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.

Symptomatic esophageal intramural pseudodiverticulosis without stricture: A case report.

Esophageal intramural pseudodiverticulosis is a benign disease characterized by numerous, small outpouchings from the esophageal epithelium. Esophageal intramural pseudodiverticulosis has scarcely been reported with only 200-300 cases worldwide. The etiology of esophageal intramural pseudodiverticulosis is also unclear; however, there is an associated increased risk with diabetes mellitus, gastroesophageal reflux disease, esophageal candidiasis, and chronic alcohol and tobacco abuse. Esophageal intramural pseudodiverticulosis has a characteristic appearance on esophagogastroduodenoscopy. Treatment of esophageal intramural pseudodiverticulosis has historically been limited to symptom management with acid suppression, anti-fungal therapy, and endoscopic dilation in areas of stricture. This report is a case of a 52-year-old female status post two esophageal stricture repairs with dilation over prior 2 years, who presented with non-remitting solid food dysphagia and food impaction found to have esophageal intramural pseudodiverticulosis with concomitant jackhammer esophagus and esophageal candidiasis.

Esophageal Intramural Pseudodiverticulosis Diagnosed by Combining Esophagogastroduodenoscopy, Esophagography, and High-resolution Manometry.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare disease. A 78-year-old man with dysphagia presented to our hospital. The presence of diffuse esophageal spasm was suspected by his primary-care doctor. High-resolution manometry (HRM) showed no abnormal findings. The patient was diagnosed with EIPD and Candida esophagitis, by esophagogastroduodenoscopy (EGD) and esophagography. His symptoms improved after symptomatic treatment for Candida esophagitis with oral administration of an antifungal drug. EIPD should be considered in patients with dysphagia; EGD and esophagography should be performed when diagnosing EIPD.

Esophageal Intramural Pseudodiverticulosis / Pseudodiverticulosis intramural del esófago

Abstract A report of two cases of esophageal intramural pseudodiverticulosis, a very unusual disease, with other 240 cases reported in the entire world literature since 1960. Its etiology and pathogenesis are still not fully understood. However, it is believed that hypertrophy of the submucosal glands, with chronic inflammation, fibrosis, and consequent esophageal stenosis, causes dysphagia, which is the primary manifestation of esophageal intramural pseudodiverticulosis. The main diagnostic methods include the radiological examination of the esophagus with barium contrast (esophagogram) and esophagogastroduodenoscopy (EGD). Both reported cases were treated with endoscopic dilation, exemplifying the safety and efficacy of this therapeutic option for treating dysphagia in these individuals.

Resumen Reporte de dos casos de pseudodiverticulosis esofágica intramural, una enfermedad muy inusual, con otros 240 casos reportados en toda la literatura mundial desde 1960. Su etiología y patogenia aún no se conocen completamente; sin embargo, se cree que existe una hipertrofia de las glándulas submucosas, con inflamación crónica, fibrosis y consecuente estenosis esofágica, lo que provoca disfagia, que es la principal manifestación de la pseudodiverticulosis esofágica intramural. El examen radiológico del esófago con contraste de bario (esofagograma) y la endoscopia digestiva alta (EDA) son los principales métodos de diagnóstico. Ambos casos reportados se trataron con dilatación endoscópica, lo que ejemplifica la seguridad y eficacia de dicha opción terapéutica para el tratamiento de la disfagia en estos individuos.

A rare cause of dysphagia due to esophageal intramural pseudodiverticulosis: a case report and review of literature.

BACKGROUND: Esophageal intramural pseudodiverticulosis is an uncommon, idiopathic disorder characterized by multiple small outpouchings protruding from the esophageal lumen. Esophageal intramural pseudodiverticulosis is associated with conditions such as gastroesophageal reflux disease and diabetes mellitus, as well as emergent complications including pneumomediastinum. The most common presenting symptom is dysphagia with associated esophageal stricture formation. While the pathogenesis of EIP has yet to be determined, it is important to bring awareness to this unique disease with distinctive diagnostic findings and treatment options. CASE PRESENTATION: In this case, we present a 62-year-old woman who suffered from dysphagia, an inability to tolerate a regular diet, and unintentional weight loss for several years prior to her diagnoses. She was diagnosed by esophagram and esophagogastroduodenoscopy to have esophageal intramural pseudodiverticulosis, complicated by severe stricture formation. Following treatment with sequential dilatation and maintenance H2-blocker therapy, she achieved significant symptomatic improvement. CONCLUSIONS: This case highlights the importance of accurate identification and treatment of an uncommon cause of dysphagia, esophageal intramural pseudodiverticulosis. Treatment includes dilatational therapy, as successfully demonstrated in our patient. Furthermore, treatment is focused on optimizing medical management, as demonstrated in our patient with the addition of an H2-blocker for GERD, or addressing potentially serious underlying causes, such as carcinoma, with surgery.

A Holistic Approach to Diagnosing and Treating Dysphagia.

