Chapter 17

### Key Points

1. Surgical repair of pectus excavatum is indicated for patients with a severe pectus deformity and associated physiologic impairment.

2. Cardiorespiratory impairment associated with severe pectus excavatum has been characterized and measured by pulmonary function testing, morphologic and functional echocardiographic assessment, cardiovascular magnetic resonance imaging (CMR), and incremental exercise testing.

3. Relief of cardiac compression after pectus repair is associated with increased stroke volumes and subsequent improvements in exercise capacity and cardiovascular performance.

4. The optimal timing for pectus excavatum repair appears to be 10 to 14 years of age, when the chest wall is still malleable. Repairs done in this age range are associated with a low incidence of recurrent pectus excavatum.

5. The Nuss procedure is now used for the majority of primary pectus excavatum repairs.

6. Open repair is used primarily for patients with severe asymmetry of the chest wall, mixed pectus deformities, or if there are objections to having a substernal bar in place for 2 to 3 years.

7. Both the Nuss procedure and open repair methods provide excellent results with low complication and recurrence rates.

### Introduction

Pectus excavatum is a depression of the sternum and lower costal cartilages. The depression can appear quite deep and the condition is commonly referred to as “funnel chest.” The most common deformity of the anterior chest wall, pectus excavatum is estimated to occur in 1 in 500 to 1000 children and is much more common in males. While pectus excavatum is usually noted at birth or within the first year of life, it often worsens during the growth spurts that occur during late childhood or adolescence.

### Pathophysiology

The etiology of pectus excavatum remains unknown. Early investigators attributed its development to an abnormality of the diaphragm, but there has been little evidence to support this theory except for the reported occurrence of pectus excavatum in patients with a congenital diaphragmatic hernia. The higher prevalence of pectus excavatum in patients with conditions such as Marfan syndrome has focused attention on defects in collagen formation as a potential etiology. In addition, the incidence of pectus deformities among members of the same family has been reported as high as 40%, suggesting a potential genetic link. Scoliosis has been identified in 26% of patients recently diagnosed with pectus excavatum.

As a structural abnormality of the chest wall, pectus excavatum potentially has a negative impact on the normal mechanics of the respiratory muscles and can limit complete expansion of the heart and lungs by compressing these structures in the thoracic cavity. If it does, this functional impairment could manifest as symptoms of cardiorespiratory insufficiency.

Infants and young children with pectus excavatum rarely have symptoms, but many older children do develop symptoms, particularly in association with exercise. Exercise intolerance is the most common symptom and is considered to result from the inability to increase minute ventilation and/or the inability to increase cardiac output ...

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