Barrett's esophagus and achalasia

Two unusual cases of achalasia with endoscopic and histologic documentation of Barrett's esophagus are presented. One patient had Barrett's esophagus at the time of initial endoscopy for achalasia, before any treatment. The other patient developed specialized columnar epithelia in the esophagus after treatment with pneumatic dilation. Each patient had evidence of low-grade dysplasia. Including these two patients, 30 cases of Barrett's esophagus in patients with achalasia have been reported in the literature. In 73% (22 of 30) of the cases, Barrett's esophagus was detected after esophagomyotomy. In 20% (6 of 30) of the cases of achalasia and Barrett's esophagus, adenocarcinoma developed. The current two cases are unusual because Barrett's esophagus in achalasia generally develops from gastroesophageal reflux after esophagomyotomy. No other patients have been reported to develop Barrett's esophagus after pneumatic dilation alone. Patients with achalasia and Barrett's esophagus may be at a particularly high risk for developing dysplasia and adenocarcinoma.     

Esophageal adenocarcinoma in a patient with surgically treated achalasia

Summary Although squamous cell carcinoma of the esophagus occurs with increased incidence in primary achalasia, esophageal adenocarcinoma has been considered rare in this condition. We report a patient with long-standing achalasia in whom adenocarcinoma of the esophagus occurred many years after Heller esophagomyotomy, presumably related to Barrett's esophagus complicating gastroesophageal reflux disease.     

Barrett''s Esophagus in a Patient with Achalasia

Barrett's esophagus has been reported in patients with achalasia who have undergone esophagomyotomy. The condition was thought to be acquired from gastroesophageal reflux secondary to the iatrogenically produced incompetent sphincter. We present the case of a patient with Barrett's esophagus and achalasia without any previous surgical intervention.     

Heller''s esophagomyotomy with or without a 360° floppy Nissen fundoplication for achalasia. Long-term results from a prospective randomized study

Heller's esophagomyotomy relieves dysphagia but does not restore esophageal peristalsis. The myotomy may induce reflux and the addition of a 360 degrees fundoplication may be hazardous with regard to the remaining aperistaltic esophagus. The aim of this prospectively randomized clinical trial was to compare the outcome for patients with uncomplicated achalasia who underwent an anterior Heller's esophagomyotomy (H group) with or without an additional floppy Nissen fundoplication (H + N group). Between 1984 and 1995, 20 patients were prospectively randomized to one or other of the performed operations, 10 patients per group. Esophagitis including Barrett's esophagus (n = 2) was seen under medical treatment, in 6 of 9 in the H group but none in the H + N group. No patient in the H + N group required postoperative continuous acid-reducing drugs. Twenty-four-hour esophageal pH-studies in median 3.4 years after surgery showed pathological reflux expressed as a percentage of time below pH 4 of 13.1% in the H group compared to 0.15% (P < 0.001) in H + N group. One patient with recurrent dysphagia in the H + N group later had an esophagectomy. The remaining patients reported significant improvement of dysphagia without symptoms of reflux at 8.0 years follow-up. Heller's esophagomyotomy eliminates dysphagia, but can induce advanced reflux that requires medical treatment. The addition of a 360 degrees fundoplication eliminates reflux without adding dysphagia in the majority of patients and can be recommended for most patients with uncomplicated achalasia.     

Carcinoma associated with achalasia

Carcinoma developed in a 67-year-old woman with achalasia of the esophagus 23 years after esophagomyotomy. Postoperative manometric and radiologic studies showed satisfactory relief of esophageal obstruction. The development of carcinoma after an unusually long interval after adequate surgical treatment emphasizes the need for lifelong surveillance for this complication.     

Long-term clinical, radiological and manometric follow-up of patients with achalasia of the esophagus treated with esophagomyotomy.

Twenty adult patients with achalasia of the esophagus were treated with an anterior esophagomyotomy and fundic patch. A significant decrease in resting gastroesophageal sphincter pressure was obtained immediately after surgery (p less than 0.001). One case was lost and the rest were followed up for 34 months. Patients had no esophageal symptoms and resting gastroesophageal sphincter pressure remained in low values. Surgical procedure is an adequate and definitive treatment for this disease.     

