1/9. Esophageal hypermotility associated with intramural pseudodiverticulosis. Primary esophageal disease or epiphenomena?Esophageal intramural pseudodiverticulosis is a very rare disease of unclear etiology. The clinical picture is characterized by progressive dysphagia. Because of its frequent association with alcohol abuse and subsequent weight loss, it must be differentiated reliably from esophageal carcinoma. The diagnosis is established by the characteristic detection of multiple intramural contrast accumulations in the barium esophagogram. Additional endoscopic and endosonographic confirmation and histological examination are required to exclude a malignant tumor. Moreover, associated diseases are almost always present and should also be diagnosed by pH-metry, cytology, and esophageal manometry. Good and long-lasting therapeutic success can be achieved by bouginage of the stenosis with concomitant treatment of the associated esophageal diseases. Based on two case reports of patients with this disease, we discuss the unusual association with esophageal hypermotility as well as the symptoms, clinical course, therapy, and pathogenesis of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Successful medical treatment of two patients with eosinophilic oesophagitis.BACKGROUND/PURPOSE: Significant oesophageal eosinophilia is associated with oesophagitis and gastroesophageal reflux (GER). Eosinophilic oesophagitis is a rare disease that causes thickening of the oesophageal wall, narrowing of the oesophageal lumen, and severe motor disturbance. methods AND RESULTS: Two 12 yr-old patients with eosinophilic oesophagitis were studied prospectively. Clinical and investigation details are presented. Elemental formula was administered until complete remission of disease. Final outcome was assessed after 3 months on regular diet with exclusion of specific allergic components. Both patients responded to the dietary manipulation. CONCLUSIONS: Eosinophilic oesophagitis must be included in the differential diagnosis when dealing with oesophageal strictures of unknown or unclear aetiology. Elimination diet therapy plays a crucial role in ameliorating the course of the illness. blood eosinophilia correlates with therapeutic response and with improvement of the disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. Congenital oesophageal stenosis: an atypical presentation in a young woman.Congenital oesophageal stenosis is a very rare disease that commonly occurs in infancy with male predominance. This report describes a highly unusual case of congenital oesophageal stenosis extended throughout the length of the oesophagus, without webs or tracheobronchial remnants in the oesophageal wall, with normal oesophageal motility and normal endosonography in an adult female.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. actinomycosis esophagitis in a patient with persistent dysphagia.Many causes of esophagitis exist in immunocompromised patients. Uncommon pathogens must be considered to facilitate timely and appropriate therapy. A limited number of cases of esophageal actinomycosis have been reported. This report describes an unusual case of esophageal actinomycosis in a patient with persistent dysphagia. The broad differential may have delayed definitive diagnosis in the case study patient. biopsy and culture are essential for accurate diagnosis. Although actinomycosis is a rare disease, it should be included in the differential diagnosis of patients presenting with oral or esophageal complaints. It may also be considered as an opportunistic infection in immunocompromised patients. The treatment of choice is parenteral penicillin g, 18 to 24 million units for 2 to 6 weeks followed by oral therapy for 6-12 months.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. Unusual cause of dysphagia: inflammatory pseudotumor of the lung.Inflammatory pseudotumor (IPT) is a rare disease that usually occurs in the lung. patients with IPT are usually asymptomatic, with a solitary pulmonary nodule or mass detected on routine chest roentgenogram. IPT can behave as a malignant tumor both clinically and radiologically. cough, fever, dyspnea, and hemoptysis are the usual presenting symptoms. This report describes the case of a 37-year-old man with a 4-month course of dysphagia secondary to lower esophageal invasion by the posterior mediastinal extension of a lung IPT.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Intramural pseudodiverticulosis of the esophagus in corrosive strictures: report of three cases.First described in 1960, esophageal intramural pseudodiverticulosis (EIPD) is a rare disease characterized by narrow-necked diverticuli confined to the esophageal wall. The cause is debatable, but esophageal strictures commonly accompany this entity. The association with EIPD of strictures caused by corrosives has been reported only once to date. We describe three additional examples.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. Esophageal stricture in Crohn's disease--a case report.We treated a 46-year-old Japanese man with Crohn's disease of the esophagus and for whom medical therapy was adequate. Crohn's disease of the esophagus is a rare disease without specific clinical features and establishment of the diagnosis with guided biopsy is extremely difficult. Therefore, Crohn's disease of the esophagus should be considered in the differential diagnosis of biopsy-negative carcinoma of the esophagus in order to avoid major surgery. Treatment of Crohn's disease of the esophagus should primarily be medical and esophagectomy should only be considered in cases of complications, intractability or a suspicion of malignancy in the biopsied specimen.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. granular cell tumor of the proximal esophagus. A rare disease.In 1926, Abrikossoff described a tumor, usually benign, which only rarely appears in the esophagus. In a 40-year-old woman, the authors found a multicentric granular cell tumor which was localized in the cervical esophagus and in the subcutaneous tissues over the right scapula; it was removed surgically. Granular cell tumors causing stenosis of the upper esophagus have been described only four times in the literature. Characteristics and treatment are discussed.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
9/9. Oesophageal involvement in benign mucous membrane pemphigoid.Benign mucous membrane pemphigoid is a relatively rare disease characterised by the presence of bullous lesions and erosions of the mucous membranes leading to scarring. Oral and conjunctival mucosae are most frequently affected. Occasionally, the oesophagus is involved and two radiological patterns, webs and smooth strictures, are seen.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |