To the Editor:

The diagnosis of esophageal Crohn's disease (CD) is rare, with an adult prevalence of 0.2%–13% in patients with coexisting ileocolonic disease, and a handful of cases of isolated esophageal involvement reported.1 CD of the esophagus almost always affects the distal two-thirds only.2 On review of the literature, only one case of proximal third involvement was identified by the Decker et al3 study, in which the entire esophagus was affected by aphthous ulceration and erosions throughout its length. A unique case of severe CD stricture that predominantly involves the upper esophagus, with relative distal sparing, is reported herein.

A 21-year-old woman with known severe CD presented with an 8-month history of progressively worsening dysphagia for solids. There was associated odynophagia, as well as ongoing postprandial nausea and vomiting. There were no abdominal symptoms or change in function of her permanent colostomy. There were no fevers, or reduction of weight below her baseline of 40 kg. Her CD was diagnosed at age 6, involving both small and large bowel. Current medications included methotrexate and 8-weekly infliximab infusions that were commenced for severe peristomal pyoderma gangrenosum, which was gradually resolving.

Upper endoscopy revealed an inflammatory stricture at 18 cm from the incisor teeth. The mucosa above this level appeared cobblestoned and was friable. A pediatric gastroscope was able to pass the stricture, and gentle dilatation was performed. The patient experienced good resolution of her symptoms and esomeprazole was commenced on discharge. However, she represented 6 months later with recurrence of the same symptoms. On repeat endoscopy a tighter stricture at the same level was seen, which did not allow passage of a pediatric gastroscope. A gastrograffin swallow and computed tomography (CT) chest were performed to further assess the stricture (Fig. 1A,B). The contrast study showed a proximal, very narrow, and irregular long stricture with focal dilatation above. There was evidence of a second stricture distal to this, which was not as narrow or as long. Correlation with CT showed circumferential mural thickening, most marked at C7-T2, measuring up to 1.5 cm. The distal esophagus was of normal caliber. Upper endoscopy was reattempted by the surgical unit in theater under general anesthetic. Successful passage and dilatation of the stricture was performed. Esophageal biopsies were taken, which showed ulcerated mucosa with nonspecific inflammatory changes. There was no granulomatous inflammation identified. There was no dysplasia or malignancy seen.

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Figure 1. (A) Gastrograffin swallow. (B) CT chest.

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Distinguishing esophageal CD from its differentials, in particular reflux esophagitis, can be difficult, as a well-defined diagnostic criteria is yet to be established. Noncaseating granulomas occur in less than 25% of cases4; thus, their presence is not required to make the diagnosis. Based on the clinical history, radiological, and endoscopic findings discussed for this patient, it was agreed among both medical and surgical specialist units that her CD was the underlying pathology for the upper esophageal stricture.

The severe proximal esophageal disease in this case report highlights the fact that the distribution of CD can and does involve any segment of the gastrointestinal tract, from the mouth to the anus.


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Jordan T. Kamel MBBS*, * Department of Upper Gastrointestinal/Hepatobiliary Surgery, St. Vincent's Hospital, Melbourne, Australia.