We report a 39-year-old Native American female with an almost 20-year history of dysphagia that had increased in the 6 months prior to the initial evaluation. Investigation revealed a number of distinct esophageal disorders including Plummer-Vinson syndrome, gastroesophageal reflux disease with esophagitis, distal esophageal stricture, esophageal intramural pseudo-diverticulosis, and recurrent esophageal Candida infections. Although prolonged therapy with proton pump inhibitors, fluconazole, nystatin, and repeated esophageal balloon dilations relieved her symptoms, her prognosis remains uncertain.

Candida albicans: a cause or a consequence of esophageal intramural pseudo-diverticulosis.

Esophageal intramural pseudo-diverticulosis is a rare disease of unknown etiology. It is characterized by multiple pseudodiverticula with segmental or diffuse involvement of the esophagus. We report, the case of a 78-year-old male who suffered from severe dysphagia. Diagnosis of esophageal intramural pseudo-diverticulosis was based on endoscopic and radiologic explorations. Histological analysis of esophageal mucosal biopsies has shown the presence of candida albicans. Antifungal treatment leads to spectacular improvement of dysphasia. Subsequently, the patient presented a cardio-respiratory failure and died despite adequate treatment.

Pneumomediastinum as a complication of esophageal intramural pseudodiverticulosis.

Dysphagia is a common complaint of patients seen at the outpatient clinic as well as at the emergency room. We report esophageal intramural pseudodiverticulosis (EIPD) as a cause of dysphagia that is less known by physicians and it is rarely described in the literature. EIPD is characterized by multiple, segmental or diffuse, flask-like outpouchings in the esophageal wall corresponding to dilated and inflamed excretory ducts of the submucosal esophageal glands. The underlying etiology remains unclear. Esophageal strictures, esophageal candidiasis and gastroesophageal reflux disease are often associated. The diagnosis can be made by upper gastrointestinal endoscopy, but barium esophagography is the modality of choice. Complications of EIPD are rare and include broncho-esophageal and esophagomediastinal fistula, pleural and pericardial effusion, abscesses, gastrointestinal bleeding from a web-like stenosis or esophageal perforation with pneumomediastinum. The treatment for EIPD should be directed towards treating underlying associated conditions and relieving symptoms rather than the pseudodiverticulosis itself.

Esophageal intramural pseudodiverticulosis as a diagnostic and therapeutic problem.

The article presents the case of a 68-year-old patient with alcohol dependence syndrome, who was admitted, in serious condition, to the Department of Surgery due to esophageal intramural lesions of unclear etiology. The imaging studies showed no signs of transmural perforation of the esophageal wall. Esophagogastroscopy revealed intramural fluid reservoirs and small oval cavities with smooth edges in the esophageal mucosa. The patient was treated conservatively with parenteral nutrition and rehabilitation. Subsequently, the patient was transferred to the intensive care unit because of cardiorespiratory failure. Despite adequate pharmacological treatment, the patient died.

Esophageal intramural pseudodiverticulosis of the residual esophagus after esophagectomy for esophageal cancer.

A 91-year-old man was referred to our hospital with intermittent dysphagia. He had undergone esophagectomy for esophageal cancer (T3N2M0 Stage III) 11 years earlier. Endoscopic examination revealed an anastomotic stricture; signs of inflammation, including redness, erosion, edema, bleeding, friability, and exudate with white plaques; and multiple depressions in the residual esophagus. Radiographical examination revealed numerous fine, gastrografin-filled projections and an anastomotic stricture. Biopsy specimens from the area of the anastomotic stricture revealed inflammatory changes without signs of malignancy. Candida glabrata was detected with a culture test of the biopsy specimens. The stricture was diagnosed as a benign stricture that was caused by esophageal intramural pseudodiverticulosis. Accordingly, endoscopic balloon dilatation was performed and anti-fungal therapy was started in the hospital. Seven weeks later, endoscopic examination revealed improvement in the mucosal inflammation; only the pseudodiverticulosis remained. Consequently, the patient was discharged. At the latest follow-up, the patient was symptom-free and the pseudodiverticulosis remained in the residual esophagus without any signs of stricture or inflammation.

Esophageal intramural pseudodiverticulosis, a rare cause of food impaction: case report and review of the literature.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare, benign condition of uncertain etiology and pathogenesis, which usually presents with either progressive or intermittent dysphagia. Acute presentation with food impaction, requiring emergency esophago-gastroduodenoscopy (EGD), is rare. We report a case of EIPD presenting as food bolus impaction in an elderly black female. The patient had no previous history of dysphagia or odynophagia. Currently accepted risk factors, such as diabetes mellitus, chronic alcoholism, and reflux esophagitis, were not present in our patient. Emergency EGD established the diagnosis and also dislodged the food bolus. Histopathological evaluation of the mucosa diagnosed co-existent acute candidal infection. Medical treatment with proton pump inhibitor and azole antifungal led to resolution of her symptoms. Review of the literature revealed that stenosis, strictures, perforation, gastro-intestinal bleed, and fistula formation are potential complications of EIPD. Multiple motility abnormalities have been described but are not consistent. Treatment of the underlying inflammatory and or infectious condition is the mainstay of management of this unusual condition.

