Primary Esophageal Motility Disorder Research Articles

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.

Opiate‐induced oesophageal dysmotility

Opiates have well characterized (troublesome) untoward effects on the gastrointestinal tract. Opioid bowel dysfunction has been a subject of research and even drug design, but surprisingly little is known with regard to clinical effects of opiates on the oesophagus. To characterize opiate effects on motor function of the oesophagus in patients presenting with dysphagia. Retrospective review of 15 patients with dysphagia referred for oesophageal manometry while on chronic opiates. Manometry was completed during opiate use and in three cases, after opiates were discontinued. All patients demonstrated motility abnormalities. Incomplete lower oesophageal sphincter (LOS) relaxation (11.5 +/- 1.6 mmHg) was seen in most cases. Ten patients demonstrated nonperistaltic contractions in > or =3 of 10 swallows. Additional abnormalities included high amplitude contractions; triple peaked contractions; and increased velocity. The average resting lower oesophageal sphincter (LOSP) met criteria for hypertensive LOS in three patients. These features were suggestive of spasm or achalasia. Repeat manometry off opiates was performed in three cases. LOS relaxation was noted to be complete upon repeat manometry in these cases. There was also improved peristalsis and normal velocity. A range of manometric abnormalities were seen in patients with dysphagia in the setting of opiate use: impaired LOS relaxation, high amplitude/velocity and simultaneous oesophageal waves. These data suggest that the oesophagus is susceptible to the effects of opiates and care must be taken before ascribing dysphagia to a primary oesophageal motility disorder in patients taking opiates.

Invited Commentary

Achalasia is the most common and best characterized primary esophageal motility disorder with a prevalence of 10 per 100,000 individuals, with an annual incidence of 0.5 cases per 100,000 individuals. The natural pathology of achalasia is such that progressive neural degeneration leads to dysphagia, a defunctionalized esophagus, and eventually end-stage disease that requires an esophagectomy. Finley and colleagues [1Finley C. Kondra J. Clifton J. Yee J. Finley R. Factors associated with postoperative symptoms after laparoscopic Heller myotomy.Ann Thorac Surg. 2010; 89: 392-396Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar] have investigated the effects of initial nonoperative management in short- and long-term symptoms after myotomy for a 14-year period of study. The most concerning finding was that Botox therapy at initial presentation was associated with a higher rate of dysphagia 1 year after surgical myotomy. A surgical concern with Botox is the development of fibrosis leading to difficulty in submucosal dissection during the myotomy and even possible incomplete myotomy. These findings suggest that initial temporizing procedures are not without consequences. Every study has its limitations. This article provides useful information, although ascertainment bias is likely present because all subjective data were recorded by the operating surgeon. There must also be some caution used to interpret the initial treatment data because of the neat separation of the three initial treatments (ie, 62.3% medication, 38.7% pneumatic dilation, and 11.1% Botox). There is no description of overlap between the three treatments, which is common to everyday practice. Half of the patients in this study underwent fundoplication. Many United States' surgeons believe that a myotomy of this extent should be followed by a partial fundoplication to prevent reflux. A prospective randomized trial conducted in the United States on 43 patients demonstrated a lower incidence of reflux without increased dysphagia in patients who underwent myotomy plus Dor compared with myotomy alone [2Richards W.O. Torquati A. Holzman M.D. et al.Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind trial.Ann Surg. 2004; 240: 405-412Crossref PubMed Scopus (423) Google Scholar]. Despite this data, the role of fundoplication remains controversial, especially outside the United States. The myotomy does not reverse the underlying pathophysiology of neural degeneration, but it does convert the esophagus into a passive conduit dependent on gravity for evacuation. A balance between obtaining a complete myotomy and the avoidance of excessive reflux is the primary goal. Delay in surgical treatment will only increase the likelihood of long-term sequela (ie, mega-esophagus, thereby leading to esophagectomy for end-stage disease). Finley and colleagues [1Finley C. Kondra J. Clifton J. Yee J. Finley R. Factors associated with postoperative symptoms after laparoscopic Heller myotomy.Ann Thorac Surg. 2010; 89: 392-396Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar] provide additional evidence to support the early treatment of achalasia by surgical intervention rather than exhaustive conservative treatments. Factors Associated With Postoperative Symptoms After Laparoscopic Heller MyotomyThe Annals of Thoracic SurgeryVol. 89Issue 2PreviewOur objective is to ascertain if preoperative and perioperative treatments affect the short- and long-term symptom frequency or symptom scores for dysphagia, regurgitation, and heartburn in patients with laparoscopic Heller myotomy for achalasia. Full-Text PDF

