What is pectus excavatum and how is it treated?

Pectus Excavatum: Definition and Treatment

Pectus excavatum is a congenital chest wall deformity where the sternum caves inward creating a depression in the chest, and surgical correction is recommended for symptomatic patients with severe deformity (Haller index >3.2) using either the modified Ravitch or minimally invasive Nuss repair techniques. 1, 2

What is Pectus Excavatum?

Pectus excavatum is the most common congenital chest wall deformity, occurring in approximately 1 in 300-1,000 live births, with males affected 5 times more frequently than females. 2, 3

Clinical Presentation

The deformity typically:

• Is recognized in infancy but becomes more severe during adolescent growth years 2
• Remains constant throughout adult life once skeletal maturity is reached 2
• Can range from mild cosmetic concerns to life-limiting cardiopulmonary symptoms 1

Associated Symptoms and Complications

Symptoms are infrequent during early childhood but become increasingly severe during adolescence, including: 2

• Easy fatigability and dyspnea with mild exertion
• Decreased endurance
• Anterior chest pain
• Tachycardia

Cardiopulmonary alterations include: 4, 2

• Heart deviation into the left chest causing reduced stroke volume and cardiac output 2
• Restrictive pulmonary defect from confined lung expansion 2
• Potential association with mitral valve prolapse (MVP), especially in connective tissue syndromes 4

Associated Conditions

Pectus excavatum can occur sporadically or as part of genetic syndromes, including: 5

• Marfan syndrome
• Noonan syndrome (with associated cardiac defects like pulmonary stenosis) 4
• Osteogenesis imperfecta (with more severe restrictive respiratory patterns) 4
• Familial forms without syndromic features 5

Diagnostic Evaluation

Imaging Assessment

The Haller index (severity index) is the standard measurement for determining surgical candidacy, calculated from chest CT or X-ray, with values >3.2 indicating moderate to severe deformity requiring surgical correction. 2, 3

Imaging modalities include: 4

• CT with IV contrast for detailed anatomic assessment and surgical planning 4
• MRI to facilitate surgical chest wall reconstruction planning 4

Cardiac Evaluation

Complete cardiac evaluation is mandatory when genetic syndromes are suspected, particularly in patients with connective tissue disorders. 4 This should include:

• Echocardiogram to assess for MVP and cardiac displacement 4, 6
• Evaluation for aortic root dilatation in suspected Marfan syndrome 5

Pulmonary Assessment

Preoperative spirometry should be performed to document restrictive defects and establish baseline function. 6

Treatment Approach

Indications for Surgical Repair

Repair is recommended for patients who are symptomatic AND have a markedly elevated Haller index (>3.2) as determined by chest imaging. 2

Timing of Surgery

The optimal age for repair is between 12 and 16 years, though repair can be performed after age 8 years or in adults with similar good results. 2

Surgical Techniques

Modified Ravitch Technique

The modified Ravitch technique removes minimal cartilage and routinely uses a temporary internal support bar for 6 months, with the following characteristics: 2

• Operation rarely exceeds 3 hours 2
• Hospitalization rarely exceeds 3 days 2
• Mild pain profile 2
• 97% of patients experience good to excellent results 2
• Low complication rate of 6.7% 7
• Recurrence rate of 1.5% major and 4.5% mild 7

Minimally Invasive Repair (Nuss/MIRPE)

The Nuss procedure (MIRPE) avoids cartilage resection and has become standard of care for pediatric and adolescent patients, though adoption in adults has been slower due to increased chest wall rigidity. 1, 2

Key characteristics include: 2

• Less operating time than Ravitch 2
• More severe postoperative pain 2
• Longer hospitalization 2
• Higher complication rate 2
• Bar remains in place for 2 or more years 2
• Less applicable to older patients and asymmetric deformities 2

Advanced Techniques

For severe pectus excavatum, modified taulinoplasty with double Pectus Up bars offers improved correction and increased stability, though with increased technical complexity. 6

Adult Considerations

Adult patients can undergo highly successful MIRPE repair with advanced preoperative evaluations and technique modifications, resulting in symptom resolution despite increased chest wall calcification and rigidity. 1

Postoperative Complications

Potential complications include: 4

• Atelectasis
• Pleural effusion
• Cardiac migration considerations in patients with kyphoscoliosis 4

Clinical Pitfalls

Important caveats to avoid: 5

• Many features of pectus excavatum occur sporadically in the general population without syndromic associations 5
• Not all patients with pectus excavatum require genetic syndrome workup unless other phenotypic features are present 5
• Familial pectus excavatum can occur without other syndromic features and represents polygenic inheritance 5