Congenital chest wall abnormalities may be divided into five categories: pectus excavatum, pectus carinatum, Poland's syndrome, sternal defects, and miscellaneous dysplasias or thoracic deformities found with diffuse skeletal disorders. Surgical approaches to the first four categories will be described in this chapter.
Pectus excavatum, also known as funnel chest, trichterbrust, or thorax en entonnoir, is the most common anterior chest wall anomaly and results from posterior depression of the sternum and the inferior costal cartilages. The male:female ratio is 3:1, and 90% of cases are appreciated by age 1.1 The etiology of this deformity is unknown. Theories include intrauterine pressure, rickets, and abnormalities of the diaphragm.2–4 Pectus excavatum has been seen in association with agenesis of the diaphragm and congenital diaphragmatic hernia, especially after surgical repair.5 There is an association between pectus excavatum and other musculoskeletal anomalies, particularly scoliosis and Marfan's syndrome. A family history has been reported in up to 37% of patients, which may indicate a genetic predisposition.1
Surgical repair of pectus excavatum was first achieved by Meyer in 19116 and Sauerbruch in 1913.7 The method of repair has evolved over the years with increasing experience and as the primary components of the deformity became clear. Early improvements were described by Ochsner and DeBakey in 1939.8 In 1949, Ravitch described a new technique that involved excision of all the deformed costal cartilages including the perichondrium, division of the xiphoid from the sternum, division of the intercostal bundles from the sternum, and transverse sternal osteotomy, by which the sternum was angled anteriorly and secured initially with Kirschner wires and later in the series with silk sutures.9 In 1958, Welch reported his technique based on Ravitch's work,10 and this was further modified and improved, culminating in the series reported by Shamberger and Welch in 1988,11 which is the basis for the current open repair.12
Evaluation and Therapeutic Plan
Treatment of pectus excavatum has two parts: the surgical repair and an exercise and posture program. When pectus excavatum patients are evaluated clinically, they are classified as having mild, moderate, or severe deformities. Patients with mild to moderate deformities are placed on an exercise and posture program and then reevaluated at 12-month intervals. Roughly two-thirds of patients may be treated in this fashion nonoperatively. Patients with severe deformities undergo an evaluation to determine whether they are candidates for surgery. The workup includes pulmonary function studies; a cardiology evaluation, including electrocardiogram and echocardiogram; and a CT scan of the chest. The recommended technique for this CT scan is that it be performed during quiet respirations, not during maximal inspiration.
Surgical correction is recommended if the child has two or more of the following criteria: (1) progressive or symptomatic pectus excavatum, (2) restrictive lung disease as measured by pulmonary function studies, (3) a CT scan showing cardiac compression or displacement, pulmonary atelectasis, and a ...