Mahmut KÖMÜRCÜ, Serdar YÜCE, Osman Yüksel YAVUZ, İsmail URAŞ, Murat UYGUN, Mustafa KÜRKLÜ

Our Experiences in Surgical Treatment of Bayne Type III and IV Radial Longitudinal Deficiencies

Radial uzunluk eksiklikleri elbileğinin radial eğriliği, kısa ve eğri önkol ve eksik veya fonksiyon görmeyen başparmak ile karakterizedir. Deformite kemikler, eklemler, kaslar, tendonlar, ligamentler sinirler ve damarların hipoplazisini içeren malformasyon ve az gelişmenin çeşitli derecelerine neden olur. Bu çalışma, Bayne tip 3 ve 4 radial uzunluk eksiklikliği olan 8 ekstremitedeki santralizasyon sonuçlarımızı tanımlamıştır. 2002 ve 2010 arasında santralizasyon yapılan 6 hastanın 8 ekstremitesi gözden geçirildi. Operasyon zamanında ortalama hasta yaşı 2.75 yıldı (2-14 yıl arası). Cinsiyet oranı 4 erkek, 2 kadın idi. Bütün hastalara centralizasyon yapıldı. Bir hastaya ek olarak ulna kemiğine sirkuler eksternal fiksatör (CEF) vasıtasıyla 6,5 cm uzama sağlandı. Santralizasyon için ortalama takip süresi 4,5 yıldı (2-10 yıl arası). Sonuç 4 olguda mükemmel, 3 olguda iyi, 1 olguda orta kabul edildi. Radial eğrilik tüm olgularda düzeldi. Takip sırasında, bir hastada 15 derece radial eğrilik gelişti; bu eğriliğe cerrahi düzeltme gerekmedi. El ve elbileği hareketleri kabul edilebilir düzeyler içindeydi. Elbileği ve önkol hareket ve fonksiyonu elbileğinin santralizasyonu ve gerektiğinde ulnar uzatma yoluyla elde edilebilir. Kozmetik olarak hoş görünüm sağlanabilir

Anahtar Kelimeler:

Radial uzunluk eksikliği, santralizasyon, uzatma

Our Experiences in Surgical Treatment of Bayne Type III and IV Radial Longitudinal Deficiencies

Radial longitudinal deficiencies are characterized by radial deviation of the wrist, a short and bowed forearm and a non-functional or absent thumb. The deformity is caused by varying degrees of underdevelopment and malformation including hypoplasia of the bones, joints, muscles, tendons, ligaments, nerves and vessels. This study describes our results from centralization in 8 limbs with Bayne type III-IV radial longitudinal deficiencies. We reviewed 8 limbs from 6 patients with a centralization performed between 2002 and 2010. The median patient age at the time of the operation was 2.75 years (range 2-14 years). The sex ratio was 4 male and 2 female. All patients underwent centralization. One patient had an additional 6.5 cm elongation of the ulna bone by means of a circular external fixator (CEF). The median follow-up time from centralization was 4,5 years (range 2-10 years). The result was considered to be excellent in 4 cases, good in 3 cases and fair in 1 case. Radial deviations regenerated in all cases. During follow-up, a radial deviation of 15 degrees developed in one patient; this deviation did not require surgical correction. Hand and wrist movements were within acceptable levels. Movement and function of the wrist and forearm can be obtained through centralization of the wrist and ulnar elongation when necessary. A cosmetically pleasant appearance can be ensured.

Keywords:

Radial longitudinal deficiency, centralization, distraction,

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de Jong JP, Moran SL, Vilkki SK. Changing paradigms in the treatment of radial club hand: microvascular joint trans- fer for correction of radial deviation and preservation of long-term growth. Clin Orthop Surg 2012;4(1):36-44.
Salati SA, Rabah SM. Congenital isolated right radial club hand. Libyan J Med 2010;1:5.
Bayne LG, Klug MS. Long-term review of the surgi- cal treatment of radial deficiencies. J Hand Surg Am 1987;12(2):169-79.
Bhat SB, Kamath AF, Sehgal K, Horn BD, Hosalkar HS. Multi-axial correction system in the treatment of radial club hand. J Child Orthop 2009;3(6):493-8.
Shariatzadeh H, Jafari D, Taheri H, Mazhar FN. Recurrence rate after radial club hand surgery in long term follow up. J Res Med Sci 2009;14(3):179-86.
Maschke SD, Seitz W, Lawton J. Radial longitudinal defi- ciency. J Am Acad Orthop Surg 2007;15(1):41-52.
Sabharwal S, Finuoli AL, Ghobadi F. Pre-centralization soft tissue distraction for Bayne type IV congenital radial de- ficiency in children. J Pediatr Orthop 2005;25(3):377-81.
Lamb DW. The treatment of longitudinal radial deficien- cy. Prosthet Orthot Int 1991;15(2):100-3.
Elbaum R, Royer M, Godart S. Radial club hand and Holt- Oram syndrome. Acta Chir Belg 1995;95(5):229-36.
James MA, McCarroll HR Jr, Manske PR. The spectrum of radial longitudinal deficiency: a modified classification. J Hand Surg Am 1999;24(6):1145-55.
Urban MA, Osterman AL. Management of radial dysplasia. Hand Clin 1990;6(4):589-605.
Kanojia RK, Sharma N, Kapoor SK. Preliminary soft tissue distraction using external fixator in radial club hand. J Hand Surg Eur Vol 2008;33(5):622-7.
Nanchahal J, Tonkin MA. Pre-operative distraction length- ening for radial longitudinal deficiency. J Hand Surg Br 1996;21(1):103-7.
Smith AA, Greene TL. Preliminary soft tissue distrac- tion in congenital forearm deficiency. J Hand Surg Am 1995;20(3):420-4.
Vilkki SK. Distraction and microvascular epiphysis transfer for radial club hand. J Hand Surg Br 1998;23(4):445-52.
Vilkki SK. Vascularized metatarsophalangeal joint trans- fer for radial hypoplasia. Semin Plast Surg 2008;22(3):195- 212.
Evans DM, Gateley DR, Lewis JS. The use of a bilobed flap in the correction of radial club hand. J Hand Surg Br 1995;20(3):333-7.
Matsuno T, Ishida O, Sunagawa T, Suzuki O, Ikuta Y, Ochi M. Radius lengthening for the treatment of Bayne and Klug type II and type III radial longitudinal deficiency. J Hand Surg Am 2006;31(5):822-9.
Kawabata H, Shibata T, Masatomi T, Yasui N. Residual deformity in congenital radial club hands after previ- ous centralisation of the wrist. Ulnar lengthening and correction by the Ilizarov method. J Bone Joint Surg Br 1998;80(5):762-5.
Pickford MA, Scheker LR. Distraction lengthening of the ulna in radial club hand using the Ilizarov technique. J Hand Surg 1998;23(2):186-91.