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.

Appearance of patients with partial expansion of the chest cage using non-surgical vacuum treatment for thoracic dystrophy. An image demonstrating how the vacuum is applied to these patients. You can observe how the patient experienced a growth spurt in their growth curves with the treatment.

Appearance of patients with partial expansion of the chest cage using non-surgical vacuum treatment for thoracic dystrophy. An image demonstrating how the vacuum is applied to these patients. You can observe how the patient experienced a growth spurt in their growth curves with the treatment.

After applying lateral thoracic expansion on the right side of our 8-year-old patient diagnosed with "Jeune Syndrome," you can observe the development and changes on the right side. By comparing the X-rays of the right lung with the left, you can easily see how the right lung expanded. In these patients, the same surgery is repeated on the opposite (left) side 6 months after the first surgery, ensuring definite expansion of the chest cage.

After applying lateral thoracic expansion on the right side of our 8-year-old patient diagnosed with "Jeune Syndrome," you can observe the development and changes on the right side. By comparing the X-rays of the right lung with the left, you can easily see how the right lung expanded. In these patients, the same surgery is repeated on the opposite (left) side 6 months after the first surgery, ensuring definite expansion of the chest cage.

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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Thoracic Dystrophies

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