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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.

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Bronchomalacia.

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Unknown.

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

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

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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.

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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.

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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.

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

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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.

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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.

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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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