Barrett's esophagus and achalasia. A case report.

A 70-year-old woman with no previous gastroesophageal surgery gave a 6-month history of dysphagia. Barium studies suggested a diagnosis of achalasia. Esophageal manometry showed absence of peristalsis and a high lower esophageal sphincter pressure. Endoscopy showed a dilated esophagus with food residue, and Barrett's esophagus was present. The association of Barrett's esophagus and achalasia must be rare.     

Pneumatic dilatation or esophagomyotomy treatment for idiopathic achalasia: Clinical outcomes and cost analysis

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 age45, 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 dilation. 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 penumatic dilatation has less overall costs than starting with esophagomyotomy. For each subsequent pneumatic dilatation, however, the clinical benefit leans toward, surgery.     

The cost-effectiveness of treatment strategies for achalasia

Achalasia is a disorder characterized by abnormal motility of the esophageal body and the lower esophageal sphincter, resulting in dysphagia, regurgitation, and chest pain. Treatment options for achalasia include Botulinum toxin injection, pneumatic balloon dilation, and surgical esophagomyotomy. The aim of this study was to determine the cost-effectiveness of these three strategies in the treatment of achalasia in adults. We constructed a Markov cost-effectiveness model comparing Botox injection, pneumatic balloon dilation, and laparoscopic esophagomyotomy as initial treatments of achalasia. Costs and probabilities were derived from the published literature. The utility for symptomatic achalasia was derived from a sample of patients with achalasia. Sensitivity analyses were performed. Over a five-year time horizon, pneumatic dilation was the most cost-effective treatment strategy for achalasia, with an incremental cost-effectiveness ratio of $1348 per quality-adjusted life-year compared to Botox. Although laparoscopic esophagomyotomy was more effective than the other treatment options, it was not cost-effective because of its high initial cost. In conclusion, pneumatic dilation is the most cost-effective treatment option for adults with achalasia. Further studies should examine the long-term relapse rates following treatment with Botox and more precisely determine the quality of life of symptomatic achalasia.     

Lugol staining and histological evaluation of esophageal mucosa in achalasia

BACKGROUND/AIMS: Esophageal cancer in achalasia is often diagnosed in the advanced stage, which makes for a poor prognosis. Therefore, the aim of this study was to evaluate the macroscopic and histological features of the esophageal mucosa in order to improve the early detection of cancer. METHODOLOGY: We studied the macroscopic features of esophageal mucosa using Lugol's solution and compared them with histological analysis of the entire mucosa in 20 esophagectomy specimens resected for achalasia. Intraepithelial neoplasia, when detected, was selected for DNA ploidy analysis through static cytometry. RESULTS: Macroscopically, the mucosa showed opacification and/or diffuse irregularities in 19 specimens. Advanced squamous cell carcinoma was diagnosed in 2 cases. Using Lugol, the esophageal mucosa acquired irregular brownish color. Clear unstained areas were circumscribed in 5 esophagi. They were macroscopically defined as ulcer, neoplasia (2 cases) and mucosal irregularities (2 cases). The histological analysis showed ulcer, squamous cell carcinoma (2 cases), Barrett's esophagus and esophagitis, respectively. The histological study of the stained mucosa revealed minute foci of DNA aneuploid intraepithelial neoplasia in 4 cases. CONCLUSIONS: Macroscopic examination using Lugol failed to identify minute foci of early carcinoma. The stained mucosa does not exclude the esophageal cancer risk in achalasia.     

