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thoracic surgery pectus excavatum

Thoracic Dystrophies

Thoracic dystrophies are a condition of shrinking in the chest cage. Thoracic dystrophies can affect multiple organs. It can be acquired after a previous chest cage surgery or can be congenital. The congenital form is genetically inherited.


Thoracic dystrophies can lead to inadequate development of lung and chest organs causing respiratory distress in the form of "Asphyxiating Thoracic Dystrophy," or it can manifest as "Fibrodysplasia Ossificans," causing issues in toe and chest wall bones. The form often seen as "Asphyxiating Thoracic Dystrophy" has 22 different types. The most commonly known is "Jeune Syndrome." Other well-known ones are Saldino-Noonan Syndrome, Majewski Syndrome, Mainzer-Saldino Syndrome, Beemer-Langer Syndrome, and Ellis-van Creveld Syndrome. The common findings in all phenotypes are inadequate development of the chest cage (thoracic hypoplasia) and shortened ribs, which can lead to various deformities in the chest wall.

Due to inadequate development of the chest cage, the lungs, heart, and other chest organs cannot grow properly. In growing children, the heart and lungs that fail to grow might not meet the body's needs and can become life-threatening. These children are also prone to frequent lung infections, and some of them may be lost due to these reasons. Therefore, applying definitive treatment to children over the age of 1 is crucial.

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