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1 Department of Radiology, Hospital of the University of Pennsylvania, 3400
Spruce St., Philadelphia, PA 19104.
2 Department of Medicine, Hospital of the University of Pennsylvania,
Philadelphia, PA 19104.
Received December 5, 2003;
accepted after revision March 1, 2004.
Address correspondence to M. S. Levine
(marc.levine{at}uphs.upenn.edu).
Abstract
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MATERIALS AND METHODS. In reviewing records in the radiology database at our hospital, we identified 14 patients with diffuse esophageal spasm confirmed on barium studies who also underwent esophageal manometry. The radiographic findings were reviewed and correlated with the manometric findings. Medical records were also reviewed to determine the clinical presentation, treatment, and patient course.
RESULTS. All 14 patients were symptomatic, presenting with dysphagia, chest pain, or both. All the barium studies revealed intermittently absent or weakened peristalsis, with nonperistaltic contractions that were moderate in six patients (43%) and marked in eight patients (57%) (contractions nearly obliterating the lumen in six and completely obliterating the lumen in two). Nine patients (64%) had impaired opening of the lower esophageal sphincter, manifested by beaklike narrowing of the distal esophagus, and five (36%) had normal opening of the lower esophageal sphincter. Manometry revealed abnormal peristalsis in all 14 patients, with repetitive simultaneous contractions in eight (57%) and lower esophageal sphincter dysfunction in 12 (86%). All eight patients with lower esophageal sphincter dysfunction or incomplete relaxation of the lower esophageal sphincter on barium studies or manometry who were treated with the Clostridium botulinum toxin or endoscopic balloon dilatation had a positive response.
CONCLUSION. Our preliminary data show that diffuse esophageal spasm is characterized on barium studies by frequent lower esophageal sphincter dysfunction rather than a classic corkscrew appearance. Barium and manometric studies may have complementary roles in the evaluation of patients with diffuse esophageal spasm.
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Manometric studies of patients with diffuse esophageal spasm have shown that some of these individuals have impaired relaxation of the lower esophageal sphincter [7–9]. Despite these manometric data, the radiology literature usually teaches that diffuse esophageal spasm spares the lower esophageal sphincter without producing achalasialike changes in the distal esophagus on barium studies [4, 6]. In fact, many radiologists are not aware of the association between diffuse esophageal spasm and lower esophageal sphincter dysfunction on manometry. The purpose of our investigation was to reassess the function and clinical characteristics of the lower esophageal sphincter in a series of patients with radiographically defined diffuse esophageal spasm.
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All 14 patients had biphasic esophagrams that included upright left posterior oblique double-contrast images using an effervescent agent (Baros, Lafayette Pharmaceuticals) and a 250% weight/volume (w/v) high-density barium suspension (E-Z-HD, E-Z-EM) and prone right anterior oblique single-contrast images using a 50% w/v barium suspension (Entrobar, Lafayette Pharmaceuticals). For all studies, patients were asked to take multiple discrete swallows in the prone right anterior oblique position so that esophageal motility could be evaluated. The studies were performed by residents, fellows, or one of three attending gastrointestinal radiologists, and all studies were interpreted by the attending radiologists.
The original reports and radiographs from these 14 barium studies were interpreted via the consensus of two of the authors (both experienced gastrointestinal radiologists) without knowledge of the clinical or manometric findings. Videotapes of swallowing studies are not stored by our department on a longterm basis, but the radiographic reports provided a relatively detailed assessment of esophageal motility in these patients. In all cases, we recorded whether primary peristalsis was intermittently absent or weakened. Weakened peristalsis was defined radiographically as delayed propagation or variable disruption of the peristaltic stripping wave as it traversed the esophagus, often associated with incomplete clearance of barium from the esophagus when the peristaltic wave reached the gastroesophageal junction. In contrast, absent peristalsis was defined radiographically as a complete absence of a peristaltic stripping wave in the esophagus. In general, peristalsis was considered abnormal if an abnormal peristaltic wave or esophageal aperistalsis was observed on fluoroscopy on two or more of five separate swallows of low-density barium in the prone right anterior oblique position, as described previously by Ott et al. [10]. We also recorded the strength of any nonperistaltic contractions, which were classified either as mild, moderate, or severe (i.e., lumen-obliterating or nearly lumen-obliterating). Finally, we recorded the presence or absence of a tapered narrowing of the distal esophagus, a well-documented radiographic sign of impaired relaxation of the lower esophageal sphincter in patients with achalasia [5, 6]. Finally, the reports and images were reviewed to determine if any patients had a hiatal hernia.
