Esophageal intramural pseudodiverticulosis.

Esophageal intramural pseudodiverticulosis (EIPD) is a disease of unknown pathogenesis characterized by usually systemic, cystic dilatation of the excretory ducts of esophageal submucosal glands. In this article, I review the epidemiology, clinical manifestations, endoscopic findings, esophagographic findings, and histopathology of EIPD. I also discuss the etiology and possible pathogenesis of EIPD based on my experiences with this disease and a review of the literature. EIPD usually presents with dysphagia in middle-aged individuals. It is often complicated with secondary infections, most commonly candidiasis. On esophagography, EIPD is delineated as small, multiple, flask-shaped outward projections within the esophageal wall. In recent years, EIPD has been mainly diagnosed by endoscopic findings of multiple, localized, small mucosal depressions. The orifices of the "pseudodiverticula" periodically open and close, and excrete mucus onto the mucosal surface. On histopathological examination, the luminal surface of dilated ducts in EIPD is covered by multilayered, hyperplastic epithelial cells, but myoepithelial cells in the glandular acini are well preserved. Treatment of EIPD is usually symptomatic therapy, and prevention of the infectious complications is important. The etiology and pathogenesis of EIPD are largely unknown, but functional abnormalities of autonomic nerve fibers innervating the esophageal glands likely play an important role, since the structures of the glands are basically preserved in this disease.

Esophageal Intramural Pseudodiverticulosis: A Histopathological and Immunohistochemical Study of 2 Cases.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare disorder characterized by an abnormal, cyst-like dilatation of the excretory ducts of esophageal submucosal glands. We aimed to elucidate the histopathological features and immunohistochemical properties of the epithelial lining of the cyst-like lesions in EIPD. We performed a histopathological and immunohistochemical study of 2 cases (one autopsy and one surgical) of EIPD. The ductal walls consisted of inner ductal cells, which were cytokeratin (CK) 7-positive and CK5/6-negative, and outer basal cells, which were CK7-negative and CK5/6-positive. The ductal epithelium frequently showed squamous metaplasia and rarely simulated a false diverticulum. Immunohistochemistry for CK7 was useful for distinction between the conditions because the surface epithelium was negative for CK7. We also confirmed that myoepithelial cells in the acinar portion of submucosal glands were well-preserved in EIPD, the finding that explained the periodic opening and closing movements of orifices of cyst-like lesions in this disorder. The immunohistochemical properties of the epithelial lining of cyst-like lesions in EIPD were essentially similar to those of the normal ducts of submucosal glands.

Occurrence of a Duodenal Polypoid Lesion During Long-Term Acid Suppression Therapy and Its Regression After Drug Discontinuation.

Formation of multiple fundic gland polyps or hyperplastic polyps in the gastric mucosa is one of the well-known adverse effects of the long-term acid suppression therapy for peptic ulcer disease. However, similar phenomenon has not been reported to occur in the duodenum. We report a case of duodenal polypoid lesion that developed after the long-term use of acid suppressants and disappeared after the cessation of the treatment. The patient was a 76-year-old man with a history of heavy cigarette smoking and excessive alcohol intake who had been treated with medication of gastric acid suppressants, including proton pump inhibitors and potassium-competitive acid blockers, for refractory gastroesophageal reflux disease. After receiving the acid suppression therapy for 3 years, a polypoid lesion of 10 mm in diameter was found at the portion of the duodenal bulb. This polypoid lesion disappeared 1.5 months after the cessation of treatment. We hypothesized that changes in serum gastrin levels caused by acid suppression therapy might have been associated with the development and regression of the duodenal polypoid lesion.

Development of Epidermoid Metaplasia of the Mucosa in Association with Esophageal Intramural Pseudodiverticulosis and Candidiasis.

We report a case of epidermoid metaplasia of the esophageal mucosa that developed in a patient with a long history of esophageal intramural pseudodiverticulosis (EIPD) complicated by candidiasis. The patient, a 69-year-old man, had been treated for about 3 years for EIPD with candidiasis. After candidiasis improved, the development of epidermoid metaplasia of the esophageal mucosa was observed. It comprised longitudinally arranged, multiple, small, whitish flecks with a scaly appearance on the mucosa of the middle to lower esophagus, and pathological examination demonstrated several fine keratohyalin granules in superficial layers of the squamous epithelium. Six months later, candidiasis was almost cured, but these small flecks had slightly increased in size, and pathological examination demonstrated epidermoid metaplasia consisting of a thick, acellular keratin layer and well-developed granular layer beneath it. We considered that chronic candida esophagitis played the principal pathogenetic role in the development of epidermoid metaplasia. EIPD may have provided an environment suitable for the growth of fungi, and mucinous material contaminated by Candida and excreted from the orifices of EIPD may have irritated the mucosa and induced epidermoid metaplasia.