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A syndrome caused by a defect of cartilage of the first and second generation bronchi leading to complete collapse during expiration. Respiratory syndromes can be severe. Related syndromes are multiple.



Characterized by the absence or markedly diminished cartilage around the bronchi. The exact pathophysiology is still unknown.

Clinical evocation is difficult; computed tomography (CT) scan and endoscopy can confirm the diagnosis.

Chronic respiratory distress in early infancy as a result of bronchial flaccidity. Firstand second-generation bronchi almost collapse during expiration. Air trapping and respiratory distress simulate bronchial asthma. Increased frequency of pulmonary infection.

Evaluate respiratory status (clinical, CT scan, pulmonary function test, arterial blood gas, bacteriological examination). Evaluate airway dynamic (awake fiberoptic evaluation) and the potential for difficult direct laryngoscopy and tracheal intubation.

Patients with structural abnormalities of the tracheobronchial tree can be at increased risk for complications when undergoing surgical procedures that impact airway dynamics. Spontaneous ventilation should be preferred when possible; for example, with regional anesthesia. Perioperative physiotherapy is needed to avoid pulmonary superinfection.

Muscle relaxants are not contraindicated but should be avoided to prevent decrease in airway dynamic.

Mounier-Kuhn Syndrome (Tracheomegaly; Tracheobronchomegaly): Autosomal recessive; death in infancy; musculomembranous tissue projects like corrugations between tracheal cartilaginous rings, composing a congenital tracheobronchomegaly associated with a connective tissue disorder; can also result from parietal fibrosing pulmonary diseases that apply traction to the tracheal walls.

Swyer-James-MacLeod Syndrome: Generally discovered on chest radiograph. Frequent expiratory adenovirus infections, decrease exercise tolerance, arterial desaturation and hemoptysis. Frequent pneumothorax may probably contraindicate the use of nitrous oxide.

Yellow Nail Syndrome: Associates yellow nails (89%), lymphedema (80%), and pleural effusion (36%); dilatation of both visceral and parietal pleural lymphatic with perilymphatic inflammation.

Benesch M, Eber E, Pfleger A, et al: Recurrent lower respiratory tract infections in a 14-year-old boy with tracheobronchomegaly (Mounier-Kuhn syndrome). Pediatr Pulmonol 29(6):476, 2000.
Jones VF, Eid NS, Franco SM, et al: Familial congenital bronchiectasis: Williams-Campbell syndrome. Pediatr Pulmonol 16(4):263, 1993.
Palmer SM Jr, Layish DT, Kussin PS, et al: Lung transplantation for Williams-Campbell syndrome. Chest 113:53, 1998.

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