In all patients, esophageal manometry was performed using a solid-state intraluminal transducer assembly. The catheter was placed in the stomach via a transnasal approach. Intraabdominal positioning of the pressure transducers was confirmed by a rise in pressure during the inspiratory phase of respiration. The patient was then placed in a supine position, and the lower esophageal sphincter was identified on the distal circumferential transducer tracing using the station pull-through technique. Catheter withdrawal was continued until the pressure inversion point was identified and lower esophageal sphincter pressure measurements were obtained distal to the inversion point. Esophageal body motility was then assessed after positioning the distal transducer 3 cm above the proximal border of the lower esophageal sphincter. A series of 10 wet swallows was completed while pressures were recorded at 3, 8, and 13 cm above the lower esophageal sphincter. We analyzed only the 10 wet swallows obtained in the distal esophagus.
One of the authors reviewed the manometric reports for all 14 patients to determine the manometric findings without knowledge of the clinical or radiographic findings in these patients. The manometric tracings were evaluated to determine whether normal peristalsis was present on none of the swallows, 1–20% of swallows, more than 20% of swallows, or all swallows and also whether abnormal simultaneous contractions occurred. The resting pressures and percentage of relaxation of the lower esophageal sphincter were also recorded. Lower esophageal sphincter resting pressures were considered high if they exceeded 45 mm Hg, and lower esophageal sphincter relaxation was considered incomplete if sphincter pressure was greater than 8 mm Hg during swallowing. Finally, to determine the clinical presentation, treatment, and patient course, one of the authors reviewed the patients' medical records without knowledge of the radiographic or manometric findings.
Our institutional review board approved all aspects of this retrospective study and did not require informed consent from the patients whose records were included in our study.
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Radiographic Findings
Eight (57%) of the 14 patients with radiographic findings of diffuse
esophageal spasm had intermittent absence of primary esophageal peristalsis
with normal peristalsis on other swallows, and the remaining six (43%) had
intermittently weakened primary peristalsis with normal peristalsis on other
swallows. Nonperistaltic contractions were moderately severe in six patients
(43%) (Fig. 1) and markedly
severe in eight (57%), with lumen-obliterating contractions that
compartmentalized the esophagus (producing a classic corkscrew appearance) in
two (14%) (Fig. 2) and nearly
lumen-obliterating contractions in six (43%)
(Fig. 3). Nine patients (64%)
also had impaired opening of the lower esophageal sphincter, manifested
radiographically by tapered, beaklike narrowing of the distal esophagus at or
adjacent to the gastroesophageal junction
(Fig. 3), and the remaining
five (36%) had normal relaxation of the lower esophageal sphincter. Twelve
(86%) of the 14 patients had hiatal hernias (Figs.
2 and
3): eight with impaired opening
and four with normal opening of the lower esophageal sphincter.
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Manometric Findings
Manometry revealed abnormal peristalsis with decreased amplitude or absence
of primary peristalsis in all 14 patients. Peristalsis was abnormal on
1–20% of swallows in two patients (14%), on greater than 20% of swallows
in four (29%), and on all swallows in eight (57%). Repetitive simultaneous
contractions were present in eight patients (57%). Manometry revealed lower
esophageal sphincter dysfunction in 12 patients (86%), including five with
incomplete relaxation of the lower esophageal sphincter, one with high resting
pressures in the lower esophageal sphincter, and six with both incomplete
relaxation and high resting pressures in the lower esophageal sphincter. The
final manometric diagnoses were diffuse esophageal spasm with a hypertensive
or abnormal lower esophageal sphincter in four patients, diffuse esophageal
spasm with a normal lower esophageal sphincter in two patients, and vigorous
achalasia in eight patients.