Utility of esophageal manometry and pH-metry in gastroesophageal reflux disease before surgery

The necessity of manometric evaluation before fundoplication in patients with gastroesophageal reflux disease is still a matter of debate. However, misdiagnosis can be responsible for postoperative problems. We aimed to evaluate the necessities of manometry and pH-metry before fundoplication in order to prevent possible complications after surgery. Between 1997 and 2004, 259 consecutive patients who referred to our laboratory with a diagnosis of gastroesophageal reflux disease and request for manometric test before surgery were evaluated retrospectively. Manometric analysis was performed in all patients and 24-hour ambulatory pH-metry in 91 of them. The mean age of the patients was 42.6+/-13 years and 51% were female. While 102 (39.4%) of the patients had normal esophageal motility, 122 (47.1%) had gastroesophageal reflux-related dysmotility (22% with ineffective esophageal motility, 25.1% with hypotensive lower esophageal sphincter). Primary esophageal motility disorders were detected in 29 (11.2%) patients (4 achalasia, 24 uncoordinated contractions and 1 nutcracker esophagus). Six patients had secondary dysmotility caused by scleroderma. Pathologic reflux was detected in 54 (59.3%) patients in whom pH-metry was performed. Our results support that manometry and pH-metry must be performed before surgery in gastroesophageal reflux disease.

Insights Gained From the Symptomatic Evaluation of Classification of Esophageal Motility Disorders: A Review of 4215 Patients

Purpose: Achalasia (Ach), diffuse esophageal spasm (DES), nutcracker esophagus (NC), and non specific motility disorder (NSMD), are described primary esophageal body motility disorders. The relationship between manometric diagnosis and clinical symptoms is poorly understood. The aim of this study is to examine the correlation between patient's presenting symptoms and manometric findings. Methods: Manometric findings and reported symptoms of all patients undergoing esophageal manometry and pH studies at the Creighton University Medical Center were prospectively entered in a database. Twenty-four year data was accessed and analyzed to understand symptom correlation. Results: Of the 4215 patients, 130(3.1%) had Ach, 192 (4.6%) had DES, 290 (6.9%) had NC, 508 (12.1%) had NSMD and 3095 (73.4%) had normal esophageal body motility. There is significant symptom overlap between the groups. The pattern of symptom distribution is similar in patients with Ach and DES with dysphagia correlating with increasing simultaneous waves. Patients with NC, normal body motility and NSMD predominantly presented with reflux symptoms. Twenty-four hour pH score and reflux associated symptoms are inversely related to the LES competence. Conclusion: Symptoms and manometric diagnosis do not correlate well on an individual basis. Achalasia- DES seem to be part of the same clinical spectrum. Lower esophageal sphincter competence plays an important role in preventing gastro-esophageal reflux. Treatment of GERD and its related esophageal body motility disturbances should be directed at restoring LES competence.

