Pectus excavatum is the most common congenital anterior chest wall defect, characterized by a posterior depression of the sternum and inferior costal cartilages. The deformity can be present at birth or develop during childhood. The sternal depression may worsen as the child grows, often peaking during pubertal growth. Incidence is reported at 1 per 1000 children. It is more common in males; the male to female ratio is 4:1. The etiology of the defect is unknown, although there is a suggestion of an intrinsic abnormality of costochondral cartilage due to the occurrence of pectus excavatum in patients with connective tissue disorders. Scoliosis is present in up to 20% of patients with pectus excavatum. In addition, a family history of pectus excavatum is present in up to 40% of patients, suggesting a genetic predisposition. Approximately one third of all children with pectus excavatum have a severe deformity that warrants evaluation for surgical repair.¹

Meyer first attempted surgical repair in 1911 and Sauerbruch in 1913.¹ In 1939, Ochsner and DeBakey reviewed the techniques of repair and reported outcomes with high morbidity and mortality.¹ Ravitch² described a technique in 1949 involving division of xiphoid from sternum, excision of deformed costal cartilages, division of intercostal bundles from sternum, transverse sternal osteotomy at junction of manubrium; angling of sternum anteriorly and suturing in position. Welch modified this technique in 1958, emphasizing the preservation of perichondrial sheaths of costal cartilages and preservation of intercostal bundles. This modified Ravitch technique is the basis of current open repair performed 50 years later.^3,4

In 1998, Nuss reported his 10-year experience with a minimally invasive technique for pectus excavatum repair. This procedure involves placement of an internal stainless steel bar to reshape the chest wall without excision of costal cartilages or sternal osteotomy.¹ The Nuss minimally invasive pectus excavatum repair has become an appealing surgical option to the traditional open repair, with excellent outcomes and a low morbidity.

Pectus excavatum includes a spectrum of severity from mild to severe. Mild to moderate deformities can benefit from an exercise and posture program, and yearly follow-up. Those patients with severe deformities should undergo a complete evaluation to define the degree of deformity and physiologic impairments which will aid in determining candidacy for surgical intervention. Approximately two-thirds of patients are treated nonoperatively.^1,4,5

Although pectus deformities may be present at birth and noticeable in early childhood, it is usually not until older childhood or teenage years that children and their parents seek evaluation and treatment. During puberty, the pectus deformity often deepens and becomes more symptomatic. Patients may describe symptoms of dyspnea on exertion, shortness of breath, exercise intolerance, air hunger, decreased endurance, and pain at the sternal border. Teenagers also present with issues with body image, which can have debilitating and life-altering psychosocial effects.^1,4–6

The operative technique performed is surgeon dependent. The minimally invasive Nuss procedure has gained widespread acceptance; however, the open technique is still performed and has comparable results. Direct comparisons of outcomes of open and minimally invasive techniques have not been performed in randomized control trials, but there have been several retrospective reviews comparing the two techniques. A meta-analysis by Nasr evaluating the published data comparing the two techniques illustrated that both open and minimally invasive repairs are acceptable techniques, with no significant difference in rate of complication, outcome, postoperative pain management, and hospital length of stay.⁷

Kelly et al. published early results of the Pectus Multicenter Study in 2007, which is a prospective study among 11 centers in the United States, attempting to compare the open and Nuss procedures with respect to complications, effect of repair on cardiopulmonary function, effect on self-image and quality of life, and patient satisfaction with pain management. The study revealed that the majority of centers were performing the Nuss procedure (284 patients) compared to open repair (43 patients). However, early results from this study demonstrated equivalent median hospital length of stay of 4 days, similar complication rates, and successful pain management in both groups.⁸

Extensive workup is performed for those patients in whom operative intervention is sought. Patient selection for operative repair is determined by performing a thorough history and physical examination to evaluate the degree of deformity and impairment. Symptoms suggesting cardiorespiratory compromise, pain, or body image issues should be investigated. On physical examination, attention to the depth of depression and associated findings of number of costal cartilages involved, rounded shoulders, protuberant abdomen (due to laxity of rectus abodminis), and presence of scoliosis should be assessed.^1,5

