1/7. Rapidly progressing case of Mounier-Kuhn syndrome in early childhood.Reports of Mounier-Kuhn syndrome in childhood are extremely rare, as it usually presents in the third or fourth decades. In the only other report of a case diagnosed in early childhood, the child was well at the time of publication. We report on a 15-month-old boy who presented with his first respiratory illness at age 3 months. His disease course was more severe than previously reported. diagnosis was characteristically delayed until acute respiratory failure complicated a respiratory tract infection at 13 months, prompting high-resolution computerized tomography (HRCT) of the chest. He gradually deteriorated, eventually requiring ventilatory support; death occurred at age 15 months. This report illustrates the clinical heterogeneity of the syndrome. We review the theories about etiology and the recognized clinical findings in adults. incidence may be higher than previously estimated, and tracheobronchomegaly (TBM) should be considered as a cause of respiratory failure and recurrent pneumonia in children where other investigations, including chest X-ray (CXR), are normal. A chest HRCT (cHRCT) scan and bronchoscopy are usually diagnostic.- - - - - - - - - - ranking = 1keywords = chest (Clic here for more details about this article) |
2/7. Renal transplantation in a patient affected by Mounier-Kuhn syndrome.tracheobronchomegaly, also called Mounier-Kuhn syndrome, consists in dilatation of the trachea and major bronchi because of atrophy or absence of their elastic fibers and smooth muscle cells. Standard chest radiography often does not permit diagnosis because only lateral imaging, obtained with x-rays or chest CT scan, shows the true degree of tracheal dilatation. Surgery has no role in tracheomegaly, except for the complications of tracheal stenosis or pneumothorax. The present work reports cadaveric renal transplantation in a 43-year-old woman affected by end-stage renal disease and suffering from congenital tracheobronchomegaly diagnosed during the first decade of life. No surgical or anesthetic problems were encountered during the immediate perioperative period. The patient did not require pulmonary physiotherapy. antibiotic prophylaxis was given for 10 days. No pulmonary infection developed, and the patient was discharged from the hospital asymptomatic with normal renal function at 25 days after the transplant. Four months later, the patient experienced bronchitis with cough and fever. Antibiotic therapy was performed with totally resolution of symptoms. At 8 months of follow-up after kidney transplantation, the patient is asymptomatic with normal renal function.- - - - - - - - - - ranking = 0.66666666666667keywords = chest (Clic here for more details about this article) |
3/7. Congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a report of 10 cases and review of the literature.Mounier-Kuhn syndrome is a congenital abnormality of the trachea and main bronchi characterized by atrophy or absence of elastic fibers and thinning of muscle, which allows the trachea and main bronchi to become flaccid and markedly dilated on inspiration with narrowing or collapse on expiration or cough. The abnormal airway dynamics and pooling of secretions in broad outpouchings of redundant musculomembranous tissue between the cartilaginous rings predispose to the development of chronic pulmonary suppuration, bronchiectasis, emphysema, and pulmonary fibrosis. A broad spectrum of clinical abnormalities has been documented in Mounier-Kuhn syndrome, ranging from minimal disease with good preservation of pulmonary function to progressive disease leading to respiratory failure and death. In the appropriate clinical setting, Mounier-Kuhn syndrome is diagnosed in women from chest radiographs when the transverse and sagittal diameters of the trachea exceed 21 mm and 23 mm, respectively, and when the transverse diameters of the right and left main bronchi exceed 19.8 mm and 17.4 mm, respectively. In men it is diagnosed when the transverse and sagittal diameters of the trachea exceed 25 mm and 27 mm, respectively, and when the transverse diameters of the right and left main bronchi exceed 21.1 mm and 18.4 mm, respectively. The diagnosis can be confirmed easily by computed tomography.- - - - - - - - - - ranking = 0.33333333333333keywords = chest (Clic here for more details about this article) |
4/7. tracheobronchomegaly in association with ankylosing spondylitis.We present a case of tracheobronchomegaly seen in association with ankylosing spondylitis. To the authors' knowledge this combination has not been previously described. A review of the chest radiographs of 30 other patients with ankylosing spondylitis revealed no evidence of tracheal dilatation.- - - - - - - - - - ranking = 0.33333333333333keywords = chest (Clic here for more details about this article) |
5/7. Acquired tracheobronchomegaly resulting in severe respiratory failure.tracheobronchomegaly is a distinct clinical and radiologic entity characterized by marked dilatation of the trachea and bronchi. In this report we describe a Marfanoid patient who developed TBM in his adulthood with rapid deterioration, leading to hypercapnic respiratory failure. This case represents an acquired form of TBM in a patient with Marfanoid features which has not been previously described. tracheobronchomegaly should be considered as a cause of chronic respiratory failure in a young adult and can be diagnosed easily with chest roentgenogram and CAT scan.- - - - - - - - - - ranking = 0.33333333333333keywords = chest (Clic here for more details about this article) |
6/7. MRI of tracheobronchomegaly.tracheobronchomegaly, a disorder of uncertain etiology, is manifested by the enlargement of the trachea and mainstem bronchi. While the diagnosis may be suggested on chest radiographs, bronchography in the past has been the definitive diagnostic modality. Recently, however, computed tomography has been used to confirm the diagnosis of TBM. We have now demonstrated TBM using MRI, and, in this patient, have shown it to be as good as CT.- - - - - - - - - - ranking = 0.33333333333333keywords = chest (Clic here for more details about this article) |
7/7. tracheobronchomegaly: an unusual presentation of a rare condition.tracheobronchomegaly is a rare cause of recurrent chest infections often with persistent, unproductive cough. A case is described which presented as a severe life threatening pneumonia in which the bronchoscopic, radiographic, and computed tomographic findings are given.- - - - - - - - - - ranking = 0.33333333333333keywords = chest (Clic here for more details about this article) |