Pediatric Surgery NaT
Chest Wall Deformities
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Introduction
Chest wall deformities represent abnormalities of the thoracic cavity and can be classified as primary or secondary. Primary pathologies represent abnormal growth of the thoracic cavity from gestation through adolescence. Secondary pathologies have multiple etiologies such as tumor burden, trauma, metabolic disorders, and postoperative sequelae [1]. Operative intervention is case-specific and management guidelines often revolve around the initial inciting event. Discussion on these topics can be found elsewhere (Thoracic Trauma and Chest Wall Tumors).
In this particular chapter, we will focus on primary pathologies of which there are five main categories:
- Pectus Excavatum
- Pectus Carinatum
- Poland Syndrome
- Sternal Cleft
- Others (including Jeune Syndrome and Jarcho-Levin Syndrome)
The symptoms associated with congenital chest wall deformities are varied and depend largely upon the etiology of the deformity. For the most common deformity, pectus excavatum, symptoms range from cosmetic disturbances to dyspnea and cardiac arrhythmias. There is little to no associated mortality from this disease [2]. Conversely, for sternal clefts with ectopia cordis mortality rates can be extremely high [3].
Content in these topics is referenced in SCORE Chest Wall Deformities overview
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Introduction
Chest wall deformities represent abnormalities of the thoracic cavity and can be classified as primary or secondary. Primary pathologies represent abnormal growth of the thoracic cavity from gestation through adolescence. Secondary pathologies have multiple etiologies such as tumor burden, trauma, metabolic disorders, and postoperative sequelae [1]. Operative intervention is case-specific and management guidelines often revolve around the initial inciting event. Discussion on these topics can be found elsewhere (Thoracic Trauma and Chest Wall Tumors).
In this particular chapter, we will focus on primary pathologies of which there are five main categories:
- Pectus Excavatum
- Pectus Carinatum
- Poland Syndrome
- Sternal Cleft
- Others (including Jeune Syndrome and Jarcho-Levin Syndrome)
The symptoms associated with congenital chest wall deformities are varied and depend largely upon the etiology of the deformity. For the most common deformity, pectus excavatum, symptoms range from cosmetic disturbances to dyspnea and cardiac arrhythmias. There is little to no associated mortality from this disease [2]. Conversely, for sternal clefts with ectopia cordis mortality rates can be extremely high [3].
Content in these topics is referenced in SCORE Chest Wall Deformities overview
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