139: Overview: Benign Disorders of Chest Wall

Introduction

This section on chest wall disorders encompasses benign disorders of the skeletal and muscular chest wall, including congenital deformities of the chest wall, a group of compressive disorders known collectively as thoracic outlet syndrome, thoracic sympathectomy for hyperhidrosis, an autonomic disorder involving the sympathetic nerve chain, and management of chest wall infections (Fig. 139-1).

Chest Wall Deformities

Congenital chest wall deformities may be categorized as (1) pectus excavatum, (2) pectus carinatum, (3) Poland syndrome, (4) sternal defects, and (5) miscellaneous anterior chest wall defects. While most patients present during childhood, some may present in early adulthood as well.

Pectus Excavatum

Pectus excavatum, or “funnel chest,” is a congenital deformity characterized by posterior depression of the middle to inferior portion of the sternum and posterior curvature of the associated costal cartilages (Fig. 139-2). Generally, the manubrium and first and second ribs are normal. The severity of the depression varies and is usually asymmetric with a deviation to the right side. Shamberger and Welch reported that most cases (86%) are diagnosed at or within a few weeks of birth.¹ Although it was once commonly believed that children would “outgrow” this deformity, the severity of the sternal depression may increase during periods of rapid growth.

Pectus excavatum is the most common congenital chest wall deformity in children, with a reported incidence of 1 in 300 to 400 to 1 in 1000 live births. A male predominance is observed, with boys reported to be affected three times more often than girls.¹ The etiology of pectus excavatum is unknown. Theories include intrauterine pressure, rickets, and abnormalities of the diaphragm, including congenital diaphragmatic hernia and diaphragmatic agenesis.² Although no clear pattern of inheritance is known, Shamberger and Welch note that 35% of patients report a positive family history for chest wall deformities.¹

Pectus excavatum may be accompanied by other abnormalities. In a study of 704 children with pectus excavatum, musculoskeletal abnormalities, such as scoliosis or kyphosis, were identified in 19%. Pectus excavatum has been associated with congenital heart defects (1.5%) and connective tissue disorders such as Marfan syndrome or Ehlers–Danlos syndrome in up to 6.4%.³ Although many pediatric patients are asymptomatic, symptoms can worsen with age, with increased physical activity or worsening of deformity during the second growth spurt.⁴ A recent multicenter prospective trial demonstrated that approximately two-thirds of patients reported dyspnea or other chest symptoms with limited exercise tolerance (64.5%), shortness of breath at rest (62.1%), and chest pain on exertion (51.1%). Chest pain unrelated to ...