus: 13.04% degree I, 52.18% degree II, 13.04% degree III, 21.74% degree IV. Average surgery time was 150.22 ± 35.63 minutes. Complication in surgery: esophageal perforation occurred in about 13.04% cases, there was 4.35% of bleeding reported. Quality of life after surgery was good and very good at 91.3%. Conclusion: The results of laparoscopic Heller myotomy and Dor fundoplication were effective, most patients satisfied the results of surgery. However, the size of this study is limited so it is necessary to follow up with other studies with larger sample size.

Primary Esophageal Motility Disorders: Beyond Achalasia.

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.

The role of Heller myotomy and POEM for nonachalasia motility disorders.

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 such as Diffuse Esophageal Spasm (DES), Nutcracker Esophagus (NE) and the Hypertensive Lower Esophageal Sphincter (HTN-LES). Most patients with DES and HTN-LES who complain of dysphagia improve after a myotomy. Patients with NE whose main complaint is chest pain, often do not have relief of the pain and can even develop dysphagia as a consequence of the myotomy. POEM is a relatively new procedure, and there are no studies with long-term follow-up and no prospective and randomized trials comparing it to surgical myotomy. Overall, the key to success is based on a complete evaluation and a careful patient selection. The best results, regardless of the technique, are in fact obtained in patients with outflow obstruction and impaired esophageal emptying, a picture similar to achalasia.

The Unusual Coexistence of Achalasia and Scleroderma

Scleroderma is an autoimmune disease characterized by excessive deposition of collagen and other connective tissue macromolecules in skin and multiple internal organs.It is called systemic Sclerosis(SS),when it involves internal organs.SS involves GI tract in 90% of the patients.Esophageal motility is the most frequently affected function in GI tract. It classically manifests as reduced LES pressure and loss of distal esophageal body peristalsis.In comparison Achalasia is a primary esophageal motor disorder characterized by insufficient relaxation of the LES and loss of esophageal peristalsis.While Sclerdoerma leads to low LESP, Achalasia causes insufficient LES relaxation.Coexistence of the two is rare.We report a case of a Scleroderma patient presenting with Achalasia. A 62 years old African American woman with history of Scleroderma and Systemic Lupus Erythematosus overlap syndrome was evaluated by gastroenterology for dysphagia. She had worsening dysphagia to both solids and liquids for three to four years.She underwent EGD which showed dilated, sigmoid shaped esophagus with retained liquid and solid food. LES was found to be hypertensive and resistance was felt while entering stomach.Barium esophagram showed dilated esophagus with narrowing at LES with bird beak appearance.So both studies were suggestive of Achalasia. Patient refused esophageal manometry at multiple occasions.Scleroderma occurs worldwide and affects all races but has higher general and age-specific rates in blacks with more severe disease and poorer outcomes than whites.GI tract involvement is very common and occurs early in the course of the disease. Esophagus is the most frequently involved internal organ causing symptoms of dysphagia, odynophagia,heartburn and regurgitation.Esophageal motility gets affected and is characterized by aperistalsis or low amplitude contractions, decreased or absent resting LES tone and lack of coordination between esophageal contractions and LES relaxations.Esophageal dysmotility in association with delayed gastric emptying results in impaired esophageal clearance of refluxed gastric contents and the development of reflux disease. In comparison Achalasia is characterized by absence of peristalsis in the distal two-thirds and elevated resting pressure in LES, typically above 45 mmhg along with failed LES relaxation with swallowing. LESP stays above 8 mmhg above gastric pressure.When the manometric characteristics of LES are compared, Achalasia is characterized by hypertensive LES and Scleroderma by hypotensive LES.Due to opposite changes in LESP, coexistence of Achalasia and scleroderma is extremely rare and so far only few cases have been reported. This case reports shows the rare coexistence of the two conditions making Achalasia one of differentials of dysphagia in Scleroderma patients.Figure 1Figure 2Figure 3

Idiopathic (primary) achalasia: a review.

Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition. Patients with achalasia commonly complain of dysphagia to solids and liquids, bland regurgitation often unresponsive to an adequate trial of proton pump inhibitor, and chest pain. Weight loss is present in many, but not all patients. Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. Endoscopic assessment of the gastroesophageal junction and gastric cardia is necessary to rule out malignancy. Newer diagnostic modalities such as high resolution manometry help in predicting treatment response in achalasia based on esophageal pressure topography patterns identifying three phenotypes of achalasia (I-III) and outcome studies suggest better treatment response with types I and II compared to type III. Although achalasia cannot be permanently cured, excellent outcomes are achieved in over 90 % of patients. Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise. The prognosis in achalasia patients is excellent. Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.

