Christine H. Oh 1 Marc S. Levine 1 David A. Katzka2 Stephen E. Rubesin 1 Lisa W. Pinheiro 3 Michael A. Amygdalos 4 Igor Laufer 1
Congenital Esophageal Stenosis in Adults: Clinical and Radiographic Findings in Seven Patients
OBJECTIVE. The purpose of this study was to determine the clinical and radiographic ﬁndings in seven adults with congenital esophageal stenosis.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.
Received August 29, 2000; accepted after revision October 10, 2000.
Department of Radiology, Hospital of the University ofPennsylvania, 3400 Spruce St., Philadelphia, PA 19104. Address correspondence to M. S. Levine.
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.
0361–803X/01/1765–1179 © AmericanRoentgen Ray Society
ongenital esophageal stenosis is thought to be a rare developmental anomaly, occurring in only one of 25,000–50,000 live births . It has been postulated that this condition results from abnormal embryologic development—defective canalization of the esophagus caused by intrauterine anoxia . Affected individuals classically present during early childhood with dysphagia,vomiting, or aspiration pneumonias [1–6]. Depending on the degree of stenosis, however, some patients may have only intermittent symptoms and eventually seek medical attention during adulthood because of worsening dysphagia, chest pain, or recurrent food impactions [7–11]. 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, 3–6]. 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 ringlikeconstrictions in the region of the strictures. We describe the clinical and radiographic ﬁndings in these seven patients.
Materials and Methods
We collected data on seven patients diagnosed with congenital esophageal stenosis from our hospital and two other area hospitals during a 2-year period from 1998 to 2000. Because the stenotic segment was not resected in any patients, it was notpossible to obtain pathologic specimens for a deﬁnitive diagnosis. Instead, the diagnosis of congenital esophageal stenosis was based on the following criteria [7–11]: a history of dysphagia for 2 or more years; the presence of a stricture revealed on esophagography; no prior history of mediastinal irradiation, caustic ingestion, drug-induced esophagitis, bullous skin diseases, or other conditionsassociated with the development of strictures; and the absence of evidence of Barrett’s esophagus on endoscopy or endoscopic biopsy specimens. Two of our seven patients had been included in a previous study that focused on the clinical and endoscopic ﬁndings of congenital esophageal stenosis in adults . All but one patient had double-contrast esophagrams that included upright double-contrastviews obtained using high-density barium and prone singlecontrast 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...