Current Management of Pectus Excavatum: A Review and Update of Therapy and Treatment Recommendations

The Journal of the American Board of Family Medicine

Dawn Jaroszewski³  Claude Deschamps¹  D. Eric Steidley⁴  David Notrica²  Lisa McMahon² 

[1]Division of Thoracic Surgery, Mayo Clinic Rochester, Minnesota (CD)

[2]Pediatric Surgeons of Phoenix, Department of Pediatric Surgery, Phoenix Children`s Hospital (DN, LM)

[3]Division of Cardiothoracic Surgery (DJ), Mayo Clinic Arizona, Scottsdale

[4]Division of Cardiology (DES), Mayo Clinic Arizona, Scottsdale

关键词: Pectus Excavatum;    Chest Wall Deformities;    Congenital Defects;    Cardiovascular Disorders;    Musculoskeletal and Connective Tissue;    Review;   

DOI  :  10.3122/jabfm.2010.02.090234

学科分类：过敏症与临床免疫学

来源: The American Board of Family Medicine

【 摘 要 】

Pectus excavatum (PE) is a posterior depression of the sternum and adjacent costal cartilages and is frequently seen by primary care providers. PE accounts for >90% of congenital chest wall deformities. Patients with PE are often dismissed by physicians as having an inconsequential problem; however, it can be more than a cosmetic deformity. Severe cases can cause cardiopulmonary impairment and physiologic limitations. Evidence continues to present that these physiologic impairments may worsen as the patient ages. Data reports improved cardiopulmonary function after repair and marked improvement in psychosocial function. More recent consensus by both the pediatric and thoracic surgical communities validates surgical repair of the significant PE and contradicts arguments that repair is primarily cosmetic. We performed a review of the current literature and treatment recommendations for patients with PE deformities.

• Pectus Excavatum
• Chest Wall Deformities
• Congenital Defects
• Cardiovascular Disorders
• Musculoskeletal and Connective Tissue
• Review

Pectus chest deformities are among the most common major congenital anomalies found in patients in the United States. They occur in approximately 1 of every 300 to 400 white male births.^1–3 Men are afflicted 5 times more often than women.^2,4 The condition is uncommon among African Americans and Latinos.^2,5 Pectus excavatum (PE) with sternal depression is approximately 6 times more common than pectus carinatum (protrusion).^4,5 The cause of PE remains unknown. Approximately 40% of patients with pectus deformities have family members who also have deformities, although a direct genetic link has not been identified.^4,6–8 Disturbances in the growth of the sternum and costal arches, as well as biomechanical factors, are suspected in the pathogenesis.^{1,2,4,6,9–11} The involved cartilages can be fused, deformed, or rotated. Intrinsic abnormality of the costochondral cartilage is suggested by the significant occurrence of PE among patients with connective tissue disorders, such as Marfan syndrome.^4,6,8,12 There is also a high percentage of scoliosis associated with the disorder.^6–8,12 Abnormalities of the cartilages removed during surgery have also been reported, including stress/strain deformation and abnormal collagen content.¹

PE can be present at birth but is most frequently recognized during early childhood. During rapid adolescent growth, many patients experience a marked increase in the severity of the depression until full skeletal maturity is achieved.^5,13–20 In severe cases or those in which depressions directly impinge on the right ventricle, PE is more than a cosmetic deformity.^21–24 Many patients do not undergo repair during childhood and subsequently experience progressive a worsening of symptoms and cardiopulmonary function with increasing age.^{18–20,24–26}

One theory for the worsening of symptoms that is experienced with age is that the chest wall is very compliant in infancy but, with aging, a steady decline in compliance is experienced and it becomes relatively stiffer compared with the lungs. In adults the loss of elasticity and flexibility of the chest can lead to cardiac symptoms with only moderate exertion.^8,26 A similar phenomena is also seen in patients with scoliosis as they age.^6,7 Publications regarding the surgical management of adult patients with pectus deformities are few, but have increased significantly during the past few years with greater recognition of the physiologic and psychologic impact of the disease.^{8,18,19,24,26–36} Despite well-documented reports to the contrary, the longstanding misconception persists that PE is a cosmetic defect with no physiologic consequences. This misconception has resulted in patients reaching adulthood with uncorrected defects. Some patients will develop cardiopulmonary symptoms for the first time as they age and others will experience a worsening of symptoms they have endured for years.

【 授权许可】

Unknown   

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