Ön Göğüs Duvarı Anomalileri
Özet
Ön göğüs duvarı deformiteleri; yaş, cinsiyet ve genetik faktörlere göre değişen varyasyonların dışında kalan patolojik defektlerdir. En sık görülen anomaliler, tüm göğüs duvarı deformitelerinin yaklaşık %90'ını oluşturan pektus ekskavatum (kunduracı göğsü) ve sternumun öne doğru protrüzyonu ile karakterize pektus karinatumdur (güvercin göğsü). Bu deformitelerin patogenezi tam olarak bilinmemekle birlikte, kostal kıkırdakların kontrolsüz ve aşırı büyümesi temel etken olarak kabul edilmektedir. Kıkırdak büyümesi sternumu içeri çektiğinde pektus ekskavatum, dışarı ittiğinde ise pektus karinatum oluşur. Hastalarda efor dispnesi, çabuk yorulma ve kardiak bası gibi fizyolojik semptomların yanı sıra özgüven eksikliği ve depresyon gibi ciddi psikososyal problemler de gözlenebilir. Tedavi yaklaşımları fonksiyonel ve estetik endikasyonlara göre belirlenir. Vakum bell ve dinamik ortez sistemleri gibi konservatif metotlar hafif vakalarda ilk adım olarak tercih edilirken; ileri derece deformitelerde cerrahi müdahale gereklidir. Cerrahi tedavide geleneksel açık yöntemlerin (Ravitch) yanı sıra, günümüzde Nuss tekniği tabanlı minimal invaziv prosedürler (MIRPE ve MIRPK) düşük komplikasyon oranları ve hızlı iyileşme avantajlarıyla yaygın olarak uygulanmaktadır. Ayrıca dökümanda pektoral kas agenezisi ile karakterize Poland sendromu, embriyolojik gelişim bozukluklarından kaynaklanan sternum defektleri (sternal kleft, ektopia kordis, Cantrell pentalojisi) ile Jeune ve Jarcho-Levin gibi nadir görülen sendromlar da detaylandırılmıştır.
Anterior chest wall deformities are pathological defects that fall outside natural variations altered by age, gender, race, and genetics. The most common anomalies are pectus excavatum (funnel chest), which constitutes nearly 90% of all congenital chest wall deformities, and pectus carinatum (pigeon chest), characterized by the anterior protrusion of the sternum and sternocostal cartilages. Although the exact pathogenesis remains unknown, the uncontrolled overgrowth of costochondral cartilages is considered the primary cause; drawing the sternum backward results in pectus excavatum, while pushing it forward leads to pectus carinatum. Patients frequently present with physiological symptoms such as exertional dyspnea, fatigue, and cardiac compression, alongside significant psychosocial challenges like low self-esteem and depression. Management strategies are driven by functional and aesthetic indications. Conservative methods, including the vacuum bell and dynamic compression brace systems, serve as first-line therapies for mild cases, whereas severe deformities require surgical correction. Beyond traditional open osteotomy techniques (Ravitch), minimally invasive repair procedures (MIRPE and MIRPK) based on the Nuss design are widely preferred today due to their low complication rates and faster recovery. Furthermore, the text details Poland syndrome characterized by pectoral muscle agenesis, embryological sternal defects (sternal cleft, ectopia cordis, pentalogy of Cantrell), and rare congenital disorders such as Jeune and Jarcho-Levin syndromes.
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