1/33. prenatal diagnosis of tracheal obstruction: possible association with maternal pertussis infection. A fetus with the sonographic appearance of echogenic and enlarged lungs and dilated trachea and bronchi, indicating laryngotracheal obstruction, is reported. Additionally, the fetus had ascites and subcutaneous edema and the amniotic fluid volume was reduced. Doppler flow investigation of the systemic venous circulation revealed signs of heart failure, and color Doppler visualized possible increased pulmonary flow. Following termination of pregnancy, autopsy confirmed the sonographic observations and revealed a hypoplastic thymus. During the present pregnancy the mother suffered from sustained cough, and serological tests revealed acute pertussis infection. polymerase chain reaction investigation for bordetella pertussis in the amniotic fluid was negative. The possibilities of pertussis toxins as noxious factors and of an atypical presentation of DiGeorge anomaly are discussed. ( info) |
| tracheobronchomegaly (Mounier-Kuhn syndrome) is characterized by dilatation of the central airways, tracheobronchial outpouchings, and chronic tracheobronchitis. Most cases are diagnosed in adulthood. We report the clinical, radiographic, and bronchoscopic findings in a 14-year-old boy with tracheobronchomegaly, ptosis of the right eyelid, and redundant skin and mucosa of the upper lip, who presented with a 2-year history of recurrent lower respiratory tract infections. Pediatricians should be aware of the possibility that tracheobronchomegaly may cause clinical symptoms in childhood and adolescence. Pediatr Pulmonol. 2000; 29: 476-479. ( info) |
3/33. Tracheomegaly in association with rheumatoid arthritis. Herein we present a case of tracheomegaly seen in a patient with rheumatoid arthritis. To the authors' knowledge, and from a review of the literature, this combination has not been previously described. ( info) |
4/33. A rare cause of dyspnea and fibrocystic pulmonary disease. tracheobronchomegaly (TBM) is a disorder of the major airways characterized by an enlargement of the trachea and main stem bronchi. It is thought to result from a congenital connective tissue defect that results in the trachea and main bronchi becoming flaccid. Although TBM is considered rare, it may be more common than is generally recognized, and may be a seldom-diagnosed cause of chronic lung disease. ( info) |
5/33. Mounier-Kuhn syndrome. A variant of Mounier-Kuhn syndrome is reported where the patient had bronchiectasis with tracheo-bronchomegaly. The patient also had facial anomalies and asthenozoospermia. This combination has not been reported so far in the literature. ( info) |
6/33. 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. ( info) |
| tracheobronchomegaly (TBM) occasionally may progress to extensive tracheomalacia which leads to respiratory failure. spirometry, dynamic expiratory multidetector computed tomography (CT), bronchoscopy are used to diagnose patients of suspected tracheobronchomalacia. We used the technique of night-time monitoring of respiratory variables to show the presence of respiratory abnormalities during sleep and which was corrected by applying nasal continuous positive airway pressure (CPAP). The study showed the presence of both apnoea and hypopnoeas, which were obstructive in nature with an apnoea-hypopnoea index (AHI) of 11, no snoring and associated oxygen desaturation of 75 per cent. A second overnight study with nasal continuous positive airway pressure at a critical pressure of 8 cm, the AHI decreased to 3 along with no drop in oxygen saturation. This non-invasive technique should be considered as a diagnostic tool in tracheobronchomalacia and to know the outcome of CPAP, surgical or stent therapy in this condition. ( info) |
| tracheobronchomegaly is a rare disorder of uncertain etiology, characterized by marked dilatation of the trachea and major bronchi, associated with tracheal diverticulosis, bronchiectasis and recurrent respiratory tract infection. We are reporting a 60-year-old man from the Kingdom of saudi arabia with this condition and review the literature for such a rare entity. ( info) |
9/33. tracheomalacia associated with Mounier-Kuhn syndrome in the intensive care Unit: treatment with Freitag stent. A case report. tracheomalacia is a process characterized by softness of the supporting tracheal cartilages, by the extension of the posterior membranous wall and by reduction of the tracheal antero-posterior diameter. Exceptionally, tracheomalacia can be associated with tracheobronchomegaly or Mounier-Kuhn syndrome. Fibro-bronchoscopy represents the ''gold standard'' for diagnosis. The case of a 79-year-old male observed after hospitalization in a medical ward for chronic pulmonary obstructive disease (COPD) decompensation, and with basal left bronchopulmonary focus, is described. During this period, a progressive worsening of clinical conditions occurred, despite cortisone and antibiotic therapy, and the patient was transferred to the ICU for dyspnea, hypoxia, hypocapnia and with a diagnosis of pulmonary fibrosis. bronchoscopy, performed during spontaneous breathing, revealed tracheomalacia which was responsible for tracheal dynamic complete stenosis during expiration and dynamic subtotal stenosis of the left primary bronchus in the first tract, together with sputum retention. Moreover, this investigation confirmed the diagnosis of tracheobronchomegaly already seen on CT. It was suggested to place a Freitag stent, since the insertion of another model would not have had enough chance of stability, due to the enormous extension of the tracheal lumen and could not have guaranteed good clearance of the secretions. Seven days after this intervention, performed in an outpatients' setting, the patient was dismissed from the ICU, without the help of O2, with good ventilation, saturation in line with his age and good expectoration. ( info) |
10/33. 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. ( info) |