Over the last few decades, significant improvement has been made in both the evaluation and treatment of esophageal achalasia. The Chicago classification, today in version 4.0, is now the standard for diagnosis of achalasia, providing a classification into 3 subtypes with important therapeutic and prognostic implications. Therapy, which was at first mostly limited to pneumatic dilatation, today includes minimally invasive surgery and peroral endoscopic myotomy, allowing for a more tailored approach to patients and better treatment of recurrent symptoms. This review chronicles my personal experience with achalasia over the last 35 years, describing the progress made in the treatment of patients with achalasia.

Innovation is of critical importance for the progress of medicine in general, and of surgery in particular. The introduction of minimally invasive surgery 30 years ago determined a revolution in the way we treat surgical diseases today, as most operations are now performed avoiding large incisions, with a consequent decrease in postoperative pain, shorter hospital stay, and a faster return to daily activities. However, for any innovation to become standard of care, the following criteria must be met: (1) Is it feasible? (2) Is it safe? (3) Does it offer clinical advantages to patients and the health care system? The treatment of esophageal achalasia represents an example of an innovation-per oral endoscopic myotomy-that although feasible and safe has become the standard of care without clear proof that it improves patients' outcome and provides an advantage for the health care system. The review of the treatment of esophageal achalasia during the past 30 years will shed light on this controversial dichotomy-innovation versus patient's care.

Achalasia is a rare primary motility disorder of esophagus; treatments include endoscopic balloon dilatation (EBD) and laparoscopic Heller's cardiomyotomy (LC). This study compared EBD versus LC for treatment of achalasia with focus on quality of life (QoL) and prevalence of post-treatment gastroesophageal reflux disease. This was a retrospective cohort study of all patients diagnosed with achalasia older than 16 treated with either EBD or LC from January 1998 to April 2008. Patients' demographic data, comorbidities, postintervention GERD symptoms, QoL, recurrence of dysphagia, reintervention rate, hospital stay, and time to resumption of diet were collected. Sixty-eight patients were recruited into the study (EBD n= 50; LC n= 18). A significant improvement in QoL was found in patients undergoing LC (0.917 vs. 0.807, P= 0.006). A higher proportion of patients treated with EBD developed post-treatment gastroesophageal reflux symptoms (60.5% vs. 43.8%) when compared with LC, although statistically insignificant (P= 0.34). Patients treated with balloon dilatation had a greater percentage of recurrence of dysphagia (55.1% vs. 26.7%; P= 0.235) and need of reintervention (42.1% vs. 9.1%; P= 0.045). However, these patients had a shorter median hospital stay (1d [range 0-4]) and earlier resumption of diet (0d [range 0-3]). Although EBD is associated with a quicker perioperative recovery, LC accomplished a better QoL, lower incidence of recurrence of dysphagia, and need of reintervention after treatment for achalasia.

This article reviews the changes that have taken place in the treatment of patients with achalasia in the last 20 years. It compares and contrasts treatment preferences in the USA with those of Canada and Europe. It provides a critical analysis of the recent randomized trial between laparoscopic Heller myotomy and pneumatic dilatation that was carried out in several European centers. It supports the use of laparoscopic Heller myotomy as the preferred treatment for the average patient with this disease in the USA.

Minimally invasive Heller myotomy has evolved the "gold standard" procedure for achalasia in the spectrum of current treatment options. The laparoscopic technique has proved superior to the thoracoscopic approach due to improved visualization of the esophagogastric junction. Operative controversies most recently include the length of the myotomy, especially of its fun-dic part, with respect to the balance between postoperative persistent dysphagia and development of gastroesophageal reflux, as well as the type of the added antireflux procedure. Peri-operative mortality should approach 0%, and favorable long-term results can be achieved in > 90%.

Thanks to the advent of laparoscopic techniques, the last decade and a half have witnessed a radical change in the treatment of esophageal achalasia. Because of the high success rate of the laparoscopic Heller myotomy, surgery has now become in many centers the first modality of treatment for achalasia. This shift in the treatment algorithm reflects the fact that laparoscopic Heller myotomy with partial fundoplication outperforms nonsurgical approaches, such as balloon dilatation and intrasphincteric botulinum toxin injection.

Thanks to the advent of laparoscopic techniques, the last decade and a half have witnessed a radical change in the treatment of esophageal achalasia. Because of the high success rate of the laparoscopic Heller myotomy, surgery has now become in many centers the first modality of treatment for achalasia. This shift in the treatment algorithm reflects the fact that laparoscopic Heller myotomy with partial fundoplication outperforms nonsurgical approaches, such as balloon dilatation and intrasphincteric botulinum toxin injection.

Laparoscopic Heller myotomy is the preferred treatment for achalasia. Post-operative leaks cause significant morbidity and impair functional outcome. This study assesses the efficacy of intra-operative leak testing on post-operative leak rate.

