[Mounier-Kuhn syndrome (tracheobronchomegaly)]. / Zespól Mouniera-Kuhna (tracheobronchomegalia).

The case presents a 43 years old man, cigarette smoker, exposed to wood dust at work, with chronic, ineffective cough symptoms, limited physical exertion and recurring respiratory system infection. Disorders appeared at the age of 33 after severe double-sided pneumonia. Despite the lack of active clinical infection the following microorganisms: Pseudomonas aeruginosa and Staphylococcus aureus MSSA were isolated from patient bronchi mucus. Diagnosis followed image examination (CT, virtual bronchofibroscope). The following was recommended: quitting smoking, avoiding dust exposure at work, physiotherapy to ease mucus removal from bronchi and preventive vaccination. Tracheobronchomegaly consists in trachea clearance and central bronchi widening which disturbs air flow in air-passages and decreases cough effectiveness. Main symptoms are: paroxysmal cough, recurring bronchi inflammation and pneumonia resulting in mucus residing in air-passages.

Recurrent lower respiratory tract infections in a 14-year-old boy with tracheobronchomegaly (Mounier-Kuhn syndrome).

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.

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.