It is usually an isolated skeletal anomaly. The
deformity at birth may be minimal or extensive, and may progress, regress,
or remain stationary. With time, there is restricted growth of the thorax in
the anteroposterior direction but lateral development is unrestricted. In
the newborn, the pliable sternum may move paradoxically with respiration but
is otherwise asymptomatic. In the older child, there may be decreased effort
tolerance, chest pain, wheezing, stridor, and repeated upper respiratory
tract infections. The narrowing of the thoracic cavity may lead to
restrictive lung disease. It may be associated with segmental
bronchomalacia, especially involving the left main stem bronchus. Cardiac
dysfunction, especially the right side of the heart, may occur in some
patients.
Mitral valve prolapse has been reported in 20-60% of cases. Exercise
tolerance is usually normal.
The Ravitch-Sutherland procedure, and, more recently, a minimally
invasive method, is usually performed to correct respiratory and/or cardiac
functional impairment caused by the malformation or mainly for cosmetic
reasons. Good functional and cosmetic long-term results are obtained.