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)
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)
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. The diagnosis can usually be made by measuring the tracheal diameter. We report the case of a 40-year-old black man with refractory lower respiratory tract infection. Tracheobronchomegaly was confirmed through computed tomography. (+info)
Mounier-Kuhn syndrome: a rare cause of severe bronchial dilatation with normal pulmonary function test: a case report.
Tracheobronchomegaly (TBM) (Mounier-Kuhn syndrome) is dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth muscle cells. We present a case of TBM with normal pulmonary function test (PFT). The patient was a 37-year-old man with increasing productive cough and without fever, wheezes, chest pain, weight loss or any respiratory disease. Chest helical computed tomography (CT) scan showed tracheomegaly with transversal diameters of the trachea of 44mm. CT scan showed collapse of the trachea. Few large diverticular out-pouching and openings in the trachea was seen in bronchoscopy. PFT results were normal. PFT in large airway disorders may be normal while abnormalities may indicate underlying small airway disorder. An underlying small airway disorders is responsible for abnormal reports in PFT of these patients. We may need to re-evaluate the role of PFT within follow-up of patients with large airway disorder. (+info)
Mounier-Kuhn syndrome: a rare cause of bronchial dilation.
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function testing. Patients may be asymptomatic; however, symptoms can range from minimal with preserved lung function to severe respiratory failure. Therapy, if any, is supportive but minimal. Surgery rarely has a place in the treatment of Mounier-Kuhn syndrome.Herein, we report the case of a 58-year-old man with chronic obstructive pulmonary disease who had a chronic cough, increased sputum production, and chest pain. Thoracic computed tomography showed tracheal dilation (diameter, 34 mm) and multiple diverticula in the posterior region of the trachea. Fiberoptic bronchoscopy revealed enlarged main bronchi, the dilated trachea, and prominent tracheal diverticula. Pulmonary function testing disclosed impaired respiratory function. Histopathologic examination of biopsy specimens from the bronchi and the tracheal wall supported the diagnosis of Mounier-Kuhn syndrome. The patient was released from the hospital and his condition was monitored for 2 years, during which time he developed no lower respiratory tract infections.Regardless of radiologic findings that suggest recurrent lower respiratory tract infection, we recommend that Mounier-Kuhn syndrome be considered in the differential diagnosis. (+info)
Central airway obstruction due to malignant fibrous histiocytoma metastasis in a case with Mounier-Kuhn syndrome.
Malign fibrous histiocytoma is one of the most observed soft tissue sarcomas seen in the adults. The most common metastasis region is the lung and metastasis. Mounier-Kuhn syndrome is characterized by the highly dilatation of the trachea and bronchi. We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinal masses or those with both components together. In this article, we would like to highlight the occurrence of a rare seen clinical situation secondary to the giant mediastinal malign fibrous histiocytoma metastasis and the clinical difficulties experienced in resolving of the main airway obstruction caused by the mass. Since the lack of the similar studies conducted previously, we found the case worth presenting. (+info)
Atypical peritracheobronchial vasculitis and an effective treatment.
We report an interesting case of vasculitis in which the inflammatory lesion was limited to the peritracheobronchus. This case showed positive antineutrophil cytoplasmic antibodies, diffuse peritracheobronchial swelling, and vasculitis in its histology. Steroid therapy was effective for both roentgenological and serological findings. Although the biopsy specimen shows only inflammation and does not satisfy the WHO criteria of Wegener's granulomatosis (WG), a possible diagnosis of WG should not be disregarded. (+info)