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1 All authors: Department of Radiology, Hospital of the University of Pennsylvania, 3400 Spruce St., Philadelphia, PA 19104.
Received January 14, 2000;
accepted after revision February 16, 2000.
Address correspondence to M. S. Levine.
Abstract
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MATERIALS AND METHODS. Radiology files from 1989 through 1999 revealed 29 patients with primary achalasia and 10 with secondary achalasia (caused by carcinoma of the esophagus in three, of the gastric cardia in three, of the lung in three, and of the uterus in one) who met our study criteria. The radiographs were reviewed to determine the morphologic features of the narrowed distal esophageal segment and gastric cardia and fundus. Medical records were also reviewed to determine the clinical presentation; endoscopic, manometric, and surgical findings; and treatment.
RESULTS. The mean patient age was 53 years in primary achalasia versus 69 years in secondary achalasia (p = 0.03). The mean duration of dysphagia was 4.5 years in primary achalasia versus 1.9 months in secondary achalasia (p <0.0001). The narrowed distal esophageal segment had a mean length of 1.9 cm in primary achalasia versus 4.4 cm in secondary achalasia (p < 0.0001), and the esophagus had a mean diameter of 6.2 cm in primary achalasia versus 4.1 cm in secondary achalasia (p <0.0001). The narrowed segment was eccentric or nodular or had abrupt proximal borders in only four of 10 patients with secondary achalasia, and evidence of tumor was present in the gastric fundus in only three.
CONCLUSION. When findings of achalasia are present on barium studies, a narrowed distal esophageal segment longer than 3.5 cm with little or no proximal dilatation in a patient with recent onset of dysphagia should be considered highly suggestive of secondary achalasia, even in the absence of other suspicious radiographic findings.
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Primary achalasia is characterized on barium studies by absent primary peristalsis and smooth, tapered narrowing of the distal esophagus caused by incomplete relaxation of the lower esophageal sphincter [11]. However, in secondary achalasia, barium studies may also reveal eccentricity, nodularity, angulation, straightening, or proximal shouldering of the narrowed segment [4, 7, 8, 12, 13]. In one report, it was suggested that the narrowed segment may be longer in secondary than in primary achalasia [12]. Secondary achalasia should also be suspected if barium studies reveal tumor at the gastric cardia [4, 12, 13].
Nevertheless, little data are available about the usefulness of barium studies in differentiating primary from secondary achalasia. In the two largest series in the literature, it was possible to distinguish these conditions on barium studies in only six (46%) of 13 patients [6, 14]. We therefore performed a retrospective investigation of patients with primary and secondary achalasia to reassess the usefulness of barium studies and various clinical parameters for differentiating these conditions.
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On the basis of the endoscopic, manometric, CT, and surgical findings, 29 patients (74%) had a final diagnosis of primary achalasia, and 10 (26%) had a final diagnosis of secondary achalasia caused by carcinoma of the esophagus in three patients, carcinoma of the gastric cardia in three, and metastases to the mediastinum or gastroesophageal junction from carcinoma of the lung in three and from carcinoma of the uterus in one.
All 39 patients underwent barium studies, including double-contrast esophagography in 11, single-contrast esophagography in five, double-contrast upper gastrointestinal examinations in 17, and single-contrast upper gastrointestinal examinations in six. In all 39 patients, the radiographic reports described absent primary peristalsis in the esophagus on fluoroscopy and a segment of distal esophageal narrowing that extended to the gastroesophageal junction. The correct diagnosis was suggested on the original radiology reports in all 10 patients with secondary achalasia.
The radiographs from these 39 studies were reviewed in a blinded fashion to determine the degree of esophageal dilatation at its widest point and to evaluate the morphologic features of the narrowed distal esophageal segment, including symmetry (symmetric versus eccentric), contour (smooth versus nodular or ulcerated), proximal borders (tapered versus abrupt or shouldered), and length (measured from the proximal border of the narrowed segment to the gastroesophageal junction, not accounting for radiographic magnification). When sufficient barium entered the stomach, the gastric cardia and fundus were also evaluated for evidence of tumor in this region.
Medical, radiologic, and endoscopic records were also reviewed to determine the clinical presentation as well as the endoscopic, manometric, CT, and surgical findings.
Univariate statistical analysis was performed on all major study variables. Wilcoxon's rank sum test was performed using JMP statistical analysis software (SAS Institute, Cary, NC) to determine whether the patient's age, the duration of dysphagia, the length of the narrowed distal esophageal segment, or the diameter of the proximal esophagus was significantly associated with achalasia etiology (i.e., primary versus secondary achalasia).
