1/12. Bronchial granuloma - where's the foreign body?Airway masses are uncommon in children. The majority of bronchial tumors are granulomata secondary to an inhaled foreign body. However, other rare diseases like primary bronchopulmonary tumors should always be considered in the evaluation of a bronchial granuloma in children. The differential diagnosis of bronchial granuloma is presented. We report a 7-year-old girl with a 3-year history of recurrent cough and fevers who was found to have a bronchial granuloma in the left upper lobe bronchus. The diagnosis of foreign body-related granuloma was eventually made after combined and repeated rigid and flexible bronchoscopy. This case highlights the need to search aggressively for a foreign body in the presence of an airway granuloma in children, even in the absence of a history of aspiration.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/12. CT and MRI appearance of a fistula between the right and left main bronchus caused by tracheobronchial tuberculosis.tuberculosis of the trachea and main bronchi is a relatively rare disease seen predominantly in elderly patients. We present a case of a fistula between the right and left main bronchus owing to tuberculosis. We describe the CT and MRI appearances.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/12. Tracheobronchopathia osteochondroplastica: report of three cases.Tracheobronchopathia osteochondroplastica (TO) is a rare disease characterized by the presence of osseous and cartilaginous submucosal nodules in the tracheobronchial tree. The majority of patients remain asymptomatic; however, a small number develop severe airway stenosis. Symptoms may include dyspnea, hoarseness, cough, hemoptysis, and recurrent pneumonia. Plain chest X-ray films are often unremarkable but may demonstrate atelectasis, consolidation, tracheal nodularity, or narrowing. CT reveals tracheal nodularity with calcification and narrowing. This article reviews the cross-sectional imaging characteristics of TO.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/12. Primary endobronchial actinomycosis associated with broncholithiasis.Primary endobronchial actinomycosis is an extremely rare disease that presents with endobronchial mass. We report 2 cases of primary endobronchial actinomycosis associated with broncholithiasis. There was no foreign body material, suggesting that these broncholiths were formed endogenously. Even though it is very rare, endobronchial actinomycosis should be included in the differential diagnosis of calcified endobronchial mass, especially when there is no clinical or radiological evidence of a granulomatous infection, such as tuberculosis, and the yellowish materials obstructing the bronchi are seen during bronchoscopy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/12. Underlying chronic granulomatous disease in a patient with bronchocentric granulomatosis.We present a case of bronchocentric granulomatosis in a woman with no history of asthma who was colonised with Aspergillusfumigatus. A family history of chronic granulomatous disease prompted further testing that demonstrated severely depressed neutrophil oxidant production and gp91(phox) deficiency compatible with the X linked carrier state of chronic granulomatous disease. Only one report of the association of these two rare diseases has previously appeared in the literature. We postulate that an ineffective immune response led to the prolonged colonisation of Afumigatus resulting in a hypersensitivity reaction that was manifest clinically as bronchocentric granulomatosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/12. Congenital bronchial atresia (CBA). A critical review of CBA as a disease entity and presentation of a case series.PURPOSE: To analyse the state of the art of diagnostic imaging in the rare disease entity known as congenital bronchial atresia (CBA) and to suggest new guidelines for diagnosis. MATERIALS AND methods: From January 2002 to December 2003 we examined 6 patients, 4 males and 2 females. Four of them complained of relapsing bronchitis; one patient was admitted with a diagnosis of pulmonary abscess. All patients underwent chest X-ray and MDCT. RESULTS: Five patients presented at chest x-ray the typical association of hilar mass and distal parenchymal hyperinflation that raised the suspicion of CBA; the remaining patient presented an area of parenchymal translucency that prompted us to perform MDCT, which showed severe stenosis of the segmental bronchus. CBA involved the left upper lobe (LUL) in 4 patients and the right upper lobe (RUL) in the remaining 2. In 50% of cases there were associated anomalies: distal bronchiectasis, bronchogenic cyst, anomaly of branching of bronchial tree and vascular structure. CONCLUSIONS: CBA is a relatively rare, generally oligosymptomatic malformation. knowledge of the signs present at chest X-ray can prompt the radiologist to perform a CT scan. MDCT with multiplanar reconstruction is fundamental for the characterisation, localisation and study of the distribution of the lesion, helping to make a precise diagnosis. Surgery is seldom required and at any rate only performed in case of complications.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/12. Ligneous conjunctivitis: an ophthalmic disease with potentially fatal tracheobronchial obstruction. Laryngeal and tracheobronchial features.Ligneous conjunctivitis is a rare disease of unknown cause characterized by pseudomembranous, fibrous, woody, plaquelike deposits on the conjunctiva. The disease appears to be hereditary and/or familial. Deposits similar to those found in the eye occur in the larynx, tracheobronchial tree, nose and nasopharynx, and vagina. When these lesions occur in the larynx and tracheobronchial tree, voice change and potentially life-threatening obstruction and pulmonary disease may occur. This paper reports the findings in a child with ligneous conjunctivitis who was followed for 1 1/2 years and who had laryngeal and tracheobronchial involvement with voice change and airway obstruction. The literature is reviewed and the histopathologic findings and endoscopic findings and management of the patient are documented. Because the patient had multiple papillomata of both hands, a possible association with human papillomavirus was suspected but never confirmed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/12. CT diagnosis of tracheobronchopathia osteochondroplastica.Computed tomography (CT) demonstrated nodular thickening with calcification in the anterior and lateral tracheal walls of a patient with tracheobronchopathia osteochondroplastica. The CT findings in this rare disease are pathognomonic.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/12. Tuberculous tracheobronchial strictures: clinicopathological features and management with the bronchoscopic carbon dioxide laser.Tuberculous tracheobronchial stricture is a rare disease entity at the present time. We report our experiences with carbon dioxide laser treatment for four patients with tuberculous strictures that were resistant to conventional management. All patients were relatively young but were debilitated by the disease. The lesions differed from other forms of benign tracheal strictures as they tended to involve the trachea and right side of the lung and were often multiple. The reasons for this are related to the organism per se and the anatomical arrangement of the tracheobronchial airway. The pathophysiology of tuberculous strictures in the tracheobronchial airway and the technique of bronchoscopic laser resection are outlined.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/12. hemoptysis and hematemesis due to a broncholith: granulomatous mediastinitis.A 40-year-old man had new onset of hemoptysis and hematemesis. bronchoscopy revealed red, inflamed mucosa with apparent rich blood supply in the left primary bronchus. Computed tomography revealed calcified subcarinal lymph nodes with a small extension into the left primary bronchus. Shortly after admission, the patient had an episode of gastrointestinal bleeding. Esophagogastroduodenoscopy showed a lesion in the posterior wall of the esophagus, 12 cm from the upper incisors. During exploratory thoracotomy, a single piece of irregular-shaped tan tissue measuring 3.5 x 2.5 x 2.0 cm and engulfing the esophagus, carina, and left primary bronchus was dissected. A single stonelike mass, or broncholith, was found to involve both the trachea and the esophagus. Microscopic examination showed multiple caseating granulomas with surrounding lymphoid tissue and germinal centers. During 12 months of follow-up, the patient has remained asymptomatic. The chronic inflammation in this case suggested granulomatous mediastinitis, a rare disease whose mass-like effects may contribute to structural compression. The trachea and esophagus are rarely involved. tuberculosis and histoplasmosis are thought to be the two most common causes of granulomatous mediastinitis. Chronic inflammation leading to calcification and broncholith may invade bronchial lumen or esophageal wall, causing life-threatening hemorrhage and necessitating prompt surgical intervention.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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