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Original Report |
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.
3
Department of Radiology, Presbyterian Medical Center, Philadelphia, PA
19104.
4
Department of Radiology, Bayhealth Medical Center, Dover, DE 19901.
Received August 29, 2000;
accepted after revision October 10, 2000.
Address correspondence to M. S. Levine.
Abstract
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CONCLUSION. In young or middle-aged individuals, particularly men with long-standing dysphagia, an upper or mid esophageal stricture with multiple ringlike constrictions is a characteristic appearance of congenital esophageal stenosis on double-contrast esophagography.
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The radiographic findings of congenital esophageal stenosis have been well documented in infants and small children; esophagography has typically revealed webs or strictures in the thoracic esophagus [1, 3,4,5,6]. Much less frequently, congenital esophageal stenosis has been reported in adults as a cause of strictures visualized on barium studies [7, 8, 10]. However, we have encountered seven patients with congenital esophageal stenosis in whom double-contrast esophagrams revealed characteristic findings with multiple ringlike constrictions in the region of the strictures. We describe the clinical and radiographic findings in these seven patients.
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All but one patient had double-contrast esophagrams that included upright double-contrast views obtained using high-density barium and prone single-contrast views obtained using low-density barium. In the final patient, only upright double-contrast views were obtained. The radiographs were reviewed to determine the morphologic features of the strictures. The original radiology reports were also reviewed for the presence or absence of a hiatal hernia, gastroesophageal reflux, and abnormal esophageal motility.
Six patients underwent endoscopy (in four, biopsy specimens were obtained), and two underwent endoscopic sonography. The endoscopic and histopathologic findings were reviewed. Medical records were also reviewed to determine the clinical presentation.
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Six patients underwent endoscopic dilatation procedures. There was substantial improvement or resolution of dysphagia in three. In the remaining three patients, follow-up data were too limited to determine the response to treatment.
Radiographic Findings
Double-contrast esophagrams revealed smooth, tapered strictures in all
seven patients. The strictures were located in the upper third of the thoracic
esophagus in three patients (Figs.
1 and
2A,2B),
the middle third in three (Fig.
3), and the lower third in one
(Fig. 4). The strictures had an
average length of 4.6 cm (range, 2-7 cm) and an average narrowest width of 1.4
cm (range, 0.6-2.5 cm). In all cases, the strictures contained smooth,
straight, ringlike constrictions (Figs.
1,2A,2B,3,4).
The average number of constrictions was 5.6 (range, 4-9 constrictions). In all
but one patient, the distance between constrictions was 1-2 mm. In the
remaining patient, the constrictions were up to 1 cm apart.
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In three patients who swallowed 12-mm barium tablets, there was prolonged retention of the tablets above the strictures (Fig. 2B). The strictures were visible on both upright double-contrast and prone single-contrast esophagrams in six patients. The remaining patient did not have single-contrast views. The ringlike constrictions were seen on double-contrast images in all seven patients and on single-contrast images in five of the six patients for whom single-contrast esophagrams were available. However, the ringlike constrictions were not as visible on single-contrast images in two of these five patients (Fig. 2B).
Other findings included mild gastroesophageal reflux in one patient, a small hiatal hernia in another, and abnormal esophageal motility (with a weakened amplitude of peristalsis and nonperistaltic contractions) in another.
Endoscopic, Histopathologic, and Sonographic Findings
Endoscopic findings confirmed the presence of strictures in all six
patients who underwent this procedure. In three patients, endoscopy also
revealed thickened, cartilagelike rings corresponding to the ringlike
constrictions seen on the barium studies. In all six patients, the mucosa
overlying the strictures appeared normal without endoscopically visualized
Barrett's esophagus. In the four patients in whom endoscopic biopsy specimens
were obtained from the region of the strictures, mild inflammatory changes
were found with no evidence of Barrett's mucosa or tumor. In one patient,
however, biopsy specimens revealed glandular mucosa suggestive of
short-segment Barrett's esophagus in the distal esophagus near the
gastroesophageal junction.
In two patients, endoscopic sonography revealed circumferential, hypoechoic wall thickening of the esophagus in the region of the stricture with disruption of the normal wall layers at this level.
