Abstract
Congenital chest wall or pectus deformities including pectus excavatum (funnel chest) and pectus carinatum (pigeon chest) affect a significant proportion of the general population and up to 70% of patients with Marfan syndrome. Patients often experience significant morbidity and psychological distress, which can worsen with age. Here we discuss new techniques for both operative and non-operative treatment of pectus deformity, the importance of a welltimed intervention and special considerations in patients with Marfan syndrome.
Highlights
Congenital chest wall or pectus deformities including pectus excavatum and pectus carinatum affect a significant proportion of the general population and up to 70% of patients with Marfan syndrome
L use Introduction ia Pectus deformities including pectus c excavatum and pectus carinar tum constitute the most come mon congenital anterior chest wall deformities. They affect a significant proportion of m the general population (0.8%) and an even greater proportion of patients with Marfan m syndrome.[1]
Whilst in Marfan syndrome, pectus can Symptoms commonly described by recurrent pneumothoraces, bullae, obstrucbe considered as integral part of the domi- patients those with pectus exca- tive sleep apnoea, fibrosis and emphysenantly inherited deficiency of Fibrillin-1, in vatum include breathlessness, chest pain on ma.[12,13] the general population, it is largely consid- exertion, palpitations, an inability to perered a primary hereditary connective tissue form physical activities at the same level of disorder.[2]
Summary
The common clinical features include abnormal development of the rib cage. Abnormal growth and elongation of the hyaline cartilaginous connection between. L use Introduction ia Pectus deformities including pectus c excavatum (funnel chest) and pectus carinar tum (pigeon chest) constitute the most come mon congenital anterior chest wall deformities They affect a significant proportion of m the general population (0.8%) and an even greater proportion of patients with Marfan m syndrome (up to 70%).[1] They are associated o with a significant degree of morbidity and c psychological distress; recent advances have highlighted several safe and n well tolerated treatment options. A number of authors have suggested abnormal collagen content as the root cause, and histological studies have demonstrated an immature collagen matrix in costal cartilage of affected children.[4] This abnormal growth results in costal cartilages, which can be elongated, rotated, deformed and fused.
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
