AJR:175, September 2000

727

Diagnosis of Primary Versus
Secondary Achalasia:

Reassessment
of Clinical and Radiographic Criteria

OBJECTIVE.



Our purpose was to reassess the usefulness of barium studies and various
clinical parameters for differentiating primary from secondary achalasia.

MATERIALS AND METHODS.

Radiology les 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 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 ndings; 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 ndings 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 ndings.
chalasia is a well-known esoph-
ageal motility disorder character-
ized by absent primary peristalsis
and incomplete relaxation of the lower esoph-
ageal sphincter [1]. Most patients have primary
(idiopathic) achalasia caused by loss of the
ganglion cells in the esophageal myenteric
plexuses [2, 3]. However, others have second-
ary achalasia (pseudoachalasia) caused by ma-
lignant tumor at the gastroesophageal junction
[49] or, less commonly, by benign conditions
such as Chagas disease [10]. Nearly 75% of
patients with secondary achalasia are found to
have underlying carcinoma of the cardia [6],
but secondary achalasia may also be caused by
carcinoma of the esophagus or by other malig-
nant tumors that metastasize to the mediasti-
num or gastroesophageal junction, including
carcinoma of the lung, breast, pancreas, uterus,
and prostate gland [4, 79].
Primary achalasia is characterized on bar-
ium studies by absent primary peristalsis and
smooth, tapered narrowing of the distal
esophagus caused by incomplete relaxation
of the lower esophageal sphincter [11]. How-
ever, in secondary achalasia, barium studies
may also reveal eccentricity, nodularity, an-
gulation, straightening, or proximal shoul-
dering of the narrowed segment [4, 7, 8, 12,
13]. In one report, it was suggested that the
narrowed segment may be longer in second-
ary than in primary achalasia [12]. Second-
ary 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 dif-
ferentiating primary from secondary achala-
sia. In the two largest series in the literature,
it was possible to distinguish these condi-
tions 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

Courtney A. Woodeld

1

Marc S. Levine
Stephen E. Rubesin
Curtis P. Langlotz
Igor Laufer

Received January 14, 2000; accepted after revision
February 16, 2000.

1

All authors: Department of Radiology, Hospital of the
University of Pennsylvania, 3400 Spruce St., Philadelphia,
PA 19104. Address correspondence to M. S. Levine.

AJR

2000;175:727731
0361803X/00/1753727
American Roentgen Ray Society

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AJR:175, September 2000

Woodeld et al.

the usefulness of barium studies and various
clinical parameters for differentiating these
conditions.

Materials and Methods

Computerized radiology les at our university
hospital from 1989 through 1999 and radiology
logs at our afliated Veterans Affairs medical cen-
ter from 1995 through 1999 revealed 150 patients
with a diagnosis of achalasia on barium studies.
Seventy-two of these 150 patients were excluded
from our analysis because of known treatment for
achalasia (e.g., pneumatic dilatation, botulinum
toxin injection, or surgical myotomy) before un-
dergoing any barium studies at our hospital, and
39 were excluded because medical records were
unavailable (36 patients) or clinical follow-up was
inadequate to establish the diagnosis (three pa-
tients). The remaining 39 patients constituted our
study group.
On the basis of the endoscopic, manometric,
CT, and surgical ndings, 29 patients (74%) had a
nal diagnosis of primary achalasia, and 10 (26%)
had a nal diagnosis of secondary achalasia
caused by carcinoma of the esophagus in three pa-
tients, carcinoma of the gastric cardia in three, and
metastases to the mediastinum or gastroesoph-
ageal junction from carcinoma of the lung in three
and from carcinoma of the uterus in one.
All 39 patients underwent barium studies, in-
cluding double-contrast esophagography in 11,
single-contrast esophagography in ve, double-
contrast upper gastrointestinal examinations in 17,
and single-contrast upper gastrointestinal exami-
nations in six. In all 39 patients, the radiographic
reports described absent primary peristalsis in the
esophagus on uoroscopy and a segment of distal
esophageal narrowing that extended to the gastro-
esophageal 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 re-
viewed in a blinded fashion to determine the de-
gree 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 junc-
tion, not accounting for radiographic magnica-
tion). When sufcient 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 pre-
sentation as well as the endoscopic, manometric,
CT, and surgical ndings.
Univariate statistical analysis was performed on
all major study variables. Wilcoxons rank sum
test was performed using JMP statistical analysis
software (SAS Institute, Cary, NC) to determine
whether the patients age, the duration of dyspha-
gia, the length of the narrowed distal esophageal
segment, or the diameter of the proximal esopha-
gus was signicantly associated with achalasia eti-
ology (i.e., primary versus secondary achalasia).

Results

Clinical Findings

Primary achalasia.

Sixteen of the 29 pa-
tients with primary achalasia were women
and 13 were men. The mean age was 53
years (range, 2287 years); 11 patients
(38%) were more than 60 years old. All 29
patients presented with dysphagia, which
had a mean duration of 4.5 years (range, 0.1
20 years); 28 patients (97%) had dysphagia
for 1 year or longer. Five patients had weight
loss, with a mean loss of 8.2 kg (range, 3.6
16 kg) over a mean period of 13 months
(range, 236 months).