Nonoperative management of esophageal strictures following esophagomyotomy for achalasia

The optimal management of reflux-induced esophageal strictures that occur after esophagomytomy for achalasia is uncertain. This paper presents our experience with the nonsurgical treatment of postesophagomyotomy strictures in achalasia patients using endoscopic dilation and gastric acid suppression. Six patients with achalasia who had undergone prior esophagomyotomy subsequently developed recurrent dysphagia and were found to have an esophageal stricture. Esophagrams typically showed a markedly dilated esophagus with a narrowed, sharply angulated gastroesophageal junction. Esophageal manometry confirmed esophageal aperistalsis and, when measured, the LES pressure was<5 mm Hg. Endoscopy showed esophageal inflammation and a fixed stricture at the gastroesophageal junction. Strictures were dilated under direct visualization using through-the-scope balloon dilators. Patients began gastric acid suppressive treatment at the same time. Five patients who remained symptomatic underwent repeat endoscopy, which demonstrated improvement in esophagitis. Dilation was then repeated with a larger balloon dilator. Over a mean follow-up period of 3.8 years, the average number of repeat dilations per patient was 3.6 (range: 0–12). All patients had symptomatic improvement and weight gain. No patient required surgery. We conclude that esophageal strictures after esophagomyotomy for achalasia can be safely treated using endoscopic dilation and gastric acid suppression, thus avoiding the need for reoperation.     

Comparison of forceful dilatation and esophagomyotomy in patients with achalasia of the esophagus.

In the present paper we have reviewed the results of forceful dilatation as compared with surgical esophagomyotomy in patients with achalasia. The review of 4 retrospective and uncontrolled studies revealed that in all, surgery produced a more effective late result than dilatation, with minimal side effects and very low or no mortality. In the only prospective randomized study comparing both treatments by the same group, good late results were seen after surgery in 95% of the cases, as compared with 65% after dilatation (p less than 0.001). The resting gastroesophageal sphincter pressure was predictive of the quality of the late results. Reflux occurred in 8% of the dilated and in 19% of the operated group as measured by standard acid reflux test. The old, classical concept that dilatation is the first choice and preferable method of treatment for patients with achalasia should be reviewed, and the idea that surgery should be reserved only for patients in whom dilatation has failed should be abandoned. We propose that surgical treatment should be the initial choice in the majority of patients with achalasia of the esophagus.     

Controversies in the treatment of gastroesophageal reflux and achalasia

The immense success of laparoscopic surgery as an effective treatment of gastroesophageal reflux disease (GERD) and achalasia has established minimal invasive surgery as the gold standard for these two conditions with lower morbidity and mortality, shorter hospital stay, faster convalescence, and less postoperative pain. One controversy in the treatment of GERD evolves around laparoscopic antireflux surgery (LARS) as the preferred treatment for Barrett's esophagus and the procedure's potential to reduce the risk of adenocarcinoma of the esophagus. GERD has also been associated with respiratory symptoms, asthma and laryngeal injury, and a second controversy prompts discussions about whether total or partial fundoplication is the more appropriate treatment for GERD. A new and promising alternative in the treatment of GERD is endoluminal therapy. Three types of this new treatment option will be discussed: radiofrequency energy delivered to the lower esophageal sphincter, the creation of a mechanical barrier at the gastroesophageal junction, and the direct endoscopic tightening of the lower esophageal sphincter. Laparoscopic surgery is discussed not only as a very effective treatment for GERD but also as permanent cure for achalasia. This review analyzes the three most important treatment options for achalasia: medications, pneumatic dilatation, and surgical therapy. Medications as the only true non-invasive option in the treatment of achalasia are not as effective as LARS because of their short half-life and variable absorption due to the poor esophageal emptying. The second treatment option, pneumatic dilatation, involves the stretching of the lower esophagus and is still considered the most effective non-surgical treatment for achalasia. Finally, surgical therapy for achalasia and the two major controversies concerning this laparoscopic treatment are discussed. The first involves the extent to which the myotomy is extended onto the stomach, and the second concerns the necessity and type of antireflux procedure to prevent GERD after myotomy. LARS and laparoscopic Heller myotomy are the agreed upon as the gold standards for surgical treatment of GERD and achalasia, respectively. In the hands of an experienced laparoscopic surgeon both are safe and effective treatments for patients with excellent subjective and objective long-term results with at least 90% patient satisfaction.     

Pneumatic dilation in achalasia.