A retrospective analysis of 106 consecutive patients undergoing laparoscopic Heller myotomy by a single surgeon between November 2001 and August 2006 was undertaken. Intra-operative leak testing was performed in all patients. Variables associated with intra-operative mucosotomy were assessed by univariate analysis and logistic regression modeling.

Intra-operative mucosotomy occurred in 25% of patients. All mucosotomies were repaired primarily and tested with methylene-blue-stained saline. Dor fundoplication was performed in 74% of the patients. There were no post-operative leaks and patients were started on diet day of surgery. Mean LOS was 1.4(+/-0.7) days. Logistic regression modeling demonstrated that prior myotomy was associated with a statistically significant increase in the rate of mucosotomy (p = 0.033), while previous botox injection (p = 0.193), pneumatic dilation (p = 0.599) or concomitant hiatal hernia (p = 0.874) were not significantly associated with mucosotomy.

Laparoscopic Heller myotomy for the treatment of achalasia is a safe procedure. Intra-operative leak testing minimizes the risk of post-operative leaks and expedites post-operative management. Prior endoscopic treatment does not impair operative results.

Certain technical features of laparoscopic Heller cardiomyotomy (LHM) remain controversial, including the extent of the myotomy and the indication for an antireflux procedure. We completed a retrospective chart review of all patients who underwent LHM for achalasia at 1 tertiary care institution to review our institutional experience with LHM without an antireflux procedure. Forty patients underwent a LHM performed by 2 surgeons, 65% of whom had previous medical management (Botox: 12 patients, LES dilatation: 14). The operating time was significantly increased in patients with Botox injections (98.3 vs. 71.1 minutes, P = 0.005). There were 3 intraoperative complications (mucosal injury in 3 patients, 2 had Botox injections). Postoperative evaluation demonstrated a mean dysphagia score of 0.2, a mean heartburn score of 3.2, and a mean LES pressure of 6.32 mm Hg. Thirty-two patients are maintained on acid-suppressing medications with good control of reflux symptoms. LHM without an antireflux procedure achieves excellent clinical outcomes in most patients with achalasia regardless of previous medical management. Previous medical management may present a greater technical challenge and may place patients at increased risk of mucosal injury.

Achalasia is a well-defined esophageal motor disorder for which pneumatic dilation is an established therapeutic method. Even though it has been used for several years, there are limited data on the long-term outcomes of patients treated with this procedure. Hence, we aimed to evaluate the long-term efficacy of pneumatic dilation to control the symptoms of achalasia.

The medical records of all patients treated in our unit for achalasia with pneumatic dilation were reviewed. We identified the long-term result of the initial procedure, the date of the first dilation, and the time interval between dilation and retreatment.

Of 260 patients who were treated with pneumatic dilation, 153 (67 men, 86 women) were followed up for more than 5 yr. The mean follow-up period was 11.09 +/- 3.91 yr, and the success rate of the dilation was 75.8%. Among these patients, 35 (19 men, 16 women) had follow-up periods of more than 15 yr. The mean follow-up time of those patients was 16.56 +/- 1.09 yr, and the success rate was 51.4%. Kaplan-Meier survival analysis showed that, overall, 50% of patients develop recurring symptoms after 10.92 yr.

Although 51.4% of patients continued to be in clinical remission more than 15 yr after the initial pneumatic dilation, the long-term success rate of pneumatic dilation seems to drop progressively with time.

The aim of this study was to review our results in the surgical management of achalasia by laparoscopic esophageal cardiomyotomy and partial fundoplication.

The patient population was comprised of a consecutive series of 20 patients with classic achalasia who underwent laparoscopic cardiomyotomy and partial fundoplication. Clinical, radiological, and physiological characteristics were analyzed prospectively, with an emphasis on the outcome and complications.

There were 12 women and eight men; their mean age was 37 years. Four intraoperative complications occurred-two mucosal perforations that were resolved laparoscopically and two cases of pneumothorax. The median hospital stay was 4 days (range, 2-14) and the median time to start oral feeding was 3 days (range, 1-7). After a median follow-up of 14 months (range, 2-83), 16 patients were asymptomatic and four had mild heartburn and/or dysphagia. All patients gained weight (median, 8.0 kg; range, 1-23). We observed a median postoperative decrease in esophageal diameter of 1.6 cm (range, 0.2-2.9). Fifteen patients were subjected to physiological esophageal studies; the results showed that power esophageal sphincter pressure had decreased from 32 (range, 15-60) to 12 mmHg (range, 6-25). The median DeMeester score was 14.5 (range, 0.9-194). The median esophageal acid exposure was 3% (range 0-34.6).

Our initial experience with the laparoscopic management of classic achalasia yielded satisfactory clinical, radiological, and physiological results.