HP17P BARRETT'S OESOPHAGUS AND THE SYMPTOMATIC EFFICACY OF FUNDOPLICATION

Purpose: To examine the efficacy of fundoplication for patients with uncomplicated gastro‐oesophageal reflux disease (GORD) and Barrett's oesophagus (BO) and to compare the outcomes for patients with BO following either Nissen or Toupet fundoplication.Methods: A prospectively collected database identified 2990 patients who underwent either Nissen or Toupet laparoscopic fundoplication for GORD or BO between 1991 and 2008. Pre‐ and post‐operative outcomes were measured using a simplified DeMeester scoring system. The scores were recorded for up to 5 years by the same research nurse and compared using small sample statistics.Results: Of the 2990 patients, 390 (13%) had BO and 2600 (87%) had GORD. Preoperatively, the BO group experienced significantly more symptoms of heartburn than did the GORD group (86 vs. 91% p < 0.01). However, both groups responded equally favourably to fundoplication with high patient satisfaction at 5 years (88 vs. 93%, p = NS). There were no statistically significant differences in post‐operative medication and re‐operations rates between the two groups. Nissen and Toupet procedures were both equally effective for patients with Barrett's disease with respect to heartburn and regurgitation. At five years there was an increased occurrence of dysphagia in the Toupet group (17%) compared with the Nissen group (4%, p = 0.01). This may follow from the Toupet procedure being favoured for patients with documented primary oesophageal motility disorder (Toupet 47% vs. Nissen 30%, p < 0.01).Conclusions: The symptoms of uncomplicated GORD and BO respond very well to fundoplication. Both the Nissen and Toupet procedures are equally effective in patients with Barrett's oesophagus.

Primary Versus Secondary Esophageal Motility Disorders: Diagnosis and Implications for Treatment

Ambulatory pH monitoring is essential in patients with a manometric pattern of either diffuse esophageal spasm (DES) or nutcracker esophagus (NE), since these diseases can be considered a primary esophageal motility disorder (PEMD) only in the absence of gastroesophageal reflux disease (GERD). If GERD is present, the motility abnormality is considered secondary, and treatment is directed toward reflux. The aims of this study were to determine in patients with a manometric picture of DES and NE (1) if symptoms alone can distinguish PEMD from GERD and (2) the value of ambulatory pH monitoring. A total of 180 patients fulfilled the manometric criteria for NE, and 124 (69%) of them had GERD detected by ambulatory pH monitoring. Among the 56 patients with PEMD, 31 (55%) were taking proton pump inhibitors on the assumption that GERD was present. Chest pain prevalence was similar when the two groups were compared; however, the symptom was more severe in the PEMD patients. Heartburn prevalence and severity was higher in the GERD group. A total of 121 patients fulfilled the manometric criteria for DES, and 73 (60%) of them had GERD detected by ambulatory pH monitoring. Among the 48 patients with PEMD, 39 (81%) were taking proton pump inhibitors. Dysphagia was more prevalent in the PEMD group. These data show that (1) 2 of 3 of patients with a manometric picture of NE or DES have GERD and (2) symptoms did not distinguish PEMD from GERD. Esophageal manometry and pH monitoring are essential to distinguish PEMD from GERD and to guide appropriate therapy.

Oesophageal achalasia: An argument for primary surgical management

Achalasia is a primary oesophageal motility disorder resulting from damage to the ganglion cells of the myenteric plexus. Impaired relaxation of the lower oesophageal sphincter and aperistalsis causes its cardinal symptoms of dysphagia, chest pain and reflux-type symptoms. Management is somewhat controversial, with options including systemic and local pharmacotherapy, dilatation and oesophagomyotomy. We review the presentation, investigation and management of oesophageal achalasia and make an argument for primary surgical management. We performed a Medline search of the term 'achalasia', limiting the search to clinical trials and meta-analyses. We then selected articles based on their abstracts using four main criteria: previously unreported findings, previously unreported techniques, size of patient cohort and journal impact factor. References in selected articles were manually searched for other relevant articles. Achalasia has been managed using a variety of techniques including systemic and local pharmacotherapy, forced dilatation and oesophagomyotomy. Success rates vary widely between techniques. Mechanical disruption ofthe lower oesophageal sphincter is most successful. In achalasia, mechanical disruption of the lower oesophageal sphincter using forced dilatation or surgical myotomy offers the only realistic prospect of long-term symptom relief. Recent evidence suggests that previous medical treatment or dilatation makes oesophagomyotomy more difficult and increases the risk of complications. As the morbidity associated with surgery continues to decrease with improvements in minimal access techniques, the argument for primary management of achalasia with oesophagomyotomy becomes more compelling.