Pneumatic dilation is the most common first-line therapy for the treatment of achalasia. The aim of dilation is a controlled disruption of circular muscle fibres of the lower esophageal sphincter to reduce the functional obstruction. Several types of dilators and different dilation techniques are used, but the achieved results are similar. The mean success rate is about 80% in the short term, but some patients need redilation in the further course (particularly young patients). Best long term results are obtained if the lower esophageal sphincter pressure can be reduced below 10 mmHg. Major complications are rare after pneumatic dilation; the most serious complication is esophageal perforation, which occurs at a mean rate of about 2.5%. Considering the pros and cons of other effective forms of treatment of achalasia (esophagomyotomy and intrasphincteric injection of botulinum toxin), pneumatic dilation is still the treatment of choice in the majority of patients with achalasia.     

Histology of esophageal mucosa from patients with achalasia

When achalasia becomes far advanced and leads to esophageal resection, inflammation of the esophageal mucosa is almost universal. The histology of the esophageal mucosa in less advanced cases of achalasia has not been firmly established. We have studied endoscopic biopsies obtained during evaluation of patients with achalasia. Two to four endoscopic biopsies from the lower esophagus of 26 patients with manometrically verified achalasia were mounted on mesh, serially sectioned, stained, coded and interpreted by two independent observers using recognized criteria. The histological findings were correlated with clinical data. Ten of 26 patients had at least one abnormal biopsy. Five of these 10 patients had a previous Heller myotomy; another patient had several pneumatic dilatations, and two other patients had endoscopically proven candida infections. Of the 16 patients with normal histology, four had prolonged stasis, five had heartburn and one patient had both heartburn and stasis. Unless the patient with achalasia has had a Heller myotomy, balloon dilatation, or a candida infection, the esophageal mucosa on biopsy appears to be within normal limits, even in patients with years of esophageal stasis or complaints of heartburn.     

p53 and MIB-1 expression of esophageal carcinoma concominant with achalasia

Achalasia, insufficient relaxation of the lower esophageal sphincter (LES) causes the retention and stasis of food and secretions, chronic hyperplastic esophagitis and eventual malignant transformation. p53 alternation has been suggested to play an important role in the malignant transformation. A 53-year-old man was endoscopically followed up for esophageal achalasia for seven years, and endoscopy revealed an ulcerative region in the upper thoracic esophagus, and histopathologically the biopsy specimens showed moderately differentiated squamous cell carcinoma. In resected specimens, p53 staining was strong and diffuse in the tumor and MIB-1 immunoreactivity was strong and patchy in the tumor and the basal layer of squamous mucosa of the esophagus. No staining for either immunostains was noted in normal esophageal mucosa. It is necessary for patients with esophageal achalasia to be followed-up with endoscopy, and that p53 and MIB-1 immunostaining of biopsy specimens should be performed to detect pre-cancerous lesions.     

Treatment of achalasia

Opinion statement Achalasia is a primary motility disorder of the esophagus that causes dysphagia. Normal esophageal motility and lower esophageal sphincter (LES) function can not be restored; thus treatment is directed at decreasing the pressure or disrupting the muscle fibers of the LES to allow passage of ingested material. Effective therapy for achalasia can be broadly characterized as surgery based or endoscopy based. Medications (calcium channel blockers and nitrate derivatives) do not provide adequate relief of dysphagia and have substantial side effects, and thus are rarely used as long-term therapy. Botulinum toxin injection, a recently introduced endoscopic therapy, enjoyed much enthusiasm initially but was shown to have only transient effect and is now recommended only for poor operative candidates. The mainstay of therapy remains endoscopic dilation or laparoscopic esophagomyotomy (LEM) combined with an antireflux procedure. We have found that patients who can tolerate a laparoscopic abdominal surgery are best served with an LEM and Toupet (270°) posterior fundoplication. This provides good or excellent relief of dysphagia in 90% to 95% of patients with very little morbidity.     

