The typical triad consists of laryngeal stenosis,
thoracic dysplasia with a small, bell-shaped chest wall, and a small pelvis.
Affected individuals usually present with respiratory distress at birth, and
tracheal intubation was difficult in all. Tracheostomy was usually indicated
for long-term ventilation. Tracheostomy was performed as an emergency
procedure in one patient at 5 weeks of age because the child could not be
reintubated after a failed extubation trial. This child later underwent
successful laryngotracheoplasty. Because of the small thorax and pelvis, the
abdomen appears big and protuberant in babies. Long-term outcome is not yet
defined. Mortality is high; in fact, the oldest described individual who
underwent thoracoplasty to increase the thoracic diameter in early childhood
eventually died of cor pulmonale at 14 years of age. The children in the two
families were delivered by cesarean section because of cephalopelvic
disproportion (mother with small pelvis, who obviously suffered from a
milder form of Barnes syndrome; however, tracheal intubation reportedly was
difficult secondary to laryngeal stenosis).