Hüseyin ARSLAN, Serdar NECMİOĞLU, Hüseyin ÇOBAN

Poland sendromu

Poland sendromu herediter olmayan ve nedeni bilinmeyen konjenital bir anomalidir. Klinik görünümü değişkendir. Bu sendromda pektoralis majör kas yokluğu, aynı tarafta sıklıkla brakisindaktili ve üst ekstremite hipoplazi vardır. Bu çalışmada pektoralis majör kas yokluğuyla birlikte kol, önkol ve elde hipoplazi, brakisindaktili, orta falankslarda hipoplazi ve/veya aplazi bulunan iki olgu sunuldu. Her iki olguda kozmetik görünümde bozukluk ve el fonksiyonlarında azalma vardı. Olgulardan birine sindaktili açılması için cerrahi tedavi uygulandı. Cerrahi tedavi sonrası kozmetik görümde kısmen düzelme ve el fonksiyonel kapasitesinde artma oldu. İkinci olgu önerilen ameliyatı kabul etmedi.

Anahtar Kelimeler:

Sindaktili, Poland sendromu, Kol, Cerrahi işlemler, operatif, Pektoralis kasları

Poland's syndrome

Poland's syndrome is a congenital anomaly that is not hereditary and is of unknown origin. Clinical features are variable and includes the absence of the pectoralis major and minor, upper extremity abnormalities, and often synbrachydactyly. The arm and more frequently the forearm are hypoplastic. We report two cases presenting with the absence of the pectoralis major and minor, hypoplasia of the upper extremity, and synbrachydactyly. Poor cosmetic appearance and low functional capacity, particularly in hand functions were the main complaints. One patient underwent surgery for the release of syndactyly, which resulted in improved functional capacity and cosmetic appearance. The latter patient refused surgery.

Keywords:

Syndactyly, Poland Syndrome, Arm, Surgical Procedures, Operative, Pectoralis Muscles,

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