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Secondary achalasia.—Nine of the 10 patients with secondary achalasia were men and one was a woman. The mean age was 69 years (range, 48-87 years); eight patients (80%) were more than 60 years old. All 10 patients presented with dysphagia, which had a mean duration of 1.9 months (range, 0.5-4 months). Patients with secondary achalasia were significantly more likely to be older (p = 0.03) and to have a shorter duration of dysphagia (p <0.0001) than patients with primary achalasia (Table 1). Seven patients had weight loss, with a mean loss of 10.5 kg (range, 2.7-30 kg) over a mean period of 5 months (range, 0.5-12 months).
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Radiographic Findings
Primary achalasia.—In all 29 patients with primary
achalasia, barium studies revealed smooth symmetric, tapered narrowing of the
distal esophagus that extended to the gastroesophageal junction (Figs.
1 and
2). The narrowed segment had a
mean length of 1.9 cm (range, 0.7-3.5 cm). The esophagus above the narrowed
segment had a mean diameter of 6.2 cm (range, 4-10 cm) and was greater than 4
cm in diameter in 26 patients (90%). In two patients, the distal esophagus had
a tortuous (i.e., sigmoid) configuration. The gastric cardia and fundus
appeared normal in 10 patients (34%) but could not be adequately evaluated
because of delayed emptying of barium from the esophagus in the remaining 19
patients (66%).
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Secondary achalasia.—In six (60%) of 10 patients with secondary achalasia, barium studies revealed smooth symmetric, tapered narrowing of the distal esophagus (Figs. 3 and 4). The remaining four patients (40%) had eccentric narrowing of the distal esophagus (Fig. 5A), with abrupt proximal borders in one, nodularity in one, and straightening in one. The narrowed segment had a mean length of 4.4 cm (range, 2.5-5.0 cm) and was longer than 3.5 cm in eight patients (80%) (Figs. 3,4,5A,5B). The esophagus above the narrowed segment had a mean diameter of 4.1 cm (range, 3.5-6 cm) and was 4 cm or less in diameter in eight patients (80%). Patients with secondary achalasia were significantly more likely to have a longer segment of narrowing (p < 0.0001) and to have a less dilated proximal esophagus (p < 0.0001) than patients with primary achalasia (Table 1).
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One patient also had an annular lesion with abrupt shelflike borders in the upper esophagus caused by esophageal carcinoma (Fig. 5B). Secondary achalasia in this patient presumably resulted from the spread of tumor via lymphatics in the esophageal wall to the gastroesophageal junction.
The gastric cardia and fundus appeared abnormal in three patients (30%) with secondary achalasia. Two had carcinoma of the cardia; barium studies revealed lobulated fundal folds in one and encasement of the fundus by tumor in the other. In one patient with esophageal carcinoma, a barium study revealed nodularity of the gastric fundus. In two other patients, barium studies revealed a normal-appearing cardia and fundus. In the remaining five patients (including one with carcinoma of the cardia), the cardia and fundus could not be adequately evaluated because of delayed emptying of barium from the esophagus.
Endoscopic, Manometric, CT, and Surgical Findings
Primary achalasia.—Twenty-five of the 29 patients with
primary achalasia had typical findings of achalasia on manometry
[1,
15]. In all 29 patients,
endoscopy revealed a closed lower esophageal sphincter that opened in response
to the advancing endoscope, allowing it to pass into the gastric fundus
[15].
Secondary achalasia.—Eight of the 10 patients with secondary achalasia underwent endoscopy, which revealed a closed lower esophageal sphincter in all cases; the endoscope could not be advanced into the stomach in four of these patients, a finding that has been associated with secondary achalasia [6, 15,16,17]. Three patients had esophageal carcinoma at endoscopy, with infiltrative lesions in the distal esophagus in two and in the upper esophagus in one; endoscopic biopsy specimens revealed squamous cell carcinoma in all three patients. Three other patients had carcinoma of the cardia on endoscopy, with polypoid masses in the gastric fundus; endoscopic biopsy specimens revealed carcinoma of the cardia in two of these patients. The third had carcinoma of the cardia at surgery. In three patients with lung carcinoma, chest CT scans revealed mediastinal adenopathy and mediastinal invasion by tumor. In the remaining patient with endometrial carcinoma, an abdominal CT scan revealed widespread intraperitoneal metastases, and a bone scan revealed diffuse osseous metastases. Although this patient did not have a chest CT scan, she was presumed to have secondary achalasia because of her widely disseminated endometrial carcinoma, advanced age (87 years), and short duration of dysphagia (3 months).
