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At a glance

Abnormal formation of the rib cage leading to a depression deformity of the lower part of the sternum, resulting in a sunken chest appearance. Associated with a large range of disorders where pectus excavatum is a prominent feature.

Synonym

Familial Congenital Funnel Chest.

Incidence

Estimated 1:150 to 1:1,000 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 and most prevalent in the white population.

Genetic inheritance

Usually sporadic. Familial occurrence has also been reported as an autosomal dominant trait.

Pathophysiology

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 cardiac function.

Diagnosis

Characteristic morphologic deformities. The manubrium is normal. The sharp slope inward and toward the vertebral column begins at the manubrio gladior junction (junction between the sternum, the manubria and the clavicles). The depth of the concavity varies from a shallow depression to near contact with the vertebral column. Chest radiography confirms the diagnosis.

Clinical aspects

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 to 60% of cases. Exercise tolerance is usually normal. The Ravitch-Sutherland procedure, and 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.

Precautions before anesthesia

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 function is restricted in patients with severe pectus excavatum and repeated chest infections. The tracheobronchial tree should ...

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