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Diagnosis of Primary Versus


Secondary Achalasia: Reassessment
of Clinical and Radiographic Criteria
Courtney A. Woodfield 1 OBJECTIVE. Our purpose was to reassess the usefulness of barium studies and various
Marc S. Levine clinical parameters for differentiating primary from secondary achalasia.
Stephen E. Rubesin MATERIALS AND METHODS. Radiology files from 1989 through 1999 revealed 29
Curtis P. Langlotz 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)
Igor Laufer
who met our study criteria. The radiographs were reviewed to determine the morphologic fea-
tures 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 esoph-
ageal 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 achala-
sia 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 achala-
sia, 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 achala-
sia, even in the absence of other suspicious radiographic findings.

A chalasia is a well-known esoph-


ageal motility disorder character-
ized by absent primary peristalsis
and incomplete relaxation of the lower esoph-
smooth, tapered narrowing of the distal
esophagus caused by incomplete relaxation
of the lower esophageal sphincter [11]. How-
ever, in secondary achalasia, barium studies
ageal sphincter [1]. Most patients have primary may also reveal eccentricity, nodularity, an-
(idiopathic) achalasia caused by loss of the gulation, straightening, or proximal shoul-
ganglion cells in the esophageal myenteric dering of the narrowed segment [4, 7, 8, 12,
plexuses [2, 3]. However, others have second- 13]. In one report, it was suggested that the
ary achalasia (pseudoachalasia) caused by ma- narrowed segment may be longer in second-
lignant tumor at the gastroesophageal junction ary than in primary achalasia [12]. Second-
[4–9] or, less commonly, by benign conditions ary achalasia should also be suspected if
such as Chagas’ disease [10]. Nearly 75% of barium studies reveal tumor at the gastric
patients with secondary achalasia are found to cardia [4, 12, 13].
have underlying carcinoma of the cardia [6], Nevertheless, little data are available
Received January 14, 2000; accepted after revision but secondary achalasia may also be caused by about the usefulness of barium studies in dif-
February 16, 2000. carcinoma of the esophagus or by other malig- ferentiating primary from secondary achala-
1
All authors: Department of Radiology, Hospital of the nant tumors that metastasize to the mediasti- sia. In the two largest series in the literature,
University of Pennsylvania, 3400 Spruce St., Philadelphia, num or gastroesophageal junction, including it was possible to distinguish these condi-
PA 19104. Address correspondence to M. S. Levine.
carcinoma of the lung, breast, pancreas, uterus, tions on barium studies in only six (46%) of
AJR 2000;175:727–731
and prostate gland [4, 7–9]. 13 patients [6, 14]. We therefore performed a
0361–803X/00/1753–727 Primary achalasia is characterized on bar- retrospective investigation of patients with
© American Roentgen Ray Society ium studies by absent primary peristalsis and primary and secondary achalasia to reassess

AJR:175, September 2000 727


Woodfield et al.

the usefulness of barium studies and various whether the patient’s age, the duration of dyspha- (90%). In two patients, the distal esophagus
clinical parameters for differentiating these gia, the length of the narrowed distal esophageal had a tortuous (i.e., sigmoid) configuration.
conditions. segment, or the diameter of the proximal esopha- The gastric cardia and fundus appeared nor-
gus was significantly associated with achalasia eti- mal in 10 patients (34%) but could not be ad-
ology (i.e., primary versus secondary achalasia).
equately evaluated because of delayed
Materials and Methods
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emptying of barium from the esophagus in