OC-071 Oesophageal physiology findings in 80 patients with recurrent dysphagia post-achalasia treatment: retest before retreating

Introduction Achalasia is a relatively rare primary oesophageal motor disorder characterised by absent peristalsis and failure of relaxation of the lower oesophageal sphincter (LOS). The standards of treatment for achalasia are pneumatic balloon dilatation and surgical myotomy. Although 85–95% success at 2 years is reported with these procedures, in the longer term, dysphagia may return in over 50%, prompting consideration of re-treatment. The definition of recurrence is often based on symptom evaluation, particularly dysphagia. Treatment of dysphagia in achalasia is targeted at the LOS, but in some patients dysphagia may be multifactorial and may not be improved by attempting to further lower LOS pressure. We aimed to evaluate the GI physiological findings of patients undergoing studies in our tertiary referral unit to investigate recurrence of dysphagia post-treatment. Method We interrogated our database for patients undergoing high resolution oesophageal manometry +/- 24-hour pH studies due to dysphagia post-treatment for achalasia between 2010–2014. We recorded achalasia subtype, LOS relaxation pressure (known as integrated relaxation pressure, or IRP), and reflux study results if performed. Results 80 eligible patients (38 female; age range 11–83 years, median 46 years) were identified with recurrent dysphagia. All had high resolution oesophageal manometry performed, and 18 patients had 24-hour pH studies due to concomitant heartburn and/or regurgitation. 52 patients had undergone at least one previous pneumatic dilatation, 23 had undergone surgical myotomy, and 5 had no response to Botox injection. 40 patients were found to have type 1 achalasia, 25 had type 2 achalasia, and 6 had type 3 achalasia. 9 patients had normal peristalsis unlikely to be consistent with a diagnosis of achalasia. 22 of the 80 patients had an IRP >15 mmHg (upper range of normal). 10 patients had a very low ( Conclusion We investigated a large cohort of patients with recurrent dysphagia post-treatment of achalasia. The most frequent achalasia subtype was type 1. Although 28% patients had high residual LOS pressures, a significant proportion of patients had findings not consistent with a need to re-treat with dilatation or myotomy (due to very low LOS pressure, gastro-oesophageal reflux disease, or possible incorrect diagnosis). These results highlight the need to carefully re-evaluate patients presenting with recurrent dysphagia after achalasia treatment before deciding on repeat therapy. Disclosure of interest None Declared.

Clinical, endoscopic and manometric features of the primary motor disorders of the esophagus.

BackgroundSignificant incidence, diagnostic difficulties, clinical relevance and therapeutic efficacy associated with the small number of publications on the primary esophageal motor disorders, motivated the present study.AimTo determine the manometric prevalence of these disorders and correlate them to the endoscopic and clinical findings.MethodsA retrospective study of 2614 patients, being 1529 (58.49%) women and 1085 (41.51%) men. From 299 manometric examinations diagnosed with primary esophageal motor disorder, were sought-clinical data (heartburn, regurgitation, dysphagia, odynophagia, non-cardiac chest pain, pharyngeal globe and extra-esophageal symptoms) and/or endoscopic (hiatal hernia, erosive esophagitis, food waste) that motivated the performance of manometry.ResultsWere found 49 cases of achalasia, 73 diffuse spasm, 89 nutcracker esophagus, 82 ineffective esophageal motility, and six lower esophageal sphincter hypertension. In relation to the correlations, it was observed that in 119 patients clinical conditions were associated with dysphagia, found in achalasia more than in other conditions; in relationship between endoscopic findings and clinical conditions there was no statistical significance between data.ConclusionsThe clinical and endoscopic findings have little value in the characterization of the primary motor disorders of the esophagus, showing even more the need for manometry, particularly in the preoperative period of gastroesophageal reflux disease.

Manejo endoscópico de la acalasia: revisión clínica

Achalasia is an incurable primary esophageal motor disorder of unknown aetiology. The intent of any treatment is to weaken the lower esophageal sphincter. Established treatments for endoscopic management are endoscopic application of botulism toxin and pneumatic dilation, along with other treatments under development such as POEM (per-oral endoscopic myotomy). The first 2 are very effective in weakening lower esophageal sphincter pressure, but their efficacy and duration vary greatly. There is a recurrence of symptoms of 50% at 6 months and almost 100% in one year for botulism toxin, while with pneumatic dilation only 60% of patients are asymptomatic at 5 years, so the need for multiple pneumatic dilation is the rule in>90% of patients. The best positive predictor of lasting symptomatic response is lower esophageal sphincter pressure<15mmHg after performing any procedure. The POEM technique is promising and still evolving, initially offering results similar to the Heller myotomy; however, we are waiting for greater experience with more patients and for long-term results.

