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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

Kaynakça

1. Achterman C, Kalamchi A. Congenital deficiency of the fibula. J Bone Joint Surg Br 1979;61–B:133–7.
2. Brown FW. The Brown operation for total hemimelia tibia in Skeletal lower-limb anomalies: surgical and prosthetic management. A. GT, Editor. Washington, DC: National Academy of Science 1971. p. 21–8.
3. Seyhan F, Ahiskali G. Congenital skeletal limb deficien- cies. Examples and their treatment. [Article in Turkish] Turk Tip Cemiy Mecm 1972;38:481–8.
4. Kalamchi A, Dawe RV. Congenital deficiency of the tibia. J Bone Joint Surg Br 1985;67:581–4.
5. Hosny GA. Treatment of tibial hemimelia without amputation: preliminary report. J Pediatr Orthop B 2005;14:250–5.
6. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula. Classification and management. J Bone Joint Surg Br 1978;60:31–9.
7. Weber M. New classification and score for tibial hemime- lia. J Child Orthop 2008;2:169–75.
8. Eamsobhana P, Kaewpornsawan K. Limb salvage in tibial hemimelia. J Med Assoc Thai 2012;95 Suppl 9:62–9.

9. Jayakumar SS, Eilert RE. Fibular transfer for congenital absence of the tibia. Clin Orthop Relat Res 1979;139:97– 101.
10. Loder RT, Herring JA. Fibular transfer for congenital absence of the tibia: a reassessment. J Pediatr Orthop 1987;7:8–13.
11. Schoenecker PL, Capelli AM, Millar EA, Sheen MR, Ha- her T, Aiona MD, et al. Congenital longitudinal deficiency of the tibia. J Bone Joint Surg Am 1989;71:278–87.
12. Wehbé MA, Weinstein SL, Ponseti IV. Tibial agenesis. J Pediatr Orthop 1981;1:395–9.
13. Clark MW. Autosomal dominant inheritance of tibial meromelia. Report of a kindred. J Bone Joint Surg Am 1975;57:262–4.
14. Epps CH Jr, Schneider PL. Treatment of hemimelias of the lower extremity. Long-term results. J Bone Joint Surg Am 1989;71:273–7.
15. Valdiserri L, Donzelli O, Saccozzi P. Congenital aplasia of the tibia. Ital J Orthop Traumatol 1984;10:477–88.
16. Coleman SS, Coleman DA. Congenital pseudarthrosis of the tibia: treatment by transfer of the ipsilateral fibula with vascular pedicle. J Pediatr Orthop 1994;14:156–60.
17. Epps CH Jr, Tooms RE, Edholm CD, Kruger LM, Bryant DD 3rd. Failure of centralization of the fibula for congeni- tal longitudinal deficiency of the tibia. J Bone Joint Surg Am 1991;73:858–67.
18. Fernandez-Palazzi F, Bendahan J, Rivas S. Congenital de- ficiency of the tibia: a report on 22 cases. J Pediatr Orthop B 1998;7:298–302.
19. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res 1990;250:81–104.
20. Turner-Bowker DM, Kosinski M, Zhao J, Saris-Baglama RN. SF-10 for Children™ A User’s Guide Lincoln Quali- tyMetric Inc, 2003.
21. Zhang L, Fos PJ, Johnson WD, Kamali V, Cox RG, Zuni- ga MA, et al. Body mass index and health related quality of life in elementary school children: a pilot study. Health Qual Life Outcomes 2008;6:77.
22. Fujii H, Doi K, Baliarsing AS. Transtibial amputation with plantar flap for congenital deficiency of the tibia. Clin Orthop Relat Res 2002;403:186–90.
23. Simmons ED Jr, Ginsburg GM, Hall JE. Brown’s proce- dure for congenital absence of the tibia revisited. J Pediatr Orthop 1996;16:85,9.
24. Wada A, Fujii T, Takamura K, Yanagida H, Urano N, Yamaguchi T. Limb salvage treatment for congenital defi- ciency of the tibia. J Pediatr Orthop 2006;26:226–32.