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Volume: Barrett's Esophagus
Chapter: Pathophysiology

Is preexisting gastroesophageal reflux necessary for the development of Barrett's esophagus in patients with achalasia?

F.H. Ellis (Boston)

Supported in part by the Thelma and Jerry Stergios Thoracic Surgery Education and Research Fund.

Esophageal achalasia is a precancerous lesion leading to squamous cell carcinoma in a small percentage of cases. In the experience of my colleagues and myself [1, 2], the rate of such a malignant transformation is 88 per 100,000 population with achalasia, a risk 14.5 times that of an age and sex-adjusted general population. Adenocarcinoma of the esophagus in a patient with achalasia, however, is extremely rare [3] (Table I) and is the result of malignant transformation of metaplastic epithelial cells lining the distal esophagus, a condition known as Barrett's esophagus. Since Barrett's esophagus is the result of gastroesophageal reflux (GER), the reflux pattern of patients with achalasia must be examined as a way of answering the question of whether preexisting GER is necessary for the development of Barrett's esophagus in patients with achalasia.

A partial answer to the question is provided by the fact that an improperly performed esophagomyotomy or forceful dilation results in GER in a small number of patients who are then at risk for the subsequent transformation of the mucosal lining of the esophagus into metaplastic intestinal-type epithelium (Barrett's esophagus). Evidence for this, however, needs documentation.

Does any evidence suggest that GER can occur in the untreated patient with achalasia? It would seem at first glance to be unlikely in view of the fact that one of the hallmarks of achalasia is a nonrelaxing or poorly relaxing lower esophageal sphincter (LES). A number of reports have been made of pathologic GER in untreated patients with achalasia [4-12], documented in some patients by 24-hour pH monitoring [6-9], the incidence ranging from 17% to 50%. However, it is difficult to determine from most of these reports whether or not the GER preceded the onset of achalasia. In some cases, the symptoms of GER and dysphagia developed simultaneously, suggesting that reflux was not a preexisting condition. However, Smart et al. [11] reported on five patients with a history of GER who developed achalasia two to 10 years later. More recently, Spechler et al. [10] reported that nine of 32 patients with achalasia plus heartburn had a long history of heartburn before the development of achalasia. These two reports clearly suggest the preexistence of GER in some patients with achalasia.

This same sequence of events was seen in one of our patients (Table I) with GER and Barrett's esophagus who 14 years later developed symptoms of achalasia [12], and 12 years after that, while under intermittent surveillance, experienced adenocarcinoma of the esophagus (see case report).

An answer to this question may be provided in an article published several years ago by my colleagues and myself [3]. We reviewed the literature relative to the development of Barrett's esophagus with or without adenocarcinoma in patients with achalasia, including that of two of our own cases. At present, 24 cases of esophageal achalasia associated with

Table I. Reported cases of achalasia and Barrett's esophagus (updated from [3], with permission from Blackwell Science).

Barrett's esophagus have been reported, with the development of adenocarcinoma in six cases. Two of these patients, cases 15 and 23, had a history of GER and clearly developed Barrett's esophagus before the onset of achalasia. GER developed postoperatively at intervals from 8 months to 30 years after operation in most cases (19 cases), and in six of those 19 cases, Barrett's esophagus was associated with adenocarcinoma. An example of a patient who experienced GER and Barrett's esophagus prior to the onset of achalasia with subsequent malignant transformation (case 23) is described below.

A 40-year-old man had progressive dysphagia for one month after a 14-year history of heartburn and other symptoms of GER. Esophagography revealed a dilated esophagus with a short, smoothly tapered distal segment at the esophagogastric junction (Figure 1A), and esophageal manometry demonstrated LES pressures between 25 and 30 mmHg without evidence of sphincteric relaxation. Deglutitory contractions in the body of the esophagus were simultaneous and of low amplitude, consistent with the diagnosis of esophageal achalasia. Endoscopic biopsies revealed evidence of columnar epithelium with focal areas of mild dysplasia consistent with the diagnosis of Barrett's esophagus. This case was previously reported as an example of a patient with Barrett's esophagus resulting from GER in whom esophageal achalasia developed later [12].

Figure 1. A. Initial esophagogram demonstrates classic picture of achalasia. B. Ten years later, a large irregular mass is seen in the distal esophagus. (Reproduced from [3] with permission of Blackwell Science.)

Forceful dilation with a pneumatic bag was performed, resulting in partial relief of the symptoms of dysphagia. However, recurrence of symptoms three years later required another dilation. Yearly surveillance biopsies of the abnormal mucosa were recommended, but the patient did not return for another two years, at which time the endoscopic and histologic appearance of the esophageal mucosa showed no change. Unfortunately, the patient was not seen again for seven years, this time with recurrence of his symptoms of dysphagia.

Esophagography revealed a large irregular mass in the distal esophagus (Figure 1B), biopsies of which showed adenocarcinoma. In the absence of clinical and laboratory evidence of metastatic disease, he underwent a transhiatal esophagogastrectomy and extramucosal pyloromyotomy. The pathologic diagnosis was moderately differentiated adenocarcinoma arising in Barrett's mucosa with extension through the wall of the esophagus and involvement of three periesophageal lymph nodes (Figure 2). The lesion was staged as T3 N1 Mo, Stage III.

Twelve days after operation, he became depressed and unresponsive and exhibited left hemiparesis. Cranial magnetic resonance imaging study showed evidence suggestive of three brain metastases and a parietal infarct. Administration of decadron and cranial radiation led to subsequent improvement in his symptoms, permitting hospital discharge on the 19th postoperative day. Unfortunately, the initial improvement after cranial radiation was not maintained, and he ultimately died of metastatic disease one and a half months after operation. Autopsy permission was not granted.

Figure 2. A. Macroscopic specimen of excised esophagus demonstrates large intraluminal mass (T) in association with Barrett's mucosa (BM) (E = esophagus; S = stomach). Photomicrographs of the tumor show (B) high-grade dysplasia (right lower) and invasive adenocarcinoma (right upper). C. High-grade dysplasia in Barrett's esophagus. (Reproduced from [3] with permission of Blackwell Science.)


The commonest cause of GER in a patient with achalasia is a postoperative or postdilation complication.

GER can occur in the untreated patient with achalasia either as a preexisting disorder or concomitantly with the development of achalasia. Barrett's esophagus as a result of GER is extremely rare in achalasic patients; only 24 cases having been reported, and only six of those patients experienced adenocarcinoma. Barrett's esophagus was a postoperative complication in all but five patients and was a preexisting disorder in two of those patients. Clearly, preexisting GER is not necessary for the development of Barrett's esophagus in the patient with achalasia.


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Publication date: August 2003 OESO©2015