Secondary achalasi

a.Nine of the 10 pa-
tients with secondary achalasia were men
and one was a woman. The mean age was 69
years (range, 4887 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,
0.54 months). Patients with secondary
achalasia were signicantly more likely to be
older (

p

= 0.03) and to have a shorter dura-
tion of dysphagia (

p

< 0.0001) than patients
with primary achalasia (Table 1). Seven pa-
tients had weight loss, with a mean loss of
10.5 kg (range, 2.730 kg) over a mean pe-
riod of 5 months (range, 0.512 months).

Radiographic Findings

Primary achalasia

.In all 29 patients
with primary achalasia, barium studies re-
vealed 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, 0.73.5 cm). The esophagus
above the narrowed segment had a mean di-
ameter of 6.2 cm (range, 410 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) conguration.
The gastric cardia and fundus appeared nor-
mal in 10 patients (34%) but could not be ad-
equately evaluated because of delayed
emptying of barium from the esophagus in
the remaining 19 patients (66%).

Secondary achalasia

.In six (60%) of 10
patients with secondary achalasia, barium stud-
ies revealed smooth symmetric, tapered nar-
rowing of the distal esophagus (Figs. 3 and 4).
The remaining four patients (40%) had eccen-
tric narrowing of the distal esophagus (Fig.
5A), with abrupt proximal borders in one, nod-
ularity in one, and straightening in one. The
narrowed segment had a mean length of 4.4 cm
(range, 2.55.0 cm) and was longer than 3.5 cm
in eight patients (80%) (Figs. 35). The esoph-
agus above the narrowed segment had a mean
diameter of 4.1 cm (range, 3.56 cm) and was
4 cm or less in diameter in eight patients
(80%). Patients with secondary achalasia were
signicantly more likely to have a longer seg-
ment of narrowing (

p

< 0.0001) and to have a
less dilated proximal esophagus (

p

< 0.0001)
than patients with primary achalasia (Table 1).
One patient also had an annular lesion
with abrupt shelike borders in the upper
esophagus caused by esophageal carcinoma
(Fig. 5B). Secondary achalasia in this patient
presumably resulted from the spread of tu-
mor via lymphatics in the esophageal wall to
the gastroesophageal junction.
The gastric cardia and fundus appeared
abnormal in three patients (30%) with sec-
ondary 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 ve patients (including one with
carcinoma of the cardia), the cardia and fun-
dus could not be adequately evaluated be-
cause of delayed emptying of barium from
the esophagus.
TABLE 1 Major Variables of Primary and Secondary Achalasia in 30 Patients
Variable
Primary Achalasia
(20 Patients)
Secondary Achalasia
(10 Patients)
p
Age (years) 53 19 69 12 0.03
Duration of dysphagia (months) 54 52 1.9 1.2 < 0.0001
Length of narrowing (cm) 1.9 0.78 4.4 0.88 < 0.0001
Diameter of proximal esophagus (cm) 6.2 1.5 4.1 0.76 < 0.0001
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Radiography of Primary Versus Secondary Achalasia

AJR:175, September 2000

729

Endoscopic, Manometric, CT, and Surgical Findings

Primary achalasia

.Twenty-ve of the 29
patients with primary achalasia had typical
ndings of achalasia on manometry [1, 15]. In
all 29 patients, endoscopy revealed a closed
lower esophageal sphincter that opened in re-
sponse to the advancing endoscope, allowing it
to pass into the gastric fundus [15].

Secondary achalasia

.Eight of the 10 pa-
tients with secondary achalasia underwent en-
doscopy, 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 nding that has been associ-
ated with secondary achalasia [6, 1517]. Three
patients had esophageal carcinoma at endos-
copy, with inltrative lesions in the distal esoph-
agus in two and in the upper esophagus in one;
endoscopic biopsy specimens revealed squa-
mous 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 sur-
gery. In three patients with lung carcinoma,
chest CT scans revealed mediastinal adenopa-
thy and mediastinal invasion by tumor. In the
remaining patient with endometrial carcinoma,
an abdominal CT scan revealed widespread in-
traperitoneal metastases, and a bone scan re-
vealed diffuse osseous metastases. Although
this patient did not have a chest CT scan, she
was presumed to have secondary achalasia be-
cause of her widely disseminated endometrial
carcinoma, advanced age (87 years), and short
duration of dysphagia (3 months).