Endoscopic and Surgical Treatments for Achalasia

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.

Traitement chirurgical des troubles moteurs de l’œsophage

Primary esophageal motility disorders are rare, the most common diagnoses being achalasia and diffuse esophageal spasm. Treatment aims to alleviate symptoms and may be medical, endoscopic, or surgical. Achalasia is most commonly treated by pneumatic dilatation or by laparoscopic Heller cardiomyotomy. Pneumatic dilatation is effective in 60-80% of cases, but functional results deteriorate over time. Surgical treatment is indicated when endoscopic dilatation is contraindicated or has failed. Functional results after cardiomyotomy are satisfactory in 90% of cases and results appear to be stable over time. The need for an associated antireflux procedure and the type of fundoplication remain controversial. For diffuse esophageal spasm, extended esophageal myotomy has yielded satisfactory functional results, but surgical treatment should be reserved for selected patients with severe symptoms.

Surgical Therapy for Primary Esophageal Motility Disorders

Surgical Therapy for Primary Esophageal Motility Disorders

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.

Ösophageale Schluckstörungen

Dysphagia can be caused by a number of disorders such as benign or malignant obstruction of the esophagus, inflammatory alterations of the mucosa or primary esophageal motility disorders. Endoscopic evaluation is recommended for all patients to exclude malignancy and to establish or confirm a diagnosis. This article provides an overview of the most frequent inflammatory and functional esophageal disorders causing dysphagia. Clinical findings, diagnostic procedures and therapeutic management of primary esophageal motility disorders such as achalasia and diffuse esophageal spasm as well as of GERD and eosinophilic esophagitis are discussed. The diagnosis of achalasia is made by barium swallow with fluoroscopy and by manometry. Therapeutic options for achalasia are pneumatic dilatation of the esophagogastric junction, laparoscopic cardiomyotomy combined with fundoplication and botulinum toxin injection of the lower esophageal sphincter Diffuse esophageal spasm is manometrically characterized by normal peristalsis intermittently interrupted by simultaneous contractions. Potential medical therapies are PPIs for underlying GERD, smooth-muscle relaxants and antidepressant medications. GERD is a multifaceted disease caused by abnormal reflux of gastric contents into the esophagus leading to chronic symptoms or mucosal damage. Therapy includes lifestyle modifications, acid suppressive medications mainly by PPI and laparoscopic fundoplication in selected patients. Eosinophilic esophagitis is a chronic inflammatory disorder of the esophagus diagnosed histologically. The main symptom of eosinophilic esophagitis is dysphagia for solid food with imminent risk of food impaction. Systemic or topical corticosteroids are the therapy of choice.

Correlation between esophageal contraction amplitude and lower esophageal sphincter pressure in patients with nutcracker esophagus