Correlation Between Radiographic and Manometric Findings
Six (75%) of the eight patients with nonperistaltic contractions that
nearly or completely obliterated the lumen on barium studies had abnormal
simultaneous contractions on manometry. Seven (78%) of the nine patients with
impaired opening of the lower esophageal sphincter on barium studies had
incomplete relaxation of the lower esophageal sphincter on manometry (four
patients with high resting pressures and three patients with normal resting
pressures in the lower esophageal sphincter), and the two remaining patients
had normal relaxation of the lower esophageal sphincter (one patient with high
resting pressures and one with normal resting pressures). Conversely, four
(80%) of the five patients who had normal opening of the lower esophageal
sphincter on barium studies had incomplete relaxation of the lower esophageal
sphincter on manometry (two patients with high resting pressures and two with
normal resting pressures), and one had normal relaxation of the lower
esophageal sphincter (with normal resting pressures). Thus, barium studies or
manometry showed incomplete opening or relaxation of the lower esophageal
sphincter in 13 patients (93%) (on barium studies alone in two, manometry
alone in four, and both studies in seven).
Treatment
Six (67%) of the nine patients with impaired opening of the lower
esophageal sphincter on barium studies (including two with normal relaxation
of the lower esophageal sphincter on manometry) underwent treatment with the
C. botulinum toxin (n = 3), endoscopic balloon dilatation
(n = 2), or both (n = 1). In all six patients, symptoms
improved or resolved. Conversely, six (67%) of the nine patients with
incomplete relaxation of the lower esophageal sphincter on manometry
(including two with normal opening of the lower esophageal sphincter on barium
studies) underwent treatment with the C. botulinum toxin (n
= 4) or balloon dilatation (n = 2), and all six also had a positive
response, with improvement or resolution of their symptoms. Thus, all patients
with impaired opening of the lower esophageal sphincter on barium studies or
incomplete relaxation of the lower esophageal sphincter on manometry who were
treated with the C. botulinum toxin or endoscopic balloon dilatation
had a positive response to therapy.
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Another related condition is "vigorous" achalasia, a variant of achalasia characterized by incomplete relaxation of the lower esophageal sphincter and esophageal aperistalsis with frequent, simultaneous, relatively high-amplitude contractions [13]. In various studies, vigorous achalasia has been found to account for as many as 30% of all cases of achalasia [13, 14]. Vigorous achalasia closely resembles diffuse esophageal spasm with associated lower esophageal sphincter dysfunction on manometry, except that patients with vigorous achalasia have no episodes of normal peristalsis, whereas patients with diffuse esophageal spasm have some episodes of normal peristalsis [13]. In the past, some investigators questioned the existence of vigorous achalasia as a separate disease entity, instead hypothesizing that achalasia and diffuse esophageal spasm represent opposite ends of a spectrum of related esophageal motility disorders, with vigorous achalasia occurring as a transitional phase between diffuse esophageal spasm and achalasia [12].
Despite confusion regarding the classification of these motility disorders, incomplete relaxation of the lower esophageal sphincter has been recognized as a common phenomenon on manometry in patients with diffuse esophageal spasm, occurring in more than 35% of the cases [7]. In contrast, reports in the radiology literature have stated that diffuse esophageal spasm is not usually associated with achalasialike lower esophageal sphincter dysfunction [4, 6]. In our series, however, nine (64%) of 14 patients with diffuse esophageal spasm had impaired opening of the lower esophageal sphincter on barium studies with the tapered, beaklike distal esophageal narrowing classically associated with achalasia (Fig. 3). Thus, our preliminary data suggest that most patients with diffuse esophageal spasm have lower esophageal sphincter dysfunction and that this combination of findings is more common than has been previously recognized in the literature.