Achalasia is a rare neurologic deficit of the esophagus, producing a syndrome of impaired relaxation of the lower esophageal sphincter and decreased motility of the esophageal body for which the cause is unknown. The resultant chronic esophageal stasis produces discomforting symptoms that can be managed with medication, chemical paralysis of the lower esophageal sphincter, mechanical dilation, or surgical esophagomyotomy. Chemical paralysis by injection of the esophagus with botulinum toxin and dilation with an inflatable balloon offers good short-term relief of symptoms; however, the best long-term results are produced by surgery, and advancing minimally invasive techniques continually reduce the morbidity of these operations. The type of surgical procedure, the necessity for fundoplication, and the order of treatment continue to be unresolved issues, but prospective evaluation with objective followup should allow us to provide the optimal treatment regimen to our patients.

Twenty years ago an average of 1.5 Heller myotomies were performed per year in our hospital, mostly for patients whose dysphagia did not improve following balloon dilatation or whose esophagus had been perforated during a balloon dilatation. Ten years ago we started using minimally invasive surgery to treat this disease.

This study measures the impact of minimally invasive surgery with regard to the following: the number of patients referred for treatment; the number of patients who came to surgery without previous treatment; and the results of surgical treatment. Between 1991 and 2001, 149 patients had minimally invasive surgery for achalasia: 25 patients (17%) had thoracoscopic Heller myotomy and 124 (84%) had laparoscopic Heller myotomy and Dor fundoplication. Of the 149 patients, 79 patients (53%) had previous treatment (56 patients [71%], balloon dilatation; 7 patients [9%], botulinum toxin injection; 16 patients [20%], both) and 70 patients (43%) had none of these treatments. Mean postoperative followup was 59 +/- 36 months. Patients were divided into two groups: group A, operated on between 1991 and 1995; and group B, operated on between 1996 and 2001.

In the past decade, the number of patients referred for surgery has increased substantially--group A, 48; group B, 101; an increasing proportion of patients were referred for surgery without previous treatment--group A, 38%; group B, 51%; and the outcomes of the operation progressively improved--group A, 87%; group B, 95%.

These data show that the high success rate of laparoscopic Heller myotomy for achalasia has brought a shift in practice; surgery has become the preferred treatment of most gastroenterologists and other referring physicians. This has followed documentation that laparoscopic treatment outperforms balloon dilatation and botulinum toxin injection.

In the past, surgical treatment in achalasia usually has been reserved for patients whose dysphagia does not respond to pneumatic dilatation. The success of minimally invasive myotomy, however, has resulted in a shift in practice in adult patients, whereby laparoscopic surgery is becoming preferred as primary treatment by most gastroenterologists and surgeons. The aim of this study was to assess the efficacy of laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia in children.

Thirteen patients with esophageal achalasia (median age, 15 years; 6 boys and 7 girls; median duration of symptoms, 24 months) underwent laparoscopic Heller myotomy and Dor fundoplication between 1996 and 1999. Two patients had been treated previously by pneumatic dilatation, and 1 patient had received intrasphincteric Botulinum toxin injections.

Median duration of the operation was 130 minutes. The patients were fed after an average of 33 hours, and they all left the hospital within 2 days. At a median follow-up of 19 months, there was no residual dysphagia in any patient.

Laparoscopic Heller myotomy and Dor fundoplication were effective and safe for children with esophageal achalasia. Hospital stay and recovery time was short, and the functional results were excellent. These data support the notion that laparoscopic Heller myotomy should become the primary treatment of esophageal achalasia in children.

Malignant pseudoachalasia can be indistinguishable from primary achalasia on routine clinical evaluation, often resulting in a delay in diagnosis. To better define the clinical features and appropriate management of this disease, the course of five patients discovered to have pseudoachalasia after being referred for a minimally invasive Heller myotomy was reviewed, as were 67 cases of pseudoachalasia previously reported in the literature. Patients with an occult malignancy tended to present with shorter durations of symptoms, greater weight loss, and at a more advanced age than patients with primary achalasia. Since contrast radiography and endoscopy frequently failed to differentiate these two diseases, persons with presumed achalasia meeting these criteria who are referred for minimally invasive surgery should undergo additional imaging to rule out an occult malignancy, since this condition cannot be reliably detected during the course of a thoracoscopic or laparoscopic esophagomyotomy.

The aim of this retrospective study was to determine the risk factors of early complications after progressive pneumatic dilation for achalasia.

Five hundred four dilations were performed in 237 consecutive achalasic patients between 1980 and 1994 with the same technique: low-pressure pneumatic dilation every other day with balloons of increasing diameter until success criteria were obtained (depending on manometric examination and esophageal x-ray transit performed 24 h after each dilation). Clinical, radiographical, endoscopical, and manometrical data as technical procedure characteristics for patients with perforations or other early complications were compared with those without complications.

We observed 15 complications (6% of patients): 7 perforations (3%), 3 asymptomatic esophageal mucosal tears, 4 esophageal hematomas, and 1 fever. Perforations occurred in 6 of 7 patients during the first dilation. The mean age was 68.5 yr in the group with complications versus 56.4 yr for the remainder (p < 0.05). Two deaths occurred in patients older than 90 yr. Instability of the balloon during dilations was noted in 8 of 15 cases of complications versus 57 of 222 patients (p < 0.05). No other data differed significantly.