Confocal Laser Endomicroscopy Guided Endoscopic Myotomy

Achalasia is a primary esophageal motor disorder characterized by degenerative changes of the myenteric plexus.1 Until recently, laparoscopic Heller myotomy, represented the most successful treatment option.2 Most recently, peroral endoscopic myotomy (POEM) was introduced as a novel, minimally invasive treatment option for patients with achalasia.3–5 Nevertheless, adequate length of the myotomy, either performed surgically or endoscopically, still remains unknown and is guided by an approximation extrapolated by clinical, manometric and radiological findings.

An Unusual Presentation of Achalasia

Purpose: Achalasia is a rare motor disorder with an incidence of 1 per 100 000 per year. Achalasia is a primary esophageal motor disorder of unknown etiology characterized by loss of esophageal peristalsis and manometrically by insufficient lower esophageal sphincter (LES) relaxation. We present an unusual case of a 50 year old gentleman with no past medical history admitted to our hospital for evaluation of cough, low grade fever and sweats for 2 months. He had been sent by his PMD for evaluation of an abnormal chest X-ray as outpatient. Physical exam was not significant, and abdominal exam showed no masses or organomegaly or lymphadenopathy. In the ED, he was found to have dry cough and fever, and an urgent CT scan of the chest was done which showed severe diffuse distension of the esophagus from the level of the thoracic inlet to the gastro-esophageal junction and nodular pleural/parenchymal opacities throughout both lungs suspected to be aspiration pneumonia. Gastroenterology was consulted and emergent Esophago-gastroduodenoscopy (EGD) was performed. EGD showed dilated esophagus with retained liquid and semisolid food particles, GE junction was puckered consistent with achalasia. The contents in the esophagus were suctioned and removed. He later underwent manometry confirming the diagnosis. Lab tests were within in normal limits. The patient was then scheduled for laparoscopic Heller myotomy. These motor abnormalities resulted in stasis of ingested food in the esophagus, leading to clinical symptoms, including dysphagia, regurgitation of ingested food, retrosternal pain, weight loss and aspiration pneumonia. Although it is well demonstrated that loss of myenteric esophageal neurons is the underlying problem, it still remains unclear why these neurons are preferentially attacked and destroyed by the immune system. The most successful therapies are clearly pneumatic dilatation and Heller myotomy with short-term success rates of 70-90%, declining to 50-65% after more than 15 years. The above case demonstrates that respiratory symptoms such as cough and pneumonia should be worked up further to rule out esophageal diseases such as Achalasia.

Endoscopic approach to achalasia

Achalasia is a primary esophageal motor disorder. The etiology is still unknown and therefore all treatment options are strictly palliative with the intention to weaken the lower esophageal sphincter (LES). Current established endoscopic therapeutic options include pneumatic dilation (PD) or botulinum toxin injection. Both treatment approaches have an excellent symptomatic short term effect, and lead to a reduction of LES pressure. However, the long term success of botulinum toxin (BT) injection is poor with symptom recurrence in more than 50% of the patients after 12 mo and in nearly 100% of the patients after 24 mo, which commonly requires repeat injections. In contrast, after a single PD 40%-60% of the patients remain asymptomatic for ≥ 10 years. Repeated on demand PD might become necessary and long term remission can be achieved with this approach in up to 90% of these patients. The main positive predictors for a symptomatic response to PD are an age > 40 years, a LES-pressure reduction to < 15 mmHg and/or an improved radiological esophageal clearance post-PD. However PD has a significant risk for esophageal perforation, which occurs in about 2%-3% of cases. In randomized, controlled studies BT injection was inferior to PD and surgical cardiomyotomy, whereas the efficacy of PD, in patients > 40 years, was nearly equivalent to surgery. A new promising technique might be peroral endoscopic myotomy, although long term results are needed and practicability as well as safety issues must be considered. Treatment with a temporary self expanding stent has been reported with favorable outcomes, but the data are all from one study group and must be confirmed by others before definite recommendations can be made. In addition to its use as a therapeutic tool, endoscopy also plays an important role in the diagnosis and surveillance of patients with achalasia.

