Pectus Excavatum

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 cardiac function.

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 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.

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 be assessed with tomography or CT scan in those patients with severe pectus excavatum. Assess for other syndromes. Important to have a detailed postoperative pain plan.

General anesthesia with controlled ventilation is employed. Invasive arterial pressure monitoring is not necessary unless there is significant underlying cardiopulmonary disease. Abnormal thoracic configuration can cause respiratory distress secondary to severe compression of the central airways. A fiberoptic bronchoscopic examination may be necessary in these patients to ensure that the endotracheal tube is below the obstructed area to optimize intraoperative ventilation. Possibility of pneumothorax during surgical correction. Massive bleeding from the internal thoracic artery has also been reported. Most patients can be extubated at the end of the surgery. Supplemental oxygen therapy is often necessary in the postoperative period. Postoperative pain leads to splinting of the chest, which can cause atelectasis and/or pneumonia. Epidural anesthesia have been shown to reduce severe postoperative pulmonary complications. Other common postoperative complications include residual pneumothorax and pleural effusions.

No particular anesthetic agent is indicated or contraindicated. Some would recommend that nitrous oxide be avoided in view of the possibility of pneumothorax development intraoperatively.

A pectus excavatum is considered a prominent feature of the following conditions: Marfan Syndrome; Ehlers-Danlos Syndrome; Homocystinuria; Osteogenesis Imperfecta; Down Syndrome; Turner Syndrome; Noonan Syndrome; Rett Syndrome; Cutis Laxa, X-Linked; Beals Syndrome; Shprintzen-Goldberg Craniosynostosis Syndrome; Camptodactyly Syndrome.