Long-term risk of oesophagitis, Barrett's oesophagus and oesophageal cancer in achalasia patients

Achalasia is a motility disorder of the oesophagus of unknown origin in which loss of relaxation of the lower oesophageal sphincter (LOS) and aperistalsis in the distal oesophagus leads to functional oesophageal obstruction. The treatment is symptomatic, aimed at lowering of the LOS pressure, and may be accompanied by various side effects, including gastro-oesophageal reflux, a risk factor for oesophagitis and its complications. Stasis and fermentation can also lead to inflammation of the oesophageal mucosa, giving rise to hyperplasia of the epithelium, multifocal dysplasia and in some patients eventually squamous cell carcinoma. Unfortunately, the sensitivity and specificity of endoscopical inspection to assess inflammation or dysplasia of the oesophageal lining is low, such that biopsy sampling is necessary for accurate assessment. Although it is generally accepted that achalasia is a pre-malignant disorder, the reported increased risk of patients with achalasia developing a squamous cell carcinoma varies from 0 to 140 times that of the normal population. In addition, achalasia may predispose to Barrett's metaplasia and oesophageal adenocarcinoma, which have been described in case reports after myotomy. Surveillance endoscopy with tissue sampling to detect pre-neoplastic lesions has been recommended, even though this can be very difficult due to mucosal adherence of food as well as hyperplastic changes of the mucosa. In the event of moderate to severe inflammation and/or persisting stasis of food despite adequate LOS pressure-lowering therapy, the surveillance interval should be shortened and performed after a 3-day liquid diet. The exact technique and time intervals still need to be established, however.     

Complications during pneumatic dilation for achalasia or diffuse esophageal spasm

A retrospective cohort study was performed to assess risk factors, early clinical characteristics, and outcome of complications in patients undergoing pneumatic dilation. Of 178 patients with achalasia or diffuse esophageal spasm who underwent 236 dilations with a Browne-McHardy dilator, 16 patients experienced a complication (9.0%). Nine major complications developed: perforations (4), hematemesis (2), fever (2), and angina (1). A prior pneumatic dilation and use of inflation pressure ≥11 PSI were independent risk factors by multivariate analysis for developing a complication. An esophagram immediately following the dilation identified three of the four perforations. Three postdilation findings were identified as indicators of patients with an increased risk of having developed a perforation: blood on the dilator, tachycardia, and prolonged chest pain lasting >4 hr after dilation. In all patients incurring a major complication, one of the three indicators, or the complication itself was recognized within 5 hr of dilation. All patients with complications, including the four with perforation who received prompt surgical repair and esophagomyotomy, recovered uneventfully. The symptomatic relief of dysphagia in patients with perforation undergoing emergent surgical repair and esophagomyotomy was similar to patients undergoing elective esophagomyotomy. Conclusions: (1) Pneumatic dilation is a safe treatment of achalasia, with a 1.7% risk of perforation. (2) The risk of developing a complication is increased by having had a previous pneumatic dilation or by use of inflation pressures ≥11 psi. (3) All patients with a major complication were identified within 5 hr after dilation. (4) Complications following pneumatic dilation, if recognized and treated promptly, were not associated with adverse, long-term sequelae.     

Results of surgical treatment of achalasia of the esophagus.

Surgical treatment of patients with achalasia of the esophagus results in dramatic and permanent relief in almost 90% of the patients. The abdominal approach seems to produce more reflux than the thoracic route. There is evidence that extending myotomy more than 10 mm onto the stomach increases reflux. The length of the hypertensive gastroesophageal sphincter is almost 4 cms and an anterior esophagomyotomy of 5 to 6 cms is long enough in these patients. Extending the section 7 to 10 cms proximally would seem to be unnecessary and may provoke more reflux. The mortality rate of the surgical procedure is very low--less than 0.2%. Postoperative complications can occur in almost 4% of them, esophageal leakage being the most dangerous. The most frequent late complication is gastroesophageal reflux, which can occur symptomatically in 10% of the cases and by objective studies in almost 20% of the patients. The addition of antireflux surgery is controversial. If performed, it must be ensured that no obstruction can occur; esophageal emptying in an aperistalsic esophagus can be seriously delayed. Comparative studies suggest that the addition of antireflux surgery gives better results than myotomy alone. Surgeons performing this operative technique should be specialized digestive tract surgeons and familiar with manometric studies.