Per-oral endoscopic myotomy: Hope at the end of the tunnel

Achalasia is a primary oesophageal motor disorder with absence f peristalsis usually associated with defective relaxation of the ower oesophageal sphincter (LES) [1]. This condition results n dysphagia, regurgitation, pain, undernutrition and potential ulmonary complications. Achalasia is also associated with an ncreased risk of oesophageal squamous cell carcinoma. Because chalasia may be refractory to systemic (calcium channel blockrs) and local (botulinum toxin injection) pharmacological agents, echanical disruption of the LES is currently the best long-term herapeutic option [1]. A recent meta-analysis showed that pneuatic dilation has clinical success rate of 88%, 70% and 29% after , 5 and 10 years, respectively, with a perforation rate of 2%. aparoscopic Heller myotomy, usually combined with an antireflux rocedure, shows 77–100% success rates after 3 years, but these ecline over time. A prospective, randomized, multicenter Euroean trial comparing graded pneumatic dilation to laparoscopic eller myotomy in 201 patients concluded similar therapeutic sucess rates in the 2 groups (reduction in the Eckardt score of below after 2 years in 86% and 90%, respectively). Either treatment can herefore be proposed as initial therapy [1]. Peroral endoscopic myotomy (POEM) has recently been introuced as a new treatment for achalasia. Briefly, POEM is a three-step rocedure. Step one is the creation of a submucosal oesophageal unnel to reach the LES. Step two consists in the precise cutting f the circular muscle bundles over a 7 cm oesophageal and 2 cm astric length. Step three is the clip closure of the entry point of he submucosal tunnel. From a technical point of view, POEM is at he merging of Natural Orifice Translumental Endoscopic Surgery NOTES) and endoscopic submucosal dissection. POEM developed rom a proof-of-concept series of experiments on transoesophageal OTES where investigators aimed to access the mediastinum using submucosal endoscopy with a mucosal flap safety valve technique 2]. Inoue et al., from Yokohama, Japan, reported on the first clinical eries of POEM including 17 patients: a 100% clinical success rate hat correlated well with significant reduction in LES pressure in all atients. No complication was noted. The median length of stay was ve days [3]. Since that initial study, there is now a rapidly growing

Progression of diffuse esophageal spasm to achalasia: incidence and predictive factors

The progression of certain primary esophageal motor disorders to achalasia has been documented; however, the true incidence of this decay is still elusive. This study aims to evaluate: (i) the incidence of the progression of diffuse esophageal spasm to achalasia, and (ii) predictive factors to this progression. Thirty-five patients (mean age 53 years, 80% females) with a manometric picture of diffuse esophageal spasm were followed for at least 1 year. Patients with gastroesophageal reflux disease confirmed by pH monitoring or systemic diseases that may affect esophageal motility were excluded. Esophageal manometry was repeated in all patients. Five (14%) of the patients progressed to achalasia at a mean follow-up of 2.1 (range 1-4) years. Demographic characteristics were not predictive of transition to achalasia, while dysphagia (P= 0.005) as the main symptom and the wave amplitude of simultaneous waves less than 50 mmHg (P= 0.003) were statistically significant. In conclusion, the transition of diffuse esophageal spasm to achalasia is not frequent at a 2-year follow-up. Dysphagia and simultaneous waves with low amplitude are predictive factors for this degeneration.

Deglutitive Inhibition, Latency Between Swallow and Esophageal Contractions and Primary Esophageal Motor Disorders

Swallowing induces an inhibitory wave that is followed by a contractile wave along the esophageal body. Deglutitive inhibition in the skeletal muscle of the esophagus is controlled in the brain stem whilst in the smooth muscle, an intrinsic peripheral control mechanism is critical. The latency between swallow and contractions is determined by the pattern of activation of the inhibitory and excitatory vagal pathways, the regional gradients of inhibitory and excitatory myenteric nerves, and the intrinsic properties of the smooth muscle. A wave of inhibition precedes a swallow-induced peristaltic contraction in the smooth muscle part of the human oesophagus involving both circular and longitudinal muscles in a peristaltic fashion. Deglutitive inhibition is necessary for drinking liquids which requires multiple rapid swallows (MRS). During MRS the esophageal body remains inhibited until the last of the series of swallows and then a peristaltic contraction wave follows. A normal response to MRS requires indemnity of both inhibitory and excitatory mechanisms and esophageal muscle. MRS has recently been used to assess deglutitive inhibition in patients with esophageal motor disorders. Examples with impairment of deglutitive inhibition are achalasia of the LES and diffuse esophageal spasm.

Idiopathic achalasia primarily diagnosed as bulimia nervosa: the importance of the nutrologyst

ABSTRACTIdiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter relaxation in response to deglutition. Bulimia nervosa is an eating disorder, which amongst other symptoms presents with self-induced vomiting after repetitive episodes of binge eating in order to prevent weight gain. The present study reports the case of a female patient initially diagnosed with bulimia nervosa, after ruling out an etiology of organic cause through lab tests and endoscopy. The patient was referred to a nutrologyst for treating the eating disorder, and after nutritional evaluation, she received nutritional supplementation due to severe dysphagia, regurgitation and weight loss. As diagnostic criteria of bulimia nervosa were not met, a further investigation was conducted, which eventually confirmed idiopathic achalasia through two specific tests: digital esophagogastroduodenal seriography, and esophageal manometry. Chagas disease, the major cause of idiopathic achalasia in Brazil, was ruled out by specific lab test. In view of the severity of the disease, the patient underwent urgent cardiomyotomy and fundoplication. Later, balloon dilators were also used in two occasions. After sixteen months of medical and nutrology follow-up, the patient now is in good nutritional status and was able to go back to her daily activities and healthy lifestyle.