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I'm posting this on my home page hoping I can find somebody else with the same breathing problems. The first one is rare and I have been searching the Internet since 1995 and have found only 2 others with the same condition. I have put this on a page by itself so anybody that is not interested would not have to be bored by reading it. Tracheobronchomegaly is of uncertain etiology, but the disorder is associated with Ehlers-Danlos syndrome. Most cases are sporadic and affect males Patients usually present as young adults with complaints of recurrent infections and chronic cough. The disorder is characterized by dilatation of trachea mainstem bronchi and bronchiectasis. Pathologically there is atrophy of the muscular and elastic elements of the trachea and central bronchi- with an abrupt change to normal caliber at the forth or fifth order of bronchial division. Because of the absence of normal cartilage, the trachea and bronchi can collapse during coughing or normal expiration. As a result of this ineffective cough mechanism, patients have retention of secretions and an increased incidence of recurrent pneumonias (bronchiectasis may be the result of recurrent infections) I found the following statement on the Internet recently, how true I don't know as to how many cases have been reported: Tracheobronchomegaly is characterized by tracheal and main bronchial dilatation usually associated with pulmonary parenchymatous disease. It is a rare condition and less than a hundred cases have been reported so far. Bronchiectasis Bronchiectasis is a disease characterized by irreversible dilatation of the bronchial tree. However, bronchiectasis is not a discrete disease entity, but instead it represents the end-stage of a variety of unrelated and often nonspecific antecedent events. The pathogenesis of this airway damage can be related to three basic mechanisms: obstruction, congenital disorder, and infection. Obstruction can arise from tumor, foreign bodies, impacted mucus, external compression, bronchial webs, or atresia. In the inflammatory form, common infectious agents include Bordetella pertussis , rubella togavirus, respiratory syncytial viruses, and mycobacterium tuberculosis. With both the obstructive and infectious types, inflammation appears to be the common denominator. The congenital form is rare and implies abnormal cartilage formation. Examples include the Williams-Campbell syndrome, in which there is an absence of annular bronchial cartilage, and the Mounier-Kuhn syndrome (congenital tracheobronchomegaly), in which there is a connective tissue disorder similar to that in Ehlers-Danlos syndrome. The anatomic changes from these diverse causes have been classified into three categories: cylindrical, varicose, and saccular or cystic. All types can be present in the same patient. The changes are characterized as follows. In cylindrical bronchiectasis, the bronchial outlines remain regular, but there is a diffuse dilatation of the bronchial wall. The distal bronchiallumen often ends abruptly because the smaller bronchi are plugged with mucus. In the varicosepattern, the extent of bronchial dilatation is greater, and constrictions and sacculations are present. The bronchial terminations appear bulbous or end in constrictive fibrosis. The saccular or cystic form is the most severe type and is characterized by a ballooning of the bronchi with fluid-filled, sac-like dilatations. Clinical Presentation Patients with bronchiectasis usually present with purulent sputum production; however, it is important to recognize that the patient may present only with cough. This has been called (dry bronchiectasis.) Recurrent or persistent bacterial infections are common, and hemoptysis is seen in approximately 50%. Indeed, hemoptysis may be the only sign of disease. Most patients date their problems to early childhood, and many have a history of a childhood contagious disease followed by bacterial pneumonia. Shortness of breath may be present, but this usually is not attributable to the bronchiectasis alone. Bronchitis and emphysema often complicate the clinical picture, which results in an obstructive pattern on pulmonary function testing . A restrictive pattern also can be seen, especially if there is substantial atelectasis and peribronchial fibrosis. On physical examination, rales are a universal finding, and clubbing of the fingers is common. |
Last Updated Wednesday, June 23, 1999
