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Öz

Amaç: Tibiyal hemimeli, tibia eksikliğinden veya gelişmemesinden kaynaklanan sert ve nadir bir hastalıktır. Bu çalışmanın amacı, diz-bilek eklemlerinin merkezileştirilmesi ve Ilizarov ilkeleri ile uzatma işlemi de dahil olmak üzere rekonstrüksif ameliyatların etkinliğini geriye dönük olarak değerlendirmekti. Ampütasyon ve rekonstrüksiyonun fiziksel ve işlevsel sonuçları da değerlendirildi.Çalışma planı: Bu, tibiyal hemimeli tanısı koyulan ve 1998-2011 yılları arasında tek bir kuruluşta ameliyata gerek duyulan bütün hastalar üzerinde gerçekleştirilen IRB onaylı geriye dönük incelemedir. Klinik ve radyografik bulgular için grafikler analiz edilmiştir. Dahil edilen bütün hastalar, ek bir fiziksel ve radyografik inceleme amacıyla son takip ziyareti için çağırılmıştır. Ebeveynler ve hastalardan SF-10™ sağlık araştırma formunu doldurmaları istenmiştir.Bulgular: Hastalıktan etkilenen toplam 30 uzuv olmak üzere 21 hasta (12 erkek, 9 kadın) çalışmaya dahil edilmiştir. İlk ameliyatta ortalama yaş 4.8’di (±3.1). 4 hastanın 6 uzvunda diz seviyesinde disartikülasyon yapıldı. Tip III hastalığı olan bir hastaya transtibiyal ampütasyon yapıldı. Her hasta için ortalama ameliyat sayısı 6.4’tü (±3.3) ve dış sabitleyici ve alçının ortalama durma süresi 17 (±6) aydı. Ortalama uzatma 4.9 (±1.3) cm’di ve ortalama uzuv uzunluğu farkı 5.8 (±3.7) yıllık takipte 3.1 (±1.7) cm’di. Eklem yerinden ayırma ve rekonstrüksiyon yapılan hastalarda SF-10™ puanları benzerdi (p=0.63). Ancak, eklem yerinden ayırma işlemi diz dengesizliği bulunan durumlarda uygulandığında, bütün puanlar anlamlı derecede daha yüksekti (p<0.01).Çıkarımlar: Tedavi yöntemi, diz eklemi dengeliyken, proksimal tibianın bulunup bulunmamasına göre seçilmelidir. Diz eklemi dengesizliği varsa ampütasyon tercih edilmelidir

Anahtar Kelimeler

• Amputasyon; eksternal fiksasyon; diz ortalanması; rekonstürkiyon; SF-10™; tibiyal hemimeli

Preliminary report on amputation versus reconstruction in treatment of tibial hemimelia

Abstract

Objective: Tibial hemimelia is a rare disorder characterized by the absence or hypoplasia of the tibia with associated rigidity. The aim of this study was to retrospectively evaluate the affectivity of reconstructive surgeries including centralization of the knee-ankle joints and lengthening with Ilizarov principles, as well as physical and functional results of amputation and reconstruction.
Methods: This is an IRB-approved retrospective review of all patients diagnosed with tibial hemimelia who required surgery at a single institution between 1998 and 2011. Charts were analyzed for clinical and radiographical findings. At final follow-up, patients underwent physical and radiographic examination. Patients and their parents were asked to complete the SF-10™ health survey (QualityMetric Inc., Lincoln, RI, USA).
Results: Twenty-one patients (12 male, 9 female) with 30 affected extremities were included. Mean age was 4.8±3.1 years at initial surgery. Knee level disarticulation was performed in 6 extremities of 4 patients. One patient with type III underwent transtibial amputation. Mean number of surgeries for each patient was 6.4±3.3, and mean duration of external fixator and casting was 17±6 months. Mean lengthening was 4.9±1.3 cm, and mean limb length discrepancy was 3.1±1.7 cm at 5.8±3.7 years at follow-up. SF-10™ scores were similar in disarticulated and reconstructed patients (p=0.63). All scores were significantly higher when disarticulation was performed in cases of knee instability (p<0.01).
Conclusion: When stability of the knee joint is present, treatment modality should be chosen according to the existence of the proximal tibia. Amputation should be preferred in cases of knee joint instability.

Keywords

• Amputation
• external fixation
• knee centralization
• reconstruction
• SF-10™
• tibial hemimelia

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