This study showed a low prevalence of early complications using this progressive technique. Patients with hiatal hernia, esophageal diverticulum, or vigorous achalasia may safely undergo progressive pneumatic dilation. Only patients older than 90 yr should be referred for progressive pneumatic dilation with caution. Most of perforations arose during the first dilation, but there was no predictive pretherapeutic factor of perforation.

Surgical myotomy is the mainstay of treatment for oesophageal achalasia. Minimally invasive surgical techniques, if feasible, reduce patient morbidity and mortality. In this study we review our experience of thoracoscopic Heller's myotomy. Thoracoscopic myotomy was undertaken in 9 patients (male = 3; female = 6, mean age = 37). All patients presented with dysphagia of 1 to 8 yr duration. Diagnosis was based on barium swallow and manometry. Two patients had previous dilatations and 1 had a transabdominal myotomy. All patients had a 5 port thoracoscopic technique. Thoracoscopic Heller's myotomy was completed in 8 out of 9 patients. In 1 patient extensive oesophagitis and peri-oesophagitis precluded both a thoracoscopic and an open myotomy, and oesophagectomy was subsequently performed. The mean duration of surgery was 142 min. Completion of myotomy and mucosal integrity was confirmed by intraoperative gastroscopy. All patients had an uneventful post-operative recovery. The mean hospital stay was 4 days. All patients are now asymptomatic, with documented weight gain. No patients have reflux oesophagitis symptoms. Our preliminary experience would suggest that thoracoscopic Heller's myotomy is a safe alternative to open surgery, with satisfactory results and reduced hospital stay.

Achalasia is a primary esophageal motor disorder of unknown etiology producing complaints of dysphagia, regurgitation, and chest pain. The current treatments for achalasia involve the reduction of lower esophageal sphincter (LES) pressure resulting in improved esophageal emptying. Calcium channel blockers and nitrates, once used as initial treatment strategy for early achalasia, are now only used in patients who are not candidates for pneumatic dilation or surgery and those not responding to botulinum toxin injections. By virtue of the more rigid balloons, the current pneumatic dilators are more effective and have better efficacy than the older more compliant balloons. The graded approach to pneumatic dilation using the Rigiflex balloons (3.0, 3.5, and 4.0 cm) are now the most commonly used nonsurgical means of treating patients with achalasia, resulting in symptom improvement in up to 90% of patients. Surgical myotomy, once with high morbidity and long hospital stay, can now be performed laparoscopically with similar efficacy to the open surgical approach (94% vs. 84%, respectively), reduced morbidity, and hospitalization time. Given the advances in both balloon dilation and laparoscopic myotomy, most patients with achalasia can now choose between these two equally efficacious treatment options. Botulinum toxin injection of the LES should be reserved for patients who cannot undergo balloon dilation and are not surgical candidates.

Recently, investigators have reported the use of endoscopic myotomy in the treatment of esophageal achalasia. As with the open operation, considerable disagreement exists regarding the appropriate length of the myotomy and the need for a concomitant antireflux procedure.

Patients presenting with symptomatic achalasia between 1993 and 1997 were included in this prospective study. Preoperative studies included barium upper gastrointestinal study, endoscopy, and esophageal manometry. Laparoscopic myotomy was completed in all 20 patients; 18 had concomitant Toupet fundoplication.

Operative times ranged from 95 to 345 minutes (mean 216). Blood loss ranged from 50 to 300 cc (mean 100 cc). There were 7 minor complications (5 mucosal injuries repaired laparoscopically, 1 bile leak and 1 splenic capsular tear). Nine patients began a liquid diet on the first day postoperatively; 19 were tolerating liquids by postoperative day 3. Hospital stay ranged from 2 to 20 days (mean 5). Eighteen patients had complete relief of dysphagia, with less than one reflux episode per month. One individual continues to have mild persistent solid food dysphagia. Another patient initially did well but subsequently developed mild recurrent dysphagia and reflux. One patient required laparoscopic take-down of the wrap because of recurrent dysphagia and now has no problems swallowing, but does complain of mild reflux. Two other patients also have mild reflux, 1 of whom did not undergo fundoplication.

Laparoscopic Heller myotomy can be performed safely with excellent results in patients with achalasia. Adding a partial fundoplication appears to help control postoperative symptoms of reflux. This procedure should be considered the procedure of choice in patients with symptomatic esophageal achalasia.