The classic description of diffuse esophageal spasm on barium studies includes the presence of repetitive, simultaneous, lumen-obliterating nonperistaltic contractions that compartmentalize the esophagus, producing a distinctive corkscrew, "rosary bead," or "shish kebab" appearance [4–6]. In our study, however, lumen-obliterating nonperistaltic contractions were detected on fluoroscopy in only two (14%) of 14 patients who had diffuse esophageal spasm on barium studies (Fig. 2). The remaining 12 patients had nonperistaltic contractions of varying magnitudes that did not obliterate lumen (Figs. 1 and 3). Thus, radiologists should bear in mind that the severity of nonperistaltic contractions can vary in patients with diffuse esophageal spasm and that the absence of a corkscrew esophagus on barium studies in no way excludes this diagnosis.
In our study, all 14 patients with radiographically diagnosed diffuse esophageal spasm had abnormal peristalsis on manometry; 10 (71%) had repetitive, simultaneous contractions and 12 (86%) had lower esophageal sphincter dysfunction. Six patients were classified as having diffuse esophageal spasm on manometry (four patients had associated lower esophageal sphincter dysfunction), and eight were classified as having vigorous achalasia because peristalsis was absent on all swallows (all eight patients had associated lower esophageal sphincter dysfunction). However, the latter eight patients all had intermittent peristalsis on fluoroscopy, so we believe that these patients also had diffuse esophageal spasm with associated lower esophageal sphincter dysfunction rather than vigorous achalasia in which esophageal aperistalsis would be expected to be seen on fluoroscopy on all or nearly all swallows [13].
Manometry is not an infallible technique for evaluating esophageal motility or lower esophageal sphincter function. For example, several investigators have found that 20–30% of patients with achalasia diagnosed on barium studies had normal relaxation of the lower esophageal sphincter on manometry [15, 16]. Because of discrepancies between the radiographic and manometric findings, the precise classification of these motility disorders remains in doubt for some of our patients. Nevertheless, 13 (93%) of the 14 patients in our series had impaired opening of the lower esophageal sphincter on barium studies or incomplete lower esophageal sphincter relaxation on manometry, so we believe that lower esophageal sphincter dysfunction is a major component of diffuse esophageal spasm in most patients with this disorder.
Hiatal hernias are common findings in the general population; such hernias can be detected on barium studies in as many as 40–50% of patients [17, 18]. In contrast, hiatal hernias have been found radiographically in only 1–14% of all patients with achalasia [19–21], so hiatal hernias are much less common in patients with this motility disorder. Some authors even believe that the presence of a hiatal hernia makes a diagnosis of achalasia unlikely because of the rarity of the association between these two entities [19]. On the other hand, one study reported hiatal hernias in 26% of patients with achalasia who were older than 50 years [21]. In our series, 12 (86%) of 14 patients with diffuse esophageal spasm and eight (89%) of nine with diffuse esophageal spasm and impaired opening of the lower esophageal sphincter on barium studies had hiatal hernias on prone right anterior oblique views of the distal esophagus (Figs. 2 and 3). However, the patients in our study had a mean age of 65 years, so the high frequency of hiatal hernias in our study group may be partly related to the older age of our patients.
Patients with diffuse esophageal spasm classically present either with chest pain, dysphagia, or both [1, 2]. However, in our study, 11 (79%) of 14 patients with diffuse esophageal spasm on barium studies had dysphagia (with or without chest pain) and seven (78%) of nine patients with diffuse esophageal spasm and impaired opening of the lower esophageal sphincter on barium studies had dysphagia (with or without chest pain). Our experience suggests that dysphagia is a more common symptom than chest pain in patients with diffuse esophageal spasm, at least partly because of the high frequency of lower esophageal sphincter dysfunction in these individuals.
