X’E BAĞLI HİPOFOSFATEMİK RAŞİTİZMDE ALT EKSTREMİTE DEFORMİTELERİNE ORTOPEDİK YAKLAŞIM

X’e bağlı hipofosfatemik raşitizmde özellikle alt ekstremite deformiteleri ile ortopedi kliniğinde karşılaşılmaktadır. Bu deformiteler yürümede güçlük ve yetmezlik kırıklarına sebep olmaktadır. Tedavileri deformitenin ciddiyetine ve yaşa göre şekillenmektedir. Tedavinin esas amacı mekanik aks kadar anatomik aksı da düzeltip yetmezlik kırıklarını ve osteomalaziye sekonder deformite nüksünü önlemektir. Bunun için literatürde bu grup hasta için altın standart tedavi çocuk hastada mekanik aksı düzeltmeye yönelik hemiepifizyodez yöntemi, erişkin hastada ise multipl osteotomilerle metafizden metafize tespit sağlayan intramedülller çivi tespitidir. Fiksatörün deformite düzeltme etkisi ile kombine edilerek (fiksatör yardımlı çivileme) hastaya konforlu bir tedavi sunulabilir. Hasta bu şekilde hayat boyu konforlu bir mobilizasyona sahip olur.

ORTHOPAEDIC TREATMENT MODALITIES FOR THE LOWER EXTREMITY DEFORMITY CAUSED BY X LINKED HYPOPHOSPHATEMIC RICKETS

X linked hipophosphatemic rickets especially effects lower extremity. It mostly causes insufficiency fractures and difficulty in mobility. Treatment depends on seriosity of the deformity and age of the patients. The main purpose of the treatment is to correct both mechanical and anatomical axis and prevent the recurrence. Hemiepiphysiodesis is the treatment choice fort he young patients. For the adults multiple osteotomy and intramedullary fixation from proximal metaphysis to distal metaphysis is suggested. Combination of the external fixator for the correction ability of the deformity with internal fixation can provide more comfortable mobilization during the life.

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  • 1. Zaleske DJ, Doppelt SH, Mankin HJ. Metabolic and endocrine abnormalities of the immature skeleton. In Morrissey RT, editors. Lovell and winter’s pediatric orthopaedics. 3rd ed. Philadelphia: J.B. Lippincott; 1