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Key Points

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  1. The main indications for medical intervention for children with pectus carinatum are severity of symptoms and the degree to which the deformity interferes with activities. This is a subjective decision-making process that requires the input of the surgeon, parent, and adolescent.

  2. Surgical repair and bracing can be effective treatments of pectus carinatum.

  3. Newer approaches include minimizing the extent of cartilage resection and thoracoscopic and subpectoral cartilage resection using minimally invasive techniques.

  4. Surgical intervention on the chest is indicated for patients with Poland syndrome and functional impairment, mainly those with aplastic ribs. In less severe cases, surgical repair to the chest is mainly for cosmesis.

  5. Several surgical approaches have been used to address the thoracic insufficiency associated with thoracic deformity syndromes, but the outcomes are generally poor. More promising are recent reports of procedures using vertical expandable prosthetic titanium ribs.

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Pectus Carinatum

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Introduction

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Pectus carinatum is a chest wall deformity caused by an overgrowth of the costal cartilages. This causes anterior protrusion of the sternum with narrowing of the sides of the chest wall, resulting in a bowed appearance often referred to as “pigeon chest.” This deformity can occur in patients with connective tissue disorders, in association with scoliosis, in families, or sporadically in patients without any other abnormalities.

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Pectus carinatum occurs in 1 of 300 births. It is more common in males by a 4:1 ratio. It is often apparent at birth, but becomes more noticeable and more severe during periods of rapid growth, such as puberty.

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Etiology

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The varied patient population affected by pectus carinatum has led to different theories about its etiology. Most of these theories focus on an abnormality in the cartilage. Damage to the growth plates in the costochondral junction, leading to the overgrowth of the costal cartilages, is thought to be the cause among the sporadic patient population. Efforts to identify a cause in genetically linked patients have led to more intensive study of collagen synthesis and structure. Studies of skin and cartilage collagen have focused on the ratios of collagen types and the stability of collagen I and II. The consistent finding of collagen with unstabilized end terminals suggests a specific mutation, but the exact mutation has not been determined.

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Associated Defects and Syndromes

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Approximately 20% of patients have associated anomalies. These include numerous conditions such as cardiac defects, microcapnia, bilateral clubfeet, hemifacial microsomia, microphthalmia, tracheoesophageal fistulae, congenital laryngeal stridor, a dolichocephalic skull, scoliosis, and other musculoskeletal anomalies. The family history is positive for a chest wall deformity in approximately 25% of cases.

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The incidence of pectus carinatum is higher in patients with Poland syndrome and Marfan syndrome. Any suspicion of Marfan stature should prompt a genetic work-up and evaluation of the aortic root. The Currarino-Silverman syndrome is associated with an abnormally short, U-shaped, ...

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