CD is a chronic inflammatory disease of unknown etiology characterized by chronic, granulomatous, segmental transmural inflammation that may occur in any part of the alimentary tract from mouth to anus. In the human upper digestive tract, the esophagus is the least common segment involved in CD [1, 2]. Almost all the esophageal CD reported in the literature has coexisted with CD at other sites, such as the ileum, rectus and colorectum [1, 3, 4]. Here, we report the case of a patient with isolated esophageal CD in need of surgical intervention.
In general, CD of the esophagus is not difficult to diagnose in cases in which other segments of the digestive tract are also involved, or in patients with a prior history of CD. Typical endoscopic and radiographic appearance combined with histological examination contribute to a definitive diagnosis [2, 5].
Previous literature reviews, demonstrate the common endoscopy findings for esophageal CD, which include aphthous ulcers, deep ulcerations, nodularity, erythema, pseudopolyps, stricture and fistula [3, 6], and that the typical histological changes of CD in the esophagus are noncaseating granulomas . Therefore, once a patient presents with the above characteristics, the diagnosis may be feasible. However, the granulomas are not always obvious. To the best of our knowledge, the typical granulomas are more likely located in the deep submucosa and lamina muscularis, difficult to obtain in effective biopsy, and the noncaseating granulomas occur only in less than 25% of reported cases [1, 6, 7]. On the other hand, most cases have presented with only nonspecific endoscopy findings as well as inflammation in pathology, which can be considered compatible with CD in highly-suspect individuals. Because of these conditions, for certain patients in whom the esophagus alone is involved, the diagnosis of esophageal CD is achieved only after definite exclusion of such possible causes as reflux esophagitis, viral esophagus, carcinoma, Behcet’s syndrome, epidermolysis bullosa acquisita, drug-induced ulcer, intramural diverticulosis, and so on. . In our case, the biopsy also revealed that only a nonspecific change caused difficulties in the diagnosis of esophageal CD before surgery. The resected specimen finally confirmed an accurate diagnosis. As has been shown in the literature, three different stages for lesions in esophageal CD have been established. In the first phase, inflammatory lesions, erosions and elongated ulcerations appear from edema of the esophageal mucosa. The patient has no significant symptoms of dysphagia or odynophagia . In the second phase, constrictions of the esophagus and stenosis appear usually on a section of over 1 cm, and the formation of mucosal bridges is observed .
In the third stage, the patient presents with progressive dysphagia and odynophagia with vomiting. At this stage, a few patients can be cured through medication and many patients with persistent dysphagia caused by stricture need esophageal dilatation. This is the end stage of the disease. Severe complications such as severe stricture and fistula occur, and many patients with persistent dysphagia, caused by a stricture, will require esophageal dilatation. Recurrent mediastinal inflammation/abscess or pneumomediastinum, could be signs of fistula formation. Barium swallow, computed tomography and esophagoscopy are used to define fistulae anatomy. Once esophageal fistula is confirmed, surgery is required to prevent further complications [9, 10].
Previous studies have reported fistula formation between the pleural cavity, bronchus, esophageal wall, and even the stomach [3, 11–13]. There was one case report describing successful CD-associated esophagobronchial fistula closure after therapy with infliximab, while another case report describes successful treatment with injection of a liquid polymer [14, 15]. However, we did not identify any reports of CD associated esophago-mediastinal fistula. Our case reports an esophageal fistula to the mediastinum due to the end stage of isolated esophageal CD. Esophagectomy with gastric pull-through was performed in a timely fashion, when the mediastinal infection was limited.