Nutcracker esophagus (NE) is a primary esophageal motility disorder characterized by high-wave amplitude at the distal esophagus. The aim of this study was to analyze patients with NE and determine the relationship between distal esophageal contraction amplitude and lower esophageal sphincter (LES) pressure. Esophageal manometry tracings of patients with NE, defined as the presence of distal contraction amplitude of more than 182 mmHg after wet swallow, were analyzed. LES pressure was measured as the mean end-expiratory value. Spearman's correlation coefficient analysis was used to compare esophageal contraction amplitude with LES pressure. This comparison was also performed in patients with isolated hypertensive LES (HLES) and in subjects with normal manometry. Forty patients (25 female, 15 male; mean age 54 years) with NE were included in the study. Mean (SD) distal esophageal contraction amplitude was 230 (35.7) mmHg and mean LES pressure was 27.3 (5.7) mmHg. Esophageal contraction amplitude showed a positive correlation with LES pressure (r = 0.49, P < 0.01). In contrast, no correlation was found in patients with HLES (r = 0.21, P > 0.05) and in those with a normal manometric study (r = 0.18, P > 0.05). It is concluded that in patients with nutcracker esophagus a positive correlation exists between distal esophageal contraction amplitude and LES pressure, suggesting a diffuse hypertensive pattern involving smooth muscle at the distal esophagus and adjacent LES.

Pneumatic dilation or myotomy for achalasia?

Pneumatic dilation or myotomy for achalasia?

Primary esophageal motility disorders: Concise review for clinicians

Primary esophageal motility disorders comprise various abnormal manometric patterns which usually present with dysphagia or chest pain. Some, such as achalasia, are diseases with a well defined pathology, characteristic manometric features, and good response to treatments directed towards the palliation of symptoms. Other disorders, such as diffuse esophageal spasm and nutcracker esophagus, have no well defined pathology and could represent a range of motility abnormalities associated with subtle neuropathic changes, gastresophageal reflux and anxiety states. On the other hand, hypocontracting esophagus is generally caused by weak musculature commonly associated with gastresophageal reflux disease. Although manometric patterns have been defined for these disorders, the relation with symptoms is poorly defined and in some cases the response to medical or surgical therapy unpredictable. The aim of this paper is to present a wide spectrum of the primary esophageal motility disorders, as well as to give a concise review for the clinicians encountering these specific diseases.

Evaluation of artificial neural networks in the classification of primary oesophageal dysmotility

Objective. Artificial neural networks (ANNs) can rapidly analyse large data sets and exploit complex mathematical relationships between variables. We investigated the feasibility of utilizing ANNs in the recognition and objective classification of primary oesophageal motor disorders, based on stationary oesophageal manometry recordings. Material andmethods. One hundred swallow sequences, including 80 that were representative of various oesophageal motor disorders and 20 of normal motility, were identified from 54 patients (34 F; median age 59 years). Two different ANN techniques were trained to recognize normal and abnormal swallow sequences using mathematical features of pressure wave patterns both with (ANN + ) and without (ANN − ) the inclusion of standard manometric criteria. The ANNs were cross-validated and their performances were compared to the diagnoses obtained by standard visual evaluation of the manometric data. Results. Interestingly, ANN−, rather than ANN+, programs gave the best overall performance, correctly classifying >80% of swallow sequences (achalasia 100%, nutcracker oesophagus 100%, ineffective oesophageal motility 80%, diffuse oesophageal spasm 60%, normal motility 80%). The standard deviation of the distal oesophageal pressure and propagated pressure wave activity were the most influential variables in the ANN− and ANN+ programs, respectively. Conclusions. ANNs represent a potentially important tool that can be used to improve the classification and diagnosis of primary oesophageal motility disorders.

Lack of necessity for corrections for pyloric losses and duodenogastric reflux in the performance of gastric secretory studies.

Gastric secretory studies are subject to considerable error owing to incomplete collections and to contamination by reflux of alkaline duodenal contents. Corrections for these sources of error have been defined, and they have been extensively applied in a research setting. In order to assess their utility in the performance of routine gastric secretory studies, the value of such corrections was assessed in 56 studies in patients with duodenal ulceration (10), previous surgical vagotomy (8), reflux oesophagitis (30) and primary oesophageal motility disorders (8). The effect of such corrections was small, and there were close correlations between uncorrected and corrected acid outputs in all four groups. The status of the vagal efferent gastric fibres was assessed by comparing the acid output after insulin hypoglycaemia with the maximal acid output after pentagastrin (insulin: pentagastrin ratio). The application of the corrections did not alter the conclusion regarding the assessment of vagal status in 55 of the 56 studies performed. It is concluded that such corrections are not necessary in the routine performance of gastric secretory studies.

