Abstract
Purpose: Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an autosomal recessive genetic disease characterized by hypoparathyroidism, adrenal insufficiency, candidiasis, and an array of other disease associations which does not include eosinophilic esophagitis. A 35 year old female patient with a history of APECED presented as an outpatient with a 3 month history of dysphagia. Esophagogastroduodenoscopy was performed and was unsuccessful due to the inability to intubate the esophagus. A barium esophogram was then performed revealing a tight stricture in the upper third of the esophagus. Given the severity of the stricture on the esophagram and the difficulty with initial EGD, the plan was to proceed with an antegrade-retrograde endoscopy with dilation of the esophageal stricture. Surgery was consulted in order to perform a surgical gastrostomy for insertion of the retrograde endoscope. The patient was taken to the operating room where a gastrostomy was created. An upper endoscope was passed through the site into the stomach to pass a wire into the esophagus through the working channel of the endoscope. Under fluoroscopic guidance, the wire was passed through the stricture into the mouth of the patient. A pediatric endoscope was then passed from the mouth to the stricture after back loading the wire. Serial balloon dilation was performed until the two scopes united. Afterwards, a nasogastric tube was passed over the wire through the stricture ensuring luminal patency. The gastrostomy site was then closed. The procedure was followed by multiple balloon dilations to relieve the pt's symptom of dysphagia. Biopsy of the proximal esophagus revealed 31 eosinophils per high power field consistent with eosinophilic esophagitis. The first unique finding is the possible association of eosinophlic esophagitis with APECED. Secondly, this case presents a unique method of performing antegrade-retrograde endoscopy, namely in performing a gastrostomy for this sole purpose and then closing the site. This may be useful in similar cases in the future where a patient presents with severe strictures or obstruction with no pre-existing gastrostomy.Figure
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