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Dilation of an esophageal stricture caused by epidermolysis bullosa. (Esophagoscopy Clinic).

A 27-year-old man with a history of epidermolysis bullosa came to the clinic complaining of progressive dysphagia. He had been unable to swallow solids or liquids for the previous 24 hours. Physical examination revealed the presence of fluid-filled blisters, scarring, and contractures over the dorsal surface of his hands (figure, A). Transnasal esophagoscopy detected an area of severe inflammation and circumferential sloughing of the mucosa in the middle portion of the esophagus, 24 cm from the nasal vestibule (figure, B). A narrow stricture 1 cm in length was seen just distal to the area of mucosal slough (figure, C).

In light of the significant risk that intubation poses in a patient with active epidermolysis bullosa, we elected to perform esophageal dilation in the clinic with Savary dilators (figure, D). Our technique for in-office dilation involves the direct visualization of the area of stricture with a transnasal esophagoscope. A Savary dilator guidewire is inserted through the instrument's biopsy port. Then the esophagoscope is removed, leaving the wire in place as it passes through the stricture and out the patient's nose. A Kelly clamp is used to reach into the patient's mouth and withdraw the wire transorally. Progressively larger Savary dilators are then advanced over the wire to dilate the stricture. Our patient tolerated the procedure easily and did not require sedation. At the 4-month follow-up, he was still eating without difficulty.

From the Center for Voice Disorders, Department of Otolaryngology, Wake Forest University Medical Center, Winston-Salem, N.C.;
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Comment:Dilation of an esophageal stricture caused by epidermolysis bullosa. (Esophagoscopy Clinic).
Author:Koufman, James A.
Publication:Ear, Nose and Throat Journal
Article Type:Brief Article
Geographic Code:1USA
Date:Feb 1, 2002
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