Abnormal formation of the rib cage leading to a
depression deformity of the lower part of the sternum, resulting in a sunken
chest appearance. Most often associated with a large range of disorders
where pectus excavatum is a prominent feature.
Familial Congenital Funnel Chest.
Pectus excavatum occurs in an estimated 1 in 300-400 births in the
United States, with male predominance (male-to-female ratio of 3:1). It is typically noticed at
birth, and more than 90% are confirmed within the first year of life. No
specific data are available in relation to the international frequency; however, it is
probably the same as that reported for the United States. Pectus excavatum
appears to be most prevalent in the white population. Clinical observation
indicates that treating African Americans with pectus excavatum is unusual. The
male-to-female ratio is 3:1. Despite such observation, no known genetic factor
linked to the X or Y chromosome exists.
Usually sporadic. Familial occurrence has also
been reported as an autosomal dominant trait.
The condition may be congenital or acquired. The
acquired form may be secondary, caused by chronic airway obstruction in
infancy secondary to enlarged tonsil and adenoids, laryngomalacia, or
external pressure applied to the anterior surface of the chest. Familial
Congenital Funnel Chest has also been described with an autosomal dominant
inheritance. This condition may result in a restrictive lung pattern defect.
Occasionally, the heart may be shifted leftward, which rarely may adversely
Characteristic morphologic deformities. The superior
manubrium is normal. The sharp slope inward and toward the vertebral column
begins at the manubriogladior junction. The depth of the concavity varies
from a shallow depression to near contact with the vertebral column. Chest
radiography confirms the diagnosis.
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
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.
Preoperative abnormalities of ECG
are common (incomplete right bundle-branch block, left atrium enlargement,
and sinus arrhythmia); usually a result of displacement rather than
intrinsic heart disease. Pulmonary ...