Gastroesophageal reflux (GER) can develop in patients with esophageal achalasia either before treatment or following pneumatic dilatation or Heller myotomy. In this study we assessed the value of pre- and postoperative pH monitoring in identifying GER in patients with esophageal achalasia. Ambulatory pH monitoring was performed preoperatively in 40 patients with achalasia (18 untreated patients and 22 patients after pneumatic dilatation), 27 (68%) of whom complained of heartburn in addition to dysphagia (group A), and postoperatively in 18 of 51 patients who underwent a thoracoscopic (n=30) or laparoscopic (n=21) Heller myotomy (group B). The DeMeester reflux score was abnormal in 14 patients in group A, 13 of whom had been treated previously by pneumatic dilatation. Two types of pH tracings were seen: (1) GER in eight patients (7 of whom had undergone dilatation) and (2) pseudo-GER in six patients (all 6 of whom had undergone dilatation). Therefore 7 (32%) of 22 patients had abnormal GER after pneumatic dilatation. Postoperatively (group B) seven patients had abnormal GER (6 after thoracoscopic and 1 after laparoscopic myotomy). Six of the seven patients were asymptomatic. These findings show that (1) approximately one third of patients treated by pneumatic dilatation had GER; (2) symptoms were an unreliable index of the presence of abnormal GER, so pH monitoring must be performed in order to make this diagnosis; and (3) the preoperative detection of GER in patients with achalasia is important because it influences the choice of operation.

Although achalasia is not a common illness in the United States and Europe, there continues to be a need for surgical therapy for treatment. Laparoscopic Heller myotomy and partial fundoplication has, for the most part, replaced open surgery (abdominal or thoracic) as the surgical treatment of choice. In order to perform this procedure well, one must select patients carefully, evaluate them fully, and adhere to the technical principles required to achieve consistently good results.

The goal of this study was to review the authors' results with laparoscopic cardiomyotomy and partial fundoplication for achalasia.

Pneumatic dilatation and botulinum toxin (BOTOX) injection of the lower esophageal sphincter largely have replaced cardiomyotomy for treatment of achalasia. After a brief experience with a thoracoscopic approach, the authors elected to perform cardiomyotomy laparoscopically, in combination with a partial fundoplication (anterior or posterior).

Forty patients were treated between July 1992 and November 1996. Thirty patients had previous therapy of achalasia, 21 with pneumatic dilation, 1 with BOTOX, 6 with balloon and BOTOX, and 2 with transthoracic cardiomyotomy. Three patients had previous laparoscopic fundoplication for gastroesophageal reflux. Symptom scores (0 = none to 4 = disabling) were obtained before surgery and after surgery. Barium swallows and esophagogastroduodenoscopy were performed in all patients. Esophageal motility study was performed in 36 patients. Laparoscopic Heller myotomy and fundoplication was performed through five upper abdominal trocars. A 7-cm myotomy extended 6 cm above the GE junction and 1 cm below the GE junction. A posterior fundoplication was performed in 32 patients, anterior fundoplication in 7 patients, and no fundoplication in 1 patient. Statistical inference was performed with a Wilcoxon signed rank test.

Mean operative duration was 199 +/- 36.2 minutes. Mean hospital stay was 2.75 days (range, 1-13 days). Dysphagia was alleviated in all but four patients (90%), and regurgitation in all but two patients (95%) (p < 0.001). Chest pain and heartburn improved significantly (p < 0.01) as well. Intraoperative complications included mucosal laceration in six patients and hypercarbia in one. Postoperative pneumonia developed in two patients, and one patient had moderate hemorrhage from an esophageal ulcer 2 weeks after surgery.

Laparoscopic cardiomyotomy and fundoplication appears to provide definitive treatment of achalasia with rapid rehabilitation and few complications.

From 1974 to 1995, 19 children with achalasia of the esophagus have been treated at our institution. Presenting symptoms included vomiting (n = 14), dysphagia (n = 13), failure to thrive (n = 6), and odynophagia (n = 1). Diagnosis was established by a barium swallow in 19, with eight also undergoing esophageal manometry. Six boys and 13 girls with an average age of 10 years (range, 1.3 to 17.6) underwent a transthoracic, modified anterior Heller esophagomyotomy (HM). Five underwent a concomitant, modified, Belsey fundoplication (BF). Follow-up ranging from 6 months to 21 years (mean, 9 years) was accomplished in all 19 patients by both office visits and telephone interviews. Early postoperative follow-up showed initial swallowing difficulty in two (14%) patients with a HM alone and in four out of five (80%) patients treated with a HM and BF. All patients (n = 5) with a HM and BF and one with a HM alone required one esophageal dilation during the first postoperative year. These initial swallowing difficulties resolved in all six patients during this first postoperative year. Late postoperative follow-up, however, indicates occasional, mild dysphagia in two out of five with an HM and BF resulting in complete relief of presenting symptoms in 17 of the 19 patients (90%). All patients rated their overall result as either excellent (68%) or good (32%) with none rating it as fair or poor. None of the 19 patients had clinical evidence of gastroesophageal reflux, although five patients had evidence of nonpathologic reflux noted during upper gastrointestinal x-ray. Recurrent vomiting, asthma, wheezing, or esophagitis symptoms have not been reported by any patients. No patients required reoperation, and there were no deaths or postoperative complications. Modified Heller esophagomyotomy is safe (0% mortality) and effective (90% relief of symptoms) in children with achalasia. A concurrent modified Belsey fundoplication results in early and late mild postoperative dysphagia that was responsive to esophageal dilation. The transthoracic, modified Heller esophagomyotomy without a fundoplication is currently our treatment of choice for achalasia in children.