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In our series, the degree of esophageal dilatation above the narrowed segment was also a statistically significant criterion for differentiating secondary achalasia from primary achalasia (p <0.0001). The diameter of the esophagus at its widest point was 4 cm or less in 80% of patients with secondary achalasia, whereas the diameter of the esophagus was greater than 4 cm in 90% of patients with primary achalasia. The greater degree of esophageal dilatation in patients with primary achalasia was presumably related to the more gradual course of the disease that allowed the esophagus to progressively dilate over a period of years. In fact, both patients who had a tortuous (i.e., sigmoid) distal esophagus were found to have primary achalasia with relatively long-standing disease.
A limitation of our study is the variable effect of magnification on our radiographic measurements of the narrowed distal esophageal segment or dilated proximal esophagus in patients with primary or secondary achalasia, depending on the height of the fluoroscopic tower above the examining table. This variable could create a potential bias if greater magnification occurred primarily in one group or the other. However, the degree of magnification was in no way related to patient selection, so this variable should not have had a significant effect on our findings.
When findings of achalasia are present on barium studies, it is important to evaluate the gastric cardia and fundus to rule out an underlying malignant tumor at the gastroesophageal junction as the cause of these findings [4, 6, 13, 18]. In our series, however, the cardia and fundus could not be adequately evaluated radiographically in 66% of patients with primary achalasia and in 50% with secondary achalasia because of delayed emptying of barium from the esophagus. Therefore, it is important to be aware of the limitations of barium studies in evaluating the cardia and fundus in patients with suspected achalasia.
In the past, some investigators have advocated amyl nitrite inhalation as a simple test for differentiating primary and secondary achalasia on barium studies. It has been shown that inhalation of amyl nitrite, a smooth-muscle relaxant, has no effect on the narrowed distal esophageal segment in secondary achalasia but causes a measurable increase of 2 mm or more in the caliber of this segment in primary achalasia [19]. Nevertheless, this technique has not gained widespread acceptance.
Although our investigation focused on the usefulness of barium studies for differentiating the two forms of achalasia, CT may also be useful in these patients. CT typically reveals little or no esophageal wall thickening and no evidence of a mass at the cardia in patients with primary achalasia [20,21,22]. In some cases, however, CT may reveal a pseudomass at the cardia in patients without tumor because of inadequate distention of this region [23]. In contrast, CT may show asymmetric thickening of the distal esophageal wall, a soft-tissue mass at the cardia, or mediastinal adenopathy in patients with secondary achalasia [21]. CT may also be helpful for identifying the site of the primary tumor in patients with secondary achalasia caused by remote tumors.
Various clinical parameters are also purported to be useful for differentiating primary achalasia from secondary achalasia, including the age of the patient, the duration of dysphagia, and substantial weight loss. Primary achalasia is more likely to occur in younger patients (<50 years old) with longstanding dysphagia (>1 year) and little or no weight loss (<7 kg) [15, 18], whereas secondary achalasia is more likely to occur in older patients (>60 years old) with recent onset of dysphagia (<6 months) and substantial weight loss (>7 kg) [14]. Nevertheless, overlap in the clinical presentation has been reported for all these parameters [17, 24]. In our series, the duration of dysphagia was a statistically significant clinical criterion for differentiating secondary achalasia from primary achalasia (p <0.0001); all patients with secondary achalasia had dysphagia for 4 months or less, whereas 97% of patients with primary achalasia had dysphagia for 1 year or more. The age of the patient was also a statistically significant but somewhat less useful criterion for differentiating these conditions (p = 0.03); 80% of patients with secondary achalasia and 38% with primary achalasia were more than 60 years old. In two previously published series, 28-30% of patients with primary achalasia were also found to be more than 60 years old [17, 24], limiting the usefulness of this criterion.
In conclusion, only 40% of patients in our series had classic radiographic features of secondary achalasia such as eccentricity, nodularity, or shouldering of the narrowed distal esophageal segment, or suspicious findings in the region of the gastric cardia or fundus. Instead, the most useful criteria for differentiating secondary from primary achalasia were the length of the narrowed segment and the degree of proximal dilatation, and the most useful clinical criterion was the duration of dysphagia. When findings of achalasia are present on barium studies, a narrowed distal esophageal segment longer than 3.5 cm with little or no proximal dilatation in a patient with recent onset of dysphagia should be considered highly suggestive of secondary achalasia, even in the absence of other suspicious radiographic findings.
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