Clinical and endoscopic characteristics of diffuse esophageal intramural pseudo-diverticulosis.

INTRODUCTION: With 250 published cases worldwide, diffuse esophageal intramural pseudo-diverticulosis (DEIPD) is a poorly understood disease. The aim of this study was to determine the prevalence of DEIPD in our own population, identify risk factors and clinical symptoms, and characterize its typical endoscopic signs. METHODS: Retrospective search in our center's endoscopic and clinical database. Reviewing of all cases by re-examining stored endoscopic photographs. Reviewing of all cases regarding age, sex, risk factors, comorbidities, histology, and clinical symptoms. RESULTS: In a population of 150.000 we found 21 cases of DEIPD. Mean age was 56 ± 10 years. 86% were males, 76% had alcohol abuse, 57% had nicotine abuse, 38% had arteriosclerosis, 33% had COPD, 29% had malignancies, 24% had liver cirrhosis, 19% had impaired kidney function, and 15% had diabetes. Dysphagia was present in 62% and food bolus impaction (single or repeated) in 48%. Endoscopically, 95% of patients had multiple (> 4), small (0.25-2.5 mm) pseudodiverticle openings in the esophageal wall. In 62%, openings were aligned longitudinally. 86% showed edematous swelling of mucosa ("frosted glass look"), 76% showed a fine-grained pattern of small (10-100 µm) red dots ("faux uni pattern"), and 76% had a rigid, narrow lumen with multiple rings ("trachealization"). CONCLUSION: With a prevalence of approximately 5 to 50/100.000, DEIPD may be more frequent than previously estimated. It preferably affects middle-aged male alcoholics. Key symptoms are chronic dysphagia and food impaction. Typical endoscopic findings are multiple, small, longitudinally aligned pseudodiverticle openings, frosted glass look, faux uni pattern, and trachealization of the esophagus.

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.

Incidental diffuse esophageal intramural pseudodiverticulosis. A rare autopsy finding / Pseudodiverticulosis intramural difusa esofágica incidental. Un raro hallazgo de autopsia

Esophageal intramural pseudodiverticulosis (EIPD) is a rare condition of uncertain etiopathogenesis which usually presents with either intermittent or progressive dysphagia. A review of the literature revealed very few studies of its histopathology. We report the case of a 70-year-old diabetic man who died after a road traffic accident. The patient had no history of dysphagia. The entity was discovered incidentally during the medico-legal autopsy. The esophagus showed numerous dilated pseudodiverticula confined to the submucosa and lined by both stratified squamous and cuboidal epithelium. EIPD can remain stable and asymptomatic. This case demonstrates the importance of a thorough investigation during a forensic autopsy (AU)

La pseudodiverticulosis intramural esofágica (PDIE) es un raro proceso de etiopatogénesis incierta que generalmente se manifiesta con disfagia intermitente o progresiva. Una revisión de la literatura ha revelado muy pocos casos publicados mostrando el aspecto histológico de esta condición. Presentamos el caso de un varón de 70 años de edad, diabético, que falleció tras un accidente de tráfico. El paciente no tenía historia de disfagia. La entidad fue descubierta incidentalmente al practicar la autopsia médico-legal. El esófago mostraba numerosos pseudodivertículos confinados a la submucosa y tapizados por epitelio estratificado escamoso y cuboideo. La PDIE puede permanecer estable y asintomática. Este caso demuestra la importancia de una exhaustiva investigación en la autopsia forense (AU)

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 treated by endoscopic balloon dilatation.

We here report a rare case of esophageal intramural pseudodiverticulosis with dysphagia. A 65-year-old man was suffering from intermittent dysphagia for 10 years, which had been worsening in recent months. Endoscopic examination revealed multiple, small, saccular diverticula and mild annular stricture with numerous white plaques from the cervical esophagus to the middle thoracic esophagus. Barium esophagography revealed stricture of the upper thoracic esophagus with multiple tiny flask-shaped outpouchings. Based on this characteristic appearance, we diagnosed esophageal intramural pseudodiverticulosis and inflammation of a narrow segment of the esophagus. Biopsy specimen showed acute and chronic esophagitis with Candida infection. Dysphagia persisted despite resolution of candidiasis, for which we successfully performed endoscopic balloon dilatation of the stricture. After the treatment, the patient has been well and has not been suffering from dysphagia.

Pseudodiverticulosis esofágica intramural: una causa poco frecuente de disfagia / Esophageal intramural pseudodiverticulosis: a rare cause of dysphagia

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[Non-neoplastic esophageal stenosis: not always so benign]. / Sténose œsophagienne non-néoplasique: pas toujours si bénigne.

Esophageal intramural pseudodiverticulosis is a rare pathology whose etiology is unknown, but which is frequently associated with three highly prevalent entities: esophageal reflux disease, esophageal candidosis and alcoholic esophagitis. With conservative treatment the course of these pathologies is usually benign. However, some severe cases are resistant to conservative treatment and may require more aggressive management. We here present the case of patient suffering from a severe esophagitis complicated by chronic mediastinitis with life-threatening repercussions, requiring esophagectomy as treatment.

La pseudodiverticulose œsophagienne intramurale est une pathologie rare, d'étiologie inconnue, mais fréquemment associée à trois entités hautement prévalentes: la maladie de reflux, la candidose œsophagienne et l'œsophagite alcoolique. L'évolution de ces pathologies est habituellement bénigne avec un traitement conservateur. Certains cas sévères nécessitent toutefois une prise en charge plus agressive. Nous présentons ici le cas d'un patient souffrant d'une œsophagite sévère compliquée d'une médiastinite chronique avec des répercussions menaçant sa survie, ayant nécessité une prise en charge chirurgicale agressive.

Intramural pseudodiverticulosis of the esophagus: a case report.

UNLABELLED: Esophageal intramural pseudodiverticulosis (EIPD) is a rare disorder in adults, and even more in infants and children. It is characterized by the dilatation of the submucosal esophageal glands. The exact etiology and pathophysiology of EIPD is, however, unknown. Dysphagia is the predominant presenting symptom in both children and adults. CONCLUSION: Here, we present a case of a boy with persistent dysphagia who had a thorough diagnostic workup and was eventually diagnosed with EIPD.

[Dysphagia and recurrent esophageal stenosis associated with intramural pseudodiverticulosis of the esophagus. A case report]. / Dysphagie et sténose oesophagienne récidivante associée à une pseudodiverticulose intramurale de l'oesophage. A propos d'un cas clinique.

Intramural pseudodiverticulosis of the esophagus is a rare benign disease of the eosphageal wall, with dilation of the submucosal glands, and the predominant symptom is dysphagia. This disorder may be associated with gastroesophageal reflux, motility disorders, candidiasis and alcoholism. Inflammation, resulting in periductal fibrosis and compression of the duct orifices, may be a causative factor. Good and long-lasting therapeutic success can be achieved by bouginage of the stenosis with concomitant treatment of the associated esophageal diseases. Esophageal intramural pseudodiverticulosis is a differential diagnosis in cases of dyspagia and/or esophageal strictures if no other causes are found.