During nine years, 157 consecutive patients with achalasia have been dilated in our unit. First, the long-term effect of dilation on clinical status was evaluated. The probability of being in clinical remission eight years after first dilation was 51%. The pressure of the LES measured after dilation was highly predictive of the long-term clinical evolution. Second, a predictive model of the individual response to pneumatic dilation was developed and simplified. Therapy was effective in 80% of the patients, after one to four dilations. Younger age was the only factor significantly associated with ineffective therapy. Depending on the prognosis of the outcome calculated with the predictive model, patients were classified in groups of risk that showed a different rate of ineffective, therapy. In the simplified model, age < or = 20 years, male gender, esophageal body diameter < or = 3 cm, esophageal body basal pressure > 15 mm Hg, and pressure of the lower esophageal sphincter > 30 mm Hg were predictors of a poor response to dilation. We conclude that pneumatic dilation is an effective therapy for achalasia. A predictive model was useful to classify the patients in groups with a different risk for ineffective dilation. A simplification of this model could be used to predict the response to dilation.

Cardiomyotomy for achalasia is one of the ideal procedures for the video endoscopic approach. Magnification of the operative field during laparoscopic surgery allows precise division of the muscle fibers with excellent results. The number of reports on cardiomyotomy performed with laparoscopic (and thoracoscopic) access is growing. They all show the same excellent results as for conventional (open) myotomy, with minimal morbidity, short hospital stay, and early return to routine activity.

The reproducibility of ambulatory 24-h esophageal pH monitoring was evaluated in 16 consecutive patients by comparing the difference in two consecutive 24-h periods. The study group included 8 patients with scleroderma esophagus and 8 treated achalasia patients. The amount of reflux was expressed as the percentage of time the pH was < 4.0. Both groups demonstrated excellent intrapatient reproducibility overall: 96% in scleroderma patients and 95% in those patients with achalasia. The least concordance was found in the lengths of the longest reflux events-70% when supine in scleroderma patients and 59% when upright in patients with achalasia. There was no significant difference (p > 0.05) between day 1 and day 2 for either group of patients for any of the elements studied. These results indicate that intrapatient variability of gastroesophageal reflux in patients with scleroderma esophagus and treated patients with achalasia is very low and following therapeutic intervention, a high level of confidence can be placed in subsequent pH monitoring as an indicator of treatment effect.

A case of gasless laparoscopic esophagogastric myotomy for achalasia is presented. The technical aspects of the technique as well as the benefits of this approach are reviewed.

Forceful dilatation under endoscopic control is a well established treatment of achalasia; several different types of dilators can be used. This study prospectively compared the clinical and manometric efficacy of a single dilatation using two different dilators. Forty one patients were randomly assigned to forceful dilatation under endoscopic control with either a pneumatic dilator (n = 17) or a metallic dilator (n = 24). Thereafter, the patients received periodic clinical and manometric evaluation for one year (before and one, six, and 12 months after dilatation). One month after dilatation all but one of the subjects in each group had experienced good to excellent results and their clinical improvement persisted for the one year follow up. Two patients (one in each group) were perforated during the procedure and required surgical treatment. Recovery was uneventful in both cases. Resting lower oesophageal sphincter pressure (mean (SEM)) significantly and similarly decreased after both methods of dilatation (pneumatic dilator: before dilatation 37 (3) mm Hg, one year after dilatation 18 (3) mm Hg; metallic dilator: before dilatation 34 (2) mm Hg, one year after dilatation 17 (3) mm Hg; p < 0.05 for both). It is concluded that in the treatment of achalasia a single dilatation under endoscopic control with either pneumatic or metallic dilator yield comparable clinical and manometric results and similar complication rates. The use of one or other dilator should depend more on the preference and experience of the endoscopist than on the type of device.

It has been shown that nasal continuous positive airway pressure (nasal CPAP) significantly reduces nocturnal reflux both in patients with sleep apnea and in patients without sleep apnea but consistent abnormal nocturnal reflux. The mechanism by which CPAP is thought to reduce reflux includes the elevation of the resting lower esophageal sphincter (LES) pressure. In this study, we tested the effect of nasal CPAP in two groups of patients with aperistaltic esophagus but with different resting LES pressure. Seven patients with scleroderma esophagus and six patients treated for achalasia were tested over a 48-h period. On the first night, the patients were untreated; on the second night, both groups received applied nasal CPAP at 8 cm H2O pressure. The percentage of time the pH < 4.0, the number of reflux events > 5 min, and the length of the longest reflux event were all significantly reduced in the patients with achalasia (p < 0.03), but not in the scleroderma group (p > 0.20). These results suggest that a residual resting LES pressure greater than that demonstrated by patients with scleroderma (> 10 mm Hg) may be necessary for nasal CPAP to affect nocturnal reflux.