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Patients with severe forms of congenital esophageal stenosis typically present during infancy or early childhood with symptoms of progressive dysphagia on ingestion of solid food and vomiting [1,2,3,4,5,6]. However, patients with milder forms of stenosis can modify their eating habits, so that dysphagia does not develop until adolescence or even adulthood [8,9,10,11]. Some individuals eventually seek medical attention because of a long-standing history of intermittent dysphagia or occasional episodes of food impaction [7,8,9,10,11].
Most adults with congenital esophageal stenosis reported in the literature have been younger than 40 years old at the time of diagnosis, but this condition has been reported in patients as old as 63 years [9]. Although congenital esophageal stenosis occurs in infants or children of both sexes with equal frequency [4], almost all reported adults have been men [7,8,9,10,11]. Whatever the reason for this discrepancy, we believe that congenital esophageal stenosis occurs in adults more commonly than is generally recognized and that, in the past, it has not often been diagnosed when evidence of it appears on barium studies because of radiologists' lack of familiarity with the condition.
In previous reports, congenital esophageal stenosis in adults has usually been characterized on barium studies by smooth, tapered strictures in the esophagus [7, 8, 10]. The strictures in our patients also appeared as smooth, tapered areas of narrowing that ranged from 2 to 7 cm in length and were predominantly located in the upper or mid thoracic esophagus. However, all the strictures in our patients contained multiple ringlike constrictions, producing a characteristic appearance on double-contrast esophagography (Figs. 1,2A,2B,3,4). Although these ringike constrictions were not seen on the single-contrast barium studies in two previously reported cases of adults with congenital esophageal stenosis [7, 8], they were seen in one patient who underwent double-contrast esophagography [10]. The data therefore suggest that these ringlike narrowings are easier to detect on images obtained with a double-contrast technique.
The etiology of the ringlike constrictions in congenital esophageal stenosis is uncertain because of a lack of definitive histopathologic correlation. As we discussed earlier, in some patients the condition is characterized by the presence of tracheobronchial remnants in the esophageal wall, including cartilaginous tissue [1, 3,4,5,6]. These ringlike constrictions therefore could represent actual cartilaginous rings in the wall of the stenotic segment. In fact, cartilagelike rings were seen at endoscopy in three of our patients and have been described as having the appearance of "tracheal" rings at endoscopy [9]. On the other hand, congenital esophageal stenosis associated with tracheobronchial remnants has usually been reported as affecting the distal esophagus [1, 3], whereas all but one of our patients had upper or mid esophageal strictures. Also, congenital esophageal stenosis with tracheobronchial remnants is thought to be less amenable to endoscopic dilatation, sometimes requiring surgical resection of the stenotic segment [6]. In contrast, adults with congenital esophageal stenosis often have an adequate response to dilatation procedures without the need for surgery [7, 11]. The data therefore are inconclusive about the origin of these ringlike constrictions.
A similar appearance has also been described on double-contrast esophagrams obtained in patients with fixed transverse folds, which are seen as a series of horizontal collections of barium in a "stepladder" arrangement caused by trapping of the barium between the folds [12]. However, these fixed transverse folds usually occur in the distal esophagus in patients with peptic strictures [12], whereas the ringlike constrictions in our patients were associated with strictures in the upper or mid esophagus in all but one patient.
The presence of these distinctive ringlike constrictions in our patients with congenital esophageal stenosis helps to differentiate this condition from other causes of stricture formation in the upper or mid esophagus, including Barrett's esophagus, mediastinal irradiation, caustic ingestion, drug-induced esophagitis, and rare bullous diseases involving the esophagus. The clinical history is also important for differentiating these various causes of strictures in the upper or mid esophagus from congenital esophageal stenosis.
Our study is limited by a lack of definitive histopathologic correlation. However, none of our patients with a final diagnosis of congenital esophageal stenosis had evidence of Barrett's esophagus in the region of the strictures at endoscopy, and none had any other risk factors for the development of these strictures. Moreover, all our patients had typical clinical findings of the adult form of congenital esophageal stenosis with a long-standing history of intermittent dysphagia.
In conclusion, we believe that congenital esophageal stenosis is a more common condition in adults than has generally been recognized and that it is likely to be diagnosed with increased frequency as radiologists become more familiar with this condition. In young or middle-aged individuals, particularly men with long-standing dysphagia, an upper or mid esophageal stricture with multiple ringlike constrictions is a characteristic appearance of congenital esophageal stenosis on double-contrast esophagography.
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