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