A retrospective study carried out on 74 patients among 101 consecutive cases of achalasia of the esophagus operated from 1967 to 1989 is reported. On 21 patients observed between 1967 and 1975, a standard transabdominal Heller cardiomyotomy was performed (group A). From 1976 to 1989, the treatment of choice was a Heller myotomy associated with a modified Dor's fundoplication. In 80 consecutive cases (group B) the extension of myotomy was regulated by intraoperative monitoring of lower esophageal sphincter pressure. A 5-year follow-up with questionnaires, physical examination, and barium swallows was carried out on 16 patients in group A and on 58 patients in group B. In 75.6% of the cases (56 patients) follow-up examinations included esophageal manometry and 24-hour esophageal pH monitoring. Recurrence of dysphagia was recognized in 3 cases in group A (18.7%) and in 2 cases in group B (3.4%) (P = 0.053); postoperative gastroesophageal reflux, measured as a percentage of total reflux time, showed a significantly lower mean value in group B than in group A (1.8% vs. 4.1%, P < 0.01). This study suggests that an anti-reflux procedure lowers postoperative gastroesophageal reflux after Heller myotomy. Due to the low incidence of postoperative reflux and the negligible recurrence of dysphagia, Heller myotomy associated with a modified Dor's fundoplication may represent the surgical treatment of choice for achalasia of the esophagus.

We treated 24 patients with achalasia using thoracoscopic (22 patients) or laparoscopic (2 patients) esophagomyotomy. The only operative complications were mucosal lacerations, which occurred in 3 patients and required conversion to an open procedure in 2. Twenty-two (91%) patients were eating by the second postoperative day. Analgesics were only required for the management of pain from the chest tube, which remained in place for a median time of 24 hours. The median postoperative hospital stay was 3 days (range, 20 to 14 days). The myotomy proved to be incomplete in the first 3 patients, who required a second myotomy; this was done laparoscopically in 2. One patient had a paraesophageal hernia repaired 6 months after the myotomy, and 1 patient required an esophagectomy 1 year after the myotomy for a large nonfunctioning esophagus. Late follow-up showed that swallowing was excellent in 17 (71%) and fair to good in 4 (17%). Sixteen (66%) of these 24 patients have regained their original weight. Thus, excellent to good results were ultimately obtained in nearly 90% of the patients. These results suggest that esophageal myotomy performed using minimally invasive techniques appears to be the treatment of choice for achalasia.

Although esophageal perforation complicates about 5% of pneumatic dilatations performed for achalasia, little is known about associated hospital and long-term courses. In order to assess the outcome of such patients undergoing emergency surgery for repair, records of seven patients sustaining perforation during pneumatic dilatation were compared to those of five patients undergoing elective myotomy during the same period. In perforation patients, mean intervals following the procedure were 3.6 hr to administration of antibiotics and 9.6 hr to surgery. The perforation and elective myotomy groups had similar mean durations of operation (3.8 vs 3.3 hr), intensive care stays (2 vs 1 days) and hospitalization (12 vs 11 days); perforation patients had a significantly longer mean interval from surgery to oral intake (7 vs 5 days). Postdischarge long-term outcomes were alike in the groups. It is concluded that patients with perforation from pneumatic dilatation that is recognized and treated promptly have outcomes that are comparable to those of patients who undergo elective myotomy.

The choice between pneumatic dilatation and surgical esophagomyotomy as the initial treatment for achalasia is controversial. The aims of this study were to determine the long term clinical outcome and costs of treating achalasia initially with pneumatic dilatation as compared to esophagomyotomy. Of 123 patients undergoing an initial pneumatic dilatation for achalasia at our institution from 1976 to 1986, 71 (58%) received no further treatment for achalasia during a mean follow up of 4.7 +/- 2.8 years. Only 15 of these 123 patients (12%) eventually underwent surgical esophagomyotomy (two for perforation during pneumatic dilatation, 13 for persistent or recurrent symptoms). The degree of dysphagia at follow up was improved to a similar degree in patients treated with an initial pneumatic dilatation as compared to patients treated with an initial esophagomyotomy. Patients with age > or = 45 years at time of initial pneumatic dilatation had fewer subsequent treatments for persistent or recurrent symptoms and had less dysphagia on follow up as compared to patients < 45 years. Subsequent pneumatic dilatations to treat persistent or recurrent symptoms were less beneficial than an initial pneumatic dilatation. The cost of esophagomyotomy was 5 times greater than the cost of pneumatic dilatation. When costs were analyzed to include subsequent treatments of symptomatic patients, the total expectant costs of treating with an initial esophagomyotomy remained 2.4 times greater than treating with an initial pneumatic dilatation. This study suggests that an initial pneumatic dilatation will be the only treatment needed for the majority of patients with achalasia. A treatment regimen starting with pneumatic dilatation has less overall costs than starting with esophagomyotomy. For each subsequent pneumatic dilatation, however, the clinical benefit leans toward surgery.