Computerized radiology files at our university the remaining 19 patients (66%).
Results
hospital from 1989 through 1999 and radiology
Secondary achalasia.—In six (60%) of 10
logs at our affiliated Veterans Affairs medical cen- Clinical Findings
patients with secondary achalasia, barium stud-
ter from 1995 through 1999 revealed 150 patients Primary achalasia.—Sixteen of the 29 pa-
with a diagnosis of achalasia on barium studies. ies revealed smooth symmetric, tapered nar-
tients with primary achalasia were women rowing of the distal esophagus (Figs. 3 and 4).
Seventy-two of these 150 patients were excluded
from our analysis because of known treatment for
and 13 were men. The mean age was 53 The remaining four patients (40%) had eccen-
achalasia (e.g., pneumatic dilatation, botulinum years (range, 22–87 years); 11 patients tric narrowing of the distal esophagus (Fig.
toxin injection, or surgical myotomy) before un- (38%) were more than 60 years old. All 29 5A), with abrupt proximal borders in one, nod-
dergoing any barium studies at our hospital, and patients presented with dysphagia, which ularity in one, and straightening in one. The
39 were excluded because medical records were had a mean duration of 4.5 years (range, 0.1– narrowed segment had a mean length of 4.4 cm
unavailable (36 patients) or clinical follow-up was 20 years); 28 patients (97%) had dysphagia (range, 2.5–5.0 cm) and was longer than 3.5 cm
inadequate to establish the diagnosis (three pa- for 1 year or longer. Five patients had weight in eight patients (80%) (Figs. 3–5). The esoph-
tients). The remaining 39 patients constituted our loss, with a mean loss of 8.2 kg (range, 3.6– agus above the narrowed segment had a mean
study group. 16 kg) over a mean period of 13 months
On the basis of the endoscopic, manometric, diameter of 4.1 cm (range, 3.5–6 cm) and was
(range, 2–36 months). 4 cm or less in diameter in eight patients
CT, and surgical findings, 29 patients (74%) had a
final diagnosis of primary achalasia, and 10 (26%)
Secondary achalasia.—Nine of the 10 pa- (80%). Patients with secondary achalasia were
had a final diagnosis of secondary achalasia tients with secondary achalasia were men significantly more likely to have a longer seg-
caused by carcinoma of the esophagus in three pa- and one was a woman. The mean age was 69 ment of narrowing ( p < 0.0001) and to have a
tients, carcinoma of the gastric cardia in three, and years (range, 48–87 years); eight patients less dilated proximal esophagus ( p < 0.0001)
metastases to the mediastinum or gastroesoph- (80%) were more than 60 years old. All 10 than patients with primary achalasia (Table 1).
ageal junction from carcinoma of the lung in three patients presented with dysphagia, which One patient also had an annular lesion
and from carcinoma of the uterus in one. had a mean duration of 1.9 months (range, with abrupt shelflike borders in the upper
All 39 patients underwent barium studies, in- 0.5–4 months). Patients with secondary esophagus caused by esophageal carcinoma
cluding double-contrast esophagography in 11, achalasia were significantly more likely to be
single-contrast esophagography in five, double- (Fig. 5B). Secondary achalasia in this patient
older ( p = 0.03) and to have a shorter dura- presumably resulted from the spread of tu-
contrast upper gastrointestinal examinations in 17,
and single-contrast upper gastrointestinal exami-
tion of dysphagia ( p < 0.0001) than patients mor via lymphatics in the esophageal wall to
nations in six. In all 39 patients, the radiographic with primary achalasia (Table 1). Seven pa- the gastroesophageal junction.
reports described absent primary peristalsis in the tients had weight loss, with a mean loss of The gastric cardia and fundus appeared
esophagus on fluoroscopy and a segment of distal 10.5 kg (range, 2.7–30 kg) over a mean pe- abnormal in three patients (30%) with sec-
esophageal narrowing that extended to the gastro- riod of 5 months (range, 0.5–12 months). ondary achalasia. Two had carcinoma of the
esophageal junction. The correct diagnosis was cardia; barium studies revealed lobulated
suggested on the original radiology reports in all Radiographic Findings fundal folds in one and encasement of the
10 patients with secondary achalasia.
Primary achalasia.—In all 29 patients fundus by tumor in the other. In one patient
The radiographs from these 39 studies were re-
viewed in a blinded fashion to determine the de- with primary achalasia, barium studies re- with esophageal carcinoma, a barium study
gree of esophageal dilatation at its widest point vealed smooth symmetric, tapered narrowing revealed nodularity of the gastric fundus. In
and to evaluate the morphologic features of the of the distal esophagus that extended to the two other patients, barium studies revealed a
narrowed distal esophageal segment, including gastroesophageal junction (Figs. 1 and 2). normal-appearing cardia and fundus. In the
symmetry (symmetric versus eccentric), contour The narrowed segment had a mean length of remaining five patients (including one with
(smooth versus nodular or ulcerated), proximal 1.9 cm (range, 0.7–3.5 cm). The esophagus carcinoma of the cardia), the cardia and fun-
borders (tapered versus abrupt or shouldered), and above the narrowed segment had a mean di- dus could not be adequately evaluated be-
length (measured from the proximal border of the ameter of 6.2 cm (range, 4–10 cm) and was cause of delayed emptying of barium from
narrowed segment to the gastroesophageal junc-
greater than 4 cm in diameter in 26 patients the esophagus.
tion, not accounting for radiographic magnifica-
tion). When sufficient barium entered the stomach,
the gastric cardia and fundus were also evaluated
for evidence of tumor in this region. TABLE 1 Major Variables of Primary and Secondary Achalasia in 30 Patients
Medical, radiologic, and endoscopic records
Primary Achalasia Secondary Achalasia
were also reviewed to determine the clinical pre- Variable p
(20 Patients) (10 Patients)
sentation as well as the endoscopic, manometric,
CT, and surgical findings. Age (years) 53 ± 19 69 ± 12 0.03
Univariate statistical analysis was performed on Duration of dysphagia (months) 54 ± 52 1.9 ± 1.2 < 0.0001
all major study variables. Wilcoxon’s rank sum Length of narrowing (cm) 1.9 ± 0.78 4.4 ± 0.88 < 0.0001
test was performed using JMP statistical analysis
Diameter of proximal esophagus (cm) 6.2 ± 1.5 4.1 ± 0.76 < 0.0001
software (SAS Institute, Cary, NC) to determine

728 AJR:175, September 2000


Radiography of Primary Versus Secondary Achalasia
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Fig. 1.—50-year-old man with primary Fig. 2.—23-year-old woman with primary achalasia. Spot ra- Fig. 3.—60-year-old man with secondary
achalasia. Spot radiograph from double- diograph from double-contrast barium study shows 3.5-cm- achalasia caused by lung carcinoma. Spot ra-
contrast barium study shows 1-cm-long long, gradually tapered segment of narrowing (straight diograph from single-contrast barium study
smooth, tapered narrowing (straight arrows ) in distal esophagus with esophageal diameter proxi- shows 5-cm-long symmetric, tapered narrow-
arrow ) of distal esophagus with esoph- mally of 7 cm and standing column of barium (curved arrow ). ing (arrows) of distal esophagus with esoph-
ageal diameter proximally of 6 cm. Note This was longest segment of narrowing shown on radiogra- ageal diameter proximally of 6 cm. Note fine
standing column of barium (curved arrow ) phy in a patient with primary achalasia. irregularity of contour of distal esophagus
on this upright view. Short length of nar- above narrowed segment caused by super-
rowed segment is characteristic of primary imposed infection with Candida esophagitis
achalasia. organisms proven on endoscopy.

Endoscopic, Manometric, CT, and Surgical Findings fundus; endoscopic biopsy specimens revealed patients with this condition. In the remaining
Primary achalasia.—Twenty-five of the 29 carcinoma of the cardia in two of these patients. 60%, the narrowed segment was smooth and
patients with primary achalasia had typical The third had carcinoma of the cardia at sur- symmetric with tapered proximal borders
findings of achalasia on manometry [1, 15]. In gery. In three patients with lung carcinoma, (Figs. 3 and 4). Therefore, secondary achalasia
all 29 patients, endoscopy revealed a closed chest CT scans revealed mediastinal adenopa- would not be suspected in most cases solely on
lower esophageal sphincter that opened in re- thy and mediastinal invasion by tumor. In the the basis of classic radiologic criteria. However,
sponse to the advancing endoscope, allowing it remaining patient with endometrial carcinoma, the narrowed distal esophageal segment was
to pass into the gastric fundus [15]. an abdominal CT scan revealed widespread in- longer than 3.5 cm in 80% of patients with sec-
Secondary achalasia.—Eight of the 10 pa- traperitoneal metastases, and a bone scan re- ondary achalasia, and an unusually long seg-
tients with secondary achalasia underwent en- vealed diffuse osseous metastases. Although ment of narrowing was the only suspicious
doscopy, which revealed a closed lower this patient did not have a chest CT scan, she finding in 40% (Figs. 3 and 4). In contrast, the
esophageal sphincter in all cases; the endoscope was presumed to have secondary achalasia be- narrowed segment was 3.5 cm or shorter in all
could not be advanced into the stomach in four cause of her widely disseminated endometrial patients with primary achalasia (Figs. 1 and 2).
of these patients, a finding that has been associ- carcinoma, advanced age (87 years), and short Therefore, the length of the narrowed distal
ated with secondary achalasia [6, 15–17]. Three duration of dysphagia (3 months). esophageal segment was a useful and statisti-
patients had esophageal carcinoma at endos- cally significant criterion for differentiating
copy, with infiltrative lesions in the distal esoph- secondary achalasia from primary achalasia on
Discussion
agus in two and in the upper esophagus in one; barium studies ( p < 0.0001).
endoscopic biopsy specimens revealed squa- In our study, barium studies revealed classic In our series, the degree of esophageal dila-
mous cell carcinoma in all three patients. Three findings of secondary achalasia with an eccen- tation above the narrowed segment was also a
other patients had carcinoma of the cardia on tric, nodular, or shouldered segment of distal statistically significant criterion for differentiat-
endoscopy, with polypoid masses in the gastric esophageal narrowing (Fig. 5A) in only 40% of ing secondary achalasia from primary achalasia

AJR:175, September 2000 729


Woodfield et al.

Fig. 4.—87-year-old woman with ( p < 0.0001). The diameter of the esophagus at
secondary achalasia caused by car- its widest point was 4 cm or less in 80% of pa-
cinoma of uterus. Spot radiograph
from double-contrast barium study tients with secondary achalasia, whereas the di-
shows 4-cm-long smooth, tapered ameter of the esophagus was greater than 4 cm
narrowing (arrows ) of distal esoph- in 90% of patients with primary achalasia. The
agus with esophageal diameter
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proximally of 3.5 cm. As in Figure 3, a greater degree of esophageal dilatation in pa-


narrowed segment longer than 3.5 tients with primary achalasia was presumably
cm should be considered highly sug- related to the more gradual course of the dis-
gestive of secondary achalasia,
even lacking other suspicious radio-
ease that allowed the esophagus to progres-
graphic findings. sively dilate over a period of years. In fact, both
patients who had a tortuous (i.e., sigmoid) dis-
tal esophagus were found to have primary
achalasia with relatively long-standing disease.
A limitation of our study is the variable ef-
fect of magnification on our radiographic
measurements of the narrowed distal esoph-
ageal segment or dilated proximal esophagus
in patients with primary or secondary achala-
sia, depending on the height of the fluoro-
scopic 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 pa-
tient 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 under-
lying malignant tumor at the gastroesophageal
junction as the cause of these findings [4, 6, 13,

Fig. 5.—63-year-old man with sec-


ondary achalasia caused by carci-
noma of esophagus.
A, Spot radiograph from double-con-
trast barium study shows 4-cm-long
eccentric, tapered narrowing (arrows)
of distal esophagus with esophageal
diameter proximally of 4 cm.
B, Additional spot radiograph shows
annular carcinoma with relatively
abrupt, shelflike margins (arrows ) in
upper thoracic esophagus.
A B

730 AJR:175, September 2000


Radiography of Primary Versus Secondary Achalasia

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AJR:175, September 2000 731

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