Bülent EROL, Cihangir TETİK, Evrim ŞİRİN, Barış KOCAOĞLU, Murat BEZER

Multipl enkondromatoziste görülen el deformitelerinin cerrahi tedavisi: Olgu sunumu

Multipl enkondromatozis (Ollier hastalığı), yaygın enkondromlarla karakterize nadir bir hastalıktır. Sıklıkla ellerdeki kısa tübüler kemikler tutulur ve genellikle lezyonlar ilerleyerek parmaklarda kozmetik sorunlara ve fonksiyonel deformiteye yol açar. El parmaklarında yerleşen yaygın enkondromların tedavisinde diyafizektomi ve yapısal otogreft veya allogreftlerle rekonstrüksiyon önerilmektedir. Küretaj ve greftleme veya sıra (ray) amputasyon, tutulumun şiddetine göre uygulanan diğer cerrahi işlemlerdir. Enkondromatozli 25 yaşında kadın hasta, her iki el parmaklarında şiddetli şişlik ve deformite yakınmalarıyla kliniğimize başvurdu. Parmaklardaki lezyonların çoğuna diyafizektomi ve yapısal greftlerle rekonstrüksiyon, bazılarına da sıra amputasyon, küretaj ve greftleme uygulandı. Uzun dönem takipte (sol el 6 yıl, sağ el 5.5 yıl) her iki elde kabul edilebilir fonksiyonel ve kozmetik sonuç elde edildi.

Anahtar Kelimeler:

Enkondromatoz, Osteokondrodisplazi, El parmağı eklemi, El deformiteleri

Surgical treatment of hand deformities in multiple enchondromatosis: A case report

Multiple enchondromatosis (Ollier’s disease) is a rare disease characterized by widespread enchondromas. In general, the short tubular bones of the hand are involved, with progressive lesions resulting in cosmetic problems and functional deformities. Diaphysectomy and reconstruction with structural autografts or allografts are usually recommended in the treatment of extensive enchondromas involving the fingers. Curettage and grafting and ray amputation are other surgical procedures that can be applied depending on the severity of involvement. A 25-year-old woman with enchondromatosis presented with severe swelling and deformities on her fingers in both hands. The majority of the lesions were managed by diaphysectomy and reconstruction with structural grafts; ray amputation, curettage and grafting were performed for more severe lesions. During a long-term follow-up (left hand 6 years, right hand 5.5 years) cosmetic and functional results were acceptable.

Keywords:

Enchondromatosis, Osteochondrodysplasias, Finger Joint, Hand Deformities,

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