++
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.