The long-term prognosis of expanding bag dilatation therapy using a Matsuo pneumatic bag dilator was evaluated in 163 cases of esophageal achalasia treated by this method over the 26-year period from 1964 to 1989. In all these cases, one year or more had passed since therapy. Practically no correlation was found between the efficacy of the therapy and the grade of esophageal dilation prior to therapy, the previous history of symptomatic distress or the number of dilatations performed. The efficacy of expanding bag cardial dilatation was most obvious in the increase of body weight, 59 cases (36.2%) showing an increase of 1-5 kg and 48 cases (29.4%) showing an increase of 6-10 kg. The therapy was rated "highly effective" in 61 cases (37.4%) and "effective" in 60 cases (36.8%), i.e. it was effective in a total of 121 cases (74.2%). It was rated as being "ineffective" in 16 cases (9.8%) including 4.3% of cases in which an operation had been performed. This indicated that surgical operation of esophageal achalasia should be performed in those cases in which good long-term results were not obtained even after expanding bag dilatation therapy had been carried out several times.

The authors treated 17 patients with achalasia by a thoracoscopic (15 patients) or laparoscopic (2 patients) Heller myotomy. All patients had dysphagia and an upper gastrointestinal series demonstrating a dilated esophagus with a bird-beak deformity at the cardia. Manometry showed a mean lower esophageal sphincter (LES) pressure of 32 +/- 4 mmHg, incomplete sphincter relaxation on swallowing, and no primary esophageal peristalsis. After operation, mean LES pressure was 10 +/- 2 mmHg. Fifteen patients were fed on the second postoperative day. The average hospital stay was 3 days, and there were no deaths or major complications. In three early patients, the myotomy was not carried far enough onto the stomach, and dysphagia persisted until a second myotomy was performed (laparoscopically in two patients). The authors found that having an endoscope in the esophagus during the operation facilitated exposure and was vital to determine the appropriate length of the myotomy. With regard to dysphagia, final results were excellent in 12 patients (70%), good in two patients (12%), fair in two patients (12%), and poor in one patient (6%). Heller myotomy can be safely and reliably performed with minimally invasive techniques. Dysphagia is relieved, postoperative pain is minimal, hospital stay is short, and the patient can return quickly to normal activity.

Balloon dilation of gastrointestinal strictures using a radiologic, endoscopic or combined approach is a safe, effective means of managing an ever-increasing variety of stricturing processes. At present the ability to dilate strictures in the gastrointestinal tract is limited mainly by access. Balloon dilation is now well established in the management of oesophageal and anastomotic lesions. The place of balloon dilation in the management of Crohn's disease and in the management of malignant disease requires further evaluation.

Perforation of the esophagus remains a diagnostic and therapeutic challenge. Currently, the most common cause of perforation is instrumentation of the esophagus, and the incidence of esophageal perforations has increased as the use of endoscopic procedures has become more frequent. Diagnosis depends on a high degree of suspicion and recognition of clinical features, and is confirmed by contrast esophagography or endoscopy. Outcome after esophageal perforation is dependent on the cause and location of the injury, the presence of underlying esophageal disease, and the interval between injury and initiation of treatment. Reinforced primary repair of the perforation is the most frequently employed and preferable approach to the surgical management of esophageal perforations. Nonoperative management consisting of antibiotics and parenteral nutrition is particularly successful for limited esophageal injuries meeting proper selection criteria.

The current evaluation of and therapy for achalasia are reviewed. Esophageal manometry remains the best means for diagnosing achalasia. Initial therapy can include either pneumatic dilation or esophagomyotomy. Symptomatic improvement occurs in 71% of patients after pneumatic dilation, with a risk of perforation of 1.4%. Eight percent of these patients require subsequent esophagomyotomy. Surgical procedures for achalasia can be performed through either an abdominal or a thoracic incision. Nearly all authors favoring an abdominal approach add an antireflux operation to esophagomyotomy, whereas many authors advocating a transthoracic esophagomyotomy believe that an antireflux wrap is unnecessary. Overall results for the various surgical approaches used as initial therapy are excellent, with symptomatic improvement in 89% of patients, a mortality rate of less than 1%, and development of gastroesophageal acid reflux in less than 10%. Factors governing the choice of initial therapy are discussed.

Achalasia is the best understood of the motor disorders described in this chapter. The pathogenesis involves loss of intramural neurones, a process that subsequently results in poor lower sphincter relaxation and atony of the oesophageal body. Treatment is appropriately focused on mechanical or pharmacological alleviation of LOS obstruction. In contrast, the pathophysiology of DOS and the non-specific disorders remains poorly understood. Some of the non-specific disorders, such as the vigorous contraction wave abnormalities (including 'nutcracker oesophagus'), appear closely related to DOS. Treatment for patients with these findings has been based on assumptions about mechanisms of symptom production. The non-specific disorders are common in referred patients with oesophageal symptoms, and the importance of these findings deserves further study. We use a method for categorization of these manometric abnormalities which aids understanding of this difficult area and recommend its more widespread use.