For those patients who appear to have a moderate-to-severe pectus excavatum deformity, further investigation is warranted. The workup includes CT scan of chest, pulmonary function testing, and echocardiogram. Echocardiogram is used to evaluate right heart function, which may be compromised due to external compression of the sternal deformity. In addition, mitral valve prolapse is associated with up to 35% of patients with pectus excavatum.^3,5 Pulmonary function testing is used to assess baseline function. It may demonstrate decrease in forced vital capacity, forced expiratory volume at 1 second, and decreased oxygen delivery, indicative of restrictive airway disease.^4,5

Chest CT allows for detailed evaluation of the degree of depression of the sternum, cardiac shift, and compression, as well as providing means to calculate the Haller index. The Haller index is a measure of pectus excavatum severity by measuring the width of the chest between the ribs at the lowest level of the pectus defect in centimeters divided by the height of the chest from the anterior spine to the back of the lowest part of the sternal defect. The quotient of this division is the Haller index. An index greater than 3.25 is considered a severe defect, warranting consideration for repair.⁴

Surgical correction of pectus excavatum is indicated with the presence of two or more of the following criteria: progressive or symptomatic pectus deformity, restrictive disease on pulmonary function testing, chest CT demonstrating cardiac compression or atelectasis, Haller index >3.25, cardiac abnormalities including mitral valve prolapse, decreased diastolic filling due to right heart compression, and recurrence after failed repair (Table 140-1).⁴ Although repair of pectus excavatum has been reported from 5 to 29 years of age, the recommended timing of repair is in the middle teenage years. During this time period, the chest wall remains malleable and allows for chest wall maturation while the support bar is still in place. Those patients who have support bars removed before puberty have an increased risk of recurrence.^9,10

If a Nuss repair is planned, the preoperative evaluation should also investigate for metal allergy, especially nickel, chromium and copper, or a family history of metal allergy. The standard Lorenz pectus bar is stainless steel, which has been shown to cause allergic reactions in those individuals with metal allergy. Those with a metal allergy and those with history of eczema should have a titanium bar and stabilizer placed instead of the standard stainless steel bar. Titanium bars are shaped by manufacturers in advance of the surgery date.⁹

The open technique described by Shamberger and Welch, is depicted in Figures 140-1 to 140-8. Preoperative antibiotics are given and continued for 24 hours postoperatively. A transverse incision is made below and within the nipple lines at the level of the inframammary crease (Fig. 140-1). Skin flaps are elevated and mobilized utilizing electrocautery to the angle of Louis superiorly and xiphoid inferiorly. The pectoral muscle flaps are elevated off the sternum and costal cartilages. This plane is defined by identifying the free area just anterior to the costal cartilages at the junction with the sternum. An empty knife handle is used to develop this plane, and muscle flap is retracted anteriorly with small right angle retractor. The muscle is dissected laterally to the level of the costochondral junctions (Fig. 140-2). The perichondrium is incised anteriorly on the third through fifth ribs, and the pectus elevator is used to preserve the perichondrial sheaths (Fig. 140-3). The cartilage is divided at the sternal junction, held with an Allis clamp, elevated, and excised from the costochondral junction. The costal cartilages of the third through seventh cartilages bilaterally are resected to the costochondral junctions (Fig. 140-4).^3,4

After removing the costal cartilages, a sternal wedge osteotomy is created above the last deformed cartilage at the posterior angulation of the sternum. The wedge osteotomy is accomplished by creating two transverse sternal osteotomies through the anterior cortex with a Hall air drill (Zimmer USA, Inc., Warsaw, IN), approximately 2 to 4 mm apart, and subsequently removing the anterior cortex and cancellous bone (Fig. 140-5).^3,4