Cause and treatment of epiphrenic diverticula

Background Epiphrenic diverticula of the esophagus are often associated with a concomitant esophageal motor disorder, which is thought to be the cause of the diverticulum and some of the patient’s symptoms. At one time diverticula were best removed via a left thoracotomy, but now the operation can be performed laparoscopically in most cases. We hypothesized that: (1) a motor disorder is the underlying cause of the diverticulum; and (2) optimal treatment consists of laparoscopic resection of the diverticulum, a Heller myotomy, and Dor fundoplication. Methods We performed a retrospective review of a prospectively collected database from a university hospital tertiary care center. Between June 1994 and December 2002, we evaluated 21 patients with epiphrenic diverticula. An associated motility disorder of the esophagus was found in 81% of patients (achalasia, 9%; diffuse esophageal spasm, 24%; nonspecific esophageal motility disorder, 24%; nutcracker esophagus, 24%). Seven (33%) of these patients, all with esophageal dysmotility, were referred for treatment. The laparoscopic operation entailed resection of the diverticulum (using an endoscopic stapler), a Heller myotomy, and a Dor fundoplication. Results All operations were completed laparoscopically. The postoperative course of 6 patients was uneventful and they left the hospital after 72 ± 21 hours. In 1 patient an acute paraesophageal hernia developed, which was repaired on the second postoperative day. Late follow-up (median 57 months) showed that all 7 patients were asymptomatic. Conclusions These data support the conclusions that: (1) a primary esophageal motility disorder is the underlying cause of most epiphrenic diverticula; and (2) laparoscopic treatment is successful and should be the method of choice. The diverticular neck can be exposed satisfactorily from the abdomen; a stapler inserted from this angle is better orientated to transect the neck than one inserted through a thoracoscopic approach. Furthermore, the myotomy and fundoplication are much more easily performed from the abdomen than from alternative approaches.

Spectrum of Esophageal Motility Disorders

The named primary esophageal motility disorders (PEMDs) are achalasia, diffuse esophageal spasm (DES), nutcracker esophagus (NE), and hypertensive lower esophageal sphincter (HTN-LES). Although the diagnosis and treatment of achalasia are well defined, such is not the case with the other disorders. (1) Symptoms do not reliably distinguish PEMDs from gastroesophageal reflux disease; (2) esophageal function tests are essential to this distinction and to identifying the type of PEMD; (3) minimally invasive surgery is effective for each condition; and (4) the laparoscopic approach is better than the thoracoscopic approach. University hospital tertiary care center. Retrospective review of a prospectively collected database. A diagnosis of PEMD was established in 397 patients by esophagogram, endoscopy, manometry, and pH monitoring. There were 305 patients (77%) with achalasia, 49 patients (12%) with DES, 41 patients (10%) with NE, and 2 patients (1%) with HTN-LES. Two hundred eight patients (52%) underwent a myotomy by either a thoracoscopic or a laparoscopic approach. Ninety-nine patients (25%) had a diagnosis of gastroesophageal reflux disease at the time of referral and had been treated with acid-suppressing medications. In achalasia and DES, a thoracoscopic or laparoscopic myotomy relieved dysphagia and chest pain in more than 80% of the patients. In contrast, in NE the results were less predictable, and the operation most often failed to relieve symptoms. These results show that (1) symptoms were unreliable in distinguishing gastroesophageal reflux disease from PEMDs; (2) esophageal function tests were essential to diagnose PEMD and to define its type; (3) the laparoscopic approach was better than the thoracoscopic approach; (4) a laparoscopic Heller myotomy is the treatment of choice for achalasia, DES, and HTN-LES; and (5) a predictably good treatment for NE is still elusive, and the results of surgery were disappointing.