Several therapeutic options have been used for diffuse esophageal spasm with variable results [1]. Drugs such as calcium-channel blockers, long-acting nitrates, and anticholinergics have been shown to decrease the high-amplitude contractions in diffuse esophageal spasm but have not had a high success rate in relieving dysphagia or chest pain [1]. In contrast, one study reported marked relief of dysphagia from endoscopic balloon dilatation of the lower esophageal sphincter in eight (89%) of nine patients with diffuse esophageal spasm and lower esophageal sphincter dysfunction who were unresponsive to medical therapy [22]. In another study, 21 (72%) of 29 patients with symptomatic diffuse esophageal spasm had a marked reduction in chest pain after endoscopic injection of the C. botulinum toxin at the gastroesophageal junction [23].
In our study, eight patients with impaired opening of the lower esophageal sphincter on barium studies or incomplete relaxation of the lower esophageal sphincter on manometry were treated with the C. botulinum toxin or endoscopic balloon dilatation, and all had a reduction of their symptoms. Although no follow-up studies were obtained, our preliminary data suggest that patients with diffuse esophageal spasm and lower esophageal sphincter dysfunction are more likely to have a clinical response to injection of the C. botulinum toxin at the gastroesophageal junction or to endoscopic balloon dilatation of the lower esophageal sphincter than to medical therapy alone. This observation raises the possibility that lower esophageal sphincter dysfunction is the underlying motor abnormality that is primarily responsible for esophageal symptoms in these individuals. We found frequent discordance between barium and manometric studies in the evaluation of lower esophageal sphincter function. Nevertheless, all patients with impaired opening or relaxation of the lower esophageal sphincter on either test responded to treatment with the C. botulinum toxin or balloon dilatation. Although further investigation is needed to corroborate our findings, we believe that barium studies and manometry may have complementary roles in the evaluation of patients with diffuse esophageal spasm.
Our retrospective study has inherent limitations, including selection bias and possible inaccuracies in recording the patients' symptoms. We also had a relatively small study group because only a minority of all patients with radiographic findings of diffuse esophageal spasm underwent esophageal manometry. If patients with more severe dysphagia were more likely to undergo manometry, our methodology could have favored inclusion of patients who were more likely to have combined diffuse esophageal spasm and lower esophageal sphincter dysfunction, another potential study bias. The relatively long mean interval (3.3 months) between the barium and manometric studies could also have affected our study results. However, none of the patients was treated for esophageal dysmotility during the interval between the two tests. Diffuse esophageal spasm also tends to be a chronic disease, with a gradual transition from diffuse esophageal spasm to achalasia sometimes occurring over a period of years [11, 12], so we believe that this interval between the two examinations did not have a major effect on our study findings. Because of the limitations of our computerized manometry database, we had no choice but to use the findings on barium studies for selection of patients with diffuse esophageal spasm. This study design created another selection bias: patients with diffuse esophageal spasm on manometry who may have had false-negative findings on barium studies were excluded from our analysis. Nevertheless, for assessment of esophageal motility, careful fluoroscopic examinations have been found to have an accuracy of greater than 90% compared with manometry [10]. We also had to rely on the original radiographic reports for characterization of the motility findings because videotapes from these examinations were not stored on a long-term basis. Finally, none of our patients had follow-up barium studies or manometry to ascertain whether opening or relaxation of the lower esophageal sphincter improved in those individuals who had a symptomatic response to treatment. Because of these limitations, a prospective study of a larger series of patients with diffuse esophageal spasm is needed to further elucidate the clinical, radiographic, and manometric findings of this esophageal motility disorder.
In conclusion, our preliminary data show that diffuse esophageal spasm is characterized on barium studies by frequent lower esophageal sphincter dysfunction rather than by a classic corkscrew appearance. All patients with impaired opening of the lower esophageal sphincter on barium studies or incomplete relaxation of the lower esophageal sphincter on manometry who were treated with the C. botulinum toxin or endoscopic balloon dilatation had a positive response to therapy, so these tests may have complementary roles in the evaluation of patients with diffuse esophageal spasm. Radiologists and gastroenterologists should be aware of the radiographic and manometric features of lower esophageal sphincter dysfunction in patients with diffuse esophageal spasm so that they can properly guide treatment of these individuals.
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