Levent ERALP, Harzem ÖZGER, KORHAN ÖZKAN, Kerem BİLSEL, Rejin KEBUDİ, İnci AYAN

Çocuklarda kemik ve yumuşak doku tutulumu gösteren Langerhans hücreli histiositozun cerrahi tedavisi

Amaç: Bu çalışmada, mono veya poliostatik tutulum ve geniş yumuşak doku tutulumuyla seyreden ve/veya patolojik kırık riski taşıyan Langerhans hücreli histiositoz lezyonları için ameliyat edilen çocuk hastalar değerlendirildi. Çalışma planı: Çalışmaya, uzun kemiklerde ve pelviste patolojik kırık riski taşıyan kemik tutulumuyla seyreden Langerhans hücreli histiositozlu 13 çocuk hasta (6 erkek, 7 kız; ort. yaş 6; dağılım 2-11) alındı. On bir hastada tek sistem (iskelet sistemi), üç hastada ise multisistem hastalık vardı. Toplam 17 kemik lezyonunun yerleşimi şöyleydi: Asetabulum (n=7), femur boynu ve subtrokanterik bölge-proksimal femur (n=5), proksimal humerus (n=2) ve distal humerus (n=2). Bir hastada çoklu kemik tutulumu vardı. Lezyonlara bağlı patolojik kırık riskini değerlendirmede Mirels’in skorlama sistemi kullanıldı. Hastalar cerrahi girişim ve bunu izleyen kemoterapiyle tedavi edildi. Tedavi sonuçlarını değerlendirmede, Langerhans Hücreli Histiositozis Çalışma Grubu’nun ölçütleri kullanıldı. Ortopedik girişim sonrasında ortalama takip süresi 67 ay (dağılım 34-172 ay) idi. Sonuçlar: Sadece bir hastada son takipler sırasında nüks saptandı. Kemik iliği tutulumu olan bu hasta uygulanan kemoterapiye iyi yanıt vererek iyileşti. Tüm hastaların üçüncü ay kontrollerindeki grafilerinde greft konsolidasyonu gözlendi. Takip dönemi boyunca hiçbir hastada kısalık veya deformite gibi kemik sekeline rastlanmadı. Çıkarımlar: Cerrahi tedavi ve kemoterapi kombinasyonu, agresif karakterli veya yüksek kırık riski taşıyan geniş yumuşak doku tutulumlu histiositik kemik lezyonlarında etkili bir tedavi olarak görünmektedir.

Anahtar Kelimeler:

Kemik hastalıkları, Langerhans hücreleri, Histiyositoz, Çocuk

Surgical treatment of Langerhans cell histiocytosis with bone and soft tissue involvement in pediatric patients

Objectives: We evaluated pediatric patients who underwent surgery for Langerhans cell histiocytosis with mono- or polyostatic involvement and an extensive soft tissue component and/or a high risk for pathologic fractures. Methods: The study included 13 children (6 boys, 7 girls; mean age 6 years; range 2 to 11 years) who were treated surgically for bone and soft tissue involvement of Langerhans cell histiocytosis with a high risk for fractures of the long bones and the pelvis. Eleven patients had only skeletal involvement while three patients had multisystem involvement. There were 17 bone lesions in the following localizations: acetabulum (n=7), femur neck and subtrochanteric region-proximal femur (n=5), and proximal (n=2) and distal (n=2) humerus. One patient had multifocal bone involvement. The risk for pathologic fractures was assessed using the scoring system of Mirels. Treatment consisted of surgery followed by chemotherapy. The results of treatment were evaluated according to the criteria by the Study Group of Langerhans Cell Histiocytosis. The mean follow-up after orthopedic surgery was 67 months (range 34 to 172 months). Results: Only one patient developed recurrence, in which case bone marrow involvement responded well to chemotherapy. Radiographically, graft consolidation was observed in all the patients in the third postoperative month. No bone sequelae such as shortness and deformity were detected throughout the follow-up period. Conclusion: Surgical treatment combined with chemotherapy is effective in the treatment of aggressive histiocytic bone lesions associated with extensive soft tissue involvement and high risk for fractures.

Keywords:

Bone Diseases, Langerhans Cells, Histiocytosis, Child,

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