A Rare Coincidence of Esophageal Intramural Pseudodiverticulosis with Esophageal Web

We report a rare case of esophageal intramural pseudodiverticulosis (EIPD) associated with esophageal web in a 67-year-old man presenting with dysphagia. EIPD is characterized by multiple tiny flask-shaped outpouchings of the mucosa that extend into the muscular layer on esophagography. EIPD commonly presents with stricture and less commonly with esophageal web. Although etiologies of both EIPD and esophageal web are unclear, a chronic inflammatory condition has been proposed. Treatment of EIPD is usually directed at the associated conditions rather than at the pseudodiverticulosis itself. In our case, dysphagial was successfully relieved by endoscopic dilatation with incision methods for the esophageal web.

A case of severe esophageal intramural pseudodiverticulosis whose symptoms were ameliorated by oral administration of anti-fungal medicine.

Esophageal intramural pseudodiverticulosis (EIPD) is a rare disease of unknown etiology that displays multiple pseudodiverticula radiologically, leading to benign esophageal stricture. Dysphagia, which sometimes slowly progresses, is the main symptom in the majority of cases. We here report a 59-year-old male EIPD patient who suffered from severe dysphagia. Radiography and endoscopy of this patient disclosed a severe constriction in the upper thoracic esophagus. Although we tried several endoscopic procedures including frequent endoscopic balloon dilatation (EBD), the effect was very limited and his dysphagia relapsed shortly after the treatments. During the procedures, we noticed some white, thick, creamy liquid emerging from the orifices of EIPD, and PAS staining of biopsy specimens revealed infection with Candida albicans. Hence, the patient was given anti-fungal medicine in addition to EBD. The additional treatment with anti-fungal medicine dramatically improved his symptoms and the esophageal constriction. This case suggests that anti-fungal treatment is an effective first-line therapy even against a severe form of esophageal constriction in EIPD.

A case of esophageal intramural pseudodiverticulosis.

Esophageal intramural pseudodiverticulosis (EIP) is a rare benign disease that is characterized by multiple tiny flask-shaped outpouching lesions of the esophageal wall. The etiology is unknown, but the pathologic findings include dilatation of excretory ducts of submucosal glands. The predominant symptom is dysphagia, and esophageal stricture occurs frequently. Diseases such as diabetes mellitus, esophageal candidiasis, gastroesophageal reflux disease, and chronic alcoholism are often combined. Since most EIP cases are benign, the mainstream treatment is symptom relief by endoscopic dilatation or medical treatment of accompanied diseases. This report describes the case of a 68-year-old male patient who suffered from chest tightness for 2 months and was diagnosed with EIP. This symptom disappeared after 2 months of medical treatment, and the patient is now being regularly followed up.

A Case of Esophageal Intramural Pseudodiverticulosis

Esophageal intramural pseudodiverticulosis (EIP) is a rare benign disease that is characterized by multiple tiny flask-shaped outpouching lesions of the esophageal wall. The etiology is unknown, but the pathologic findings include dilatation of excretory ducts of submucosal glands. The predominant symptom is dysphagia, and esophageal stricture occurs frequently. Diseases such as diabetes mellitus, esophageal candidiasis, gastroesophageal reflux disease, and chronic alcoholism are often combined. Since most EIP cases are benign, the mainstream treatment is symptom relief by endoscopic dilatation or medical treatment of accompanied diseases. This report describes the case of a 68-year-old male patient who suffered from chest tightness for 2 months and was diagnosed with EIP. This symptom disappeared after 2 months of medical treatment, and the patient is now being regularly followed up.

A Case of Esophageal Intramural Pseudodiverticulosis / 대한소화기내시경학회지

Esophageal intramural pseudodiverticulosis is a rare condition with an unknown etiology, and it is characterized by the typical morpholgic findings of multiple tiny pseudodiverticula in a portion of, or in the entire length of the esophagus. It has two peaks in incidence, the teen years and between the 5th and 7th decade. Most patients present with dysphagia, and radiological narrowing of the esophagus is commonly seen. The clinical course of this condition is benign and dilatation of any strictures, if present, results in an excellent clinical response. We report here on a case of esophageal intramural pseudodiverticulosis in a 76-year-old man who had a 6-year history of dysphagia, and we also include a review of the literature.

A Case of Esophageal Intramural Pseudodiverticulosis / 대한소화기내시경학회지

Esophageal intramural pseudodiverticulosis is a rare disease characterized by multiple, flask-shaped outpouchings in the esophageal wall. These pseudodiverticula represent abnormally dilated excretory ducts of esophageal submucosal gland, which cause is unknown. The most common symptom is dysphagia associated with esophageal stricture. Frequently associated diseases were gastroesophageal reflux disease, diabetes mellitus, candida esophagitis, and chronic alcoholism. We have experienced a patient with dysphagia and anemia, who was diagnosed as esophageal stricture with intramural pseudodiverticulosis and pancreatic pseudoaneurysm. He was treated with esophageal bougienage for the esophageal stricture and transcatheter embolization for pancreatic pseudoaneurysm. This may be the first case of esophageal intramural pseudodiverticulosis reported in Korea.