A barium swallow is performed in a 44 year old female who has had difficulty swallowing for months. Radiographically, there is marked dilation of th esophagus with breaking in the distal poion where marked luminal narrowing exists. A biopsy of the lower esophagus shows prominent submucosal fibrosis with out much inflammation. Most likely cause is
Correct Answer: CREST syndrome
Description: Esophageal dysmotility is the "E" in CREST syndrome, the limited form of systemic sclerosis (i.e., scierodernia). Although this disease is autoinmiune in nature, little inflammation is seen by the time the patients come to clinical attention. There is increased collagen deposition in submucosa and muscularis. Fibrosis may affect any pa of the gastrointestinal tract, but the esophagus is the site most often involved. Poal hypeension gives rise to esophageal varices, not fibrosis. For a diagnosis of Barrett esophagus, columnar metaplasia must be seen histologically, and there \ is often a history of gastroesophageal reflux disease. An. upper esophageal web associated with iron deficiency anemia might produce difficulty in swallowing, but this is a rare condition. Hiatal hernia is frequently diagnosed in persons with reflux esophagitis, and this can lead to inflammation, ulceration, and bleeding, but formation of a stricture is uncommon.
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