Discussion

In our study, barium studies revealed classic
ndings of secondary achalasia with an eccen-
tric, nodular, or shouldered segment of distal
esophageal narrowing (Fig. 5A) in only 40% of
patients with this condition. In the remaining
60%, the narrowed segment was smooth and
symmetric with tapered proximal borders
(Figs. 3 and 4). Therefore, secondary achalasia
would not be suspected in most cases solely on
the basis of classic radiologic criteria. However,
the narrowed distal esophageal segment was
longer than 3.5 cm in 80% of patients with sec-
ondary achalasia, and an unusually long seg-
ment of narrowing was the only suspicious
nding in 40% (Figs. 3 and 4). In contrast, the
narrowed segment was 3.5 cm or shorter in all
patients with primary achalasia (Figs. 1 and 2).
Therefore, the length of the narrowed distal
esophageal segment was a useful and statisti-
cally signicant criterion for differentiating
secondary achalasia from primary achalasia on
barium studies (

p

< 0.0001).
In our series, the degree of esophageal dila-
tation above the narrowed segment was also a
statistically signicant criterion for differentiat-
ing secondary achalasia from primary achalasia
Fig. 1.50-year-old man with primary
achalasia. Spot radiograph from double-
contrast barium study shows 1-cm-long
smooth, tapered narrowing (straight
arrow) of distal esophagus with esoph-
ageal diameter proximally of 6 cm. Note
standing column of barium (curved arrow)
on this upright view. Short length of nar-
rowed segment is characteristic of primary
achalasia.
Fig. 2.23-year-old woman with primary achalasia. Spot ra-
diograph from double-contrast barium study shows 3.5-cm-
long, gradually tapered segment of narrowing (straight
arrows) in distal esophagus with esophageal diameter proxi-
mally of 7 cm and standing column of barium (curved arrow).
This was longest segment of narrowing shown on radiogra-
phy in a patient with primary achalasia.
Fig. 3.60-year-old man with secondary
achalasia caused by lung carcinoma. Spot ra-
diograph from single-contrast barium study
shows 5-cm-long symmetric, tapered narrow-
ing (arrows) of distal esophagus with esoph-
ageal diameter proximally of 6 cm. Note ne
irregularity of contour of distal esophagus
above narrowed segment caused by super-
imposed infection with Candida esophagitis
organisms proven on endoscopy.
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AJR:175, September 2000

Woodeld et al.

(

p

< 0.0001). The diameter of the esophagus at
its widest point was 4 cm or less in 80% of pa-
tients with secondary achalasia, whereas the di-
ameter of the esophagus was greater than 4 cm
in 90% of patients with primary achalasia. The
greater degree of esophageal dilatation in pa-
tients with primary achalasia was presumably
related to the more gradual course of the dis-
ease that allowed the esophagus to progres-
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 magnication 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 uoro-
scopic tower above the examining table. This
variable could create a potential bias if
greater magnication occurred primarily in
one group or the other. However, the degree
of magnication was in no way related to pa-
tient selection, so this variable should not
have had a signicant effect on our ndings.
When ndings 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 ndings [4, 6, 13,
Fig. 4.87-year-old woman with
secondary achalasia caused by car-
cinoma of uterus. Spot radiograph
from double-contrast barium study
shows 4-cm-long smooth, tapered
narrowing (arrows) of distal esoph-
agus with esophageal diameter
proximally of 3.5 cm. As in Figure 3, a
narrowed segment longer than 3.5
cm should be considered highly sug-
gestive of secondary achalasia,
even lacking other suspicious radio-
graphic ndings.
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, shelike margins (arrows) in
upper thoracic esophagus.
B A
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AJR:175, September 2000

731

18]. In our series, however, the cardia and fun-
dus could not be adequately evaluated radio-
graphically 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 advo-
cated 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 sec-
ondary achalasia but causes a measurable in-
crease of 2 mm or more in the caliber of this
segment in primary achalasia [19]. Neverthe-
less, this technique has not gained wide-
spread acceptance.
Although our investigation focused on the
usefulness of barium studies for differentiat-
ing the two forms of achalasia, CT may also
be useful in these patients. CT typically re-
veals little or no esophageal wall thickening
and no evidence of a mass at the cardia in pa-
tients with primary achalasia [2022]. 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 esoph-
ageal wall, a soft-tissue mass at the cardia, or
mediastinal adenopathy in patients with sec-
ondary achalasia [21]. CT may also be help-
ful for identifying the site of the primary
tumor in patients with secondary achalasia
caused by remote tumors.
Various clinical parameters are also pur-
ported to be useful for differentiating pri-
mary 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 long-
standing dysphagia (>1 year) and little or no
weight loss (<7 kg) [15, 18], whereas sec-
ondary achalasia is more likely to occur in
older patients (>60 years old) with recent on-
set of dysphagia (<6 months) and substantial
weight loss (>7 kg) [14]. Nevertheless, over-
lap in the clinical presentation has been re-
ported for all these parameters [17, 24]. In
our series, the duration of dysphagia was a
statistically signicant clinical criterion for
differentiating secondary achalasia from pri-
mary 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 signicant but somewhat less
useful criterion for differentiating these con-
ditions (

p

= 0.03); 80% of patients with sec-
ondary achalasia and 38% with primary
achalasia were more than 60 years old. In
two previously published series, 2830% 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 se-
ries had classic radiographic features of second-
ary achalasia such as eccentricity, nodularity, or
shouldering of the narrowed distal esophageal
segment, or suspicious ndings 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 ndings
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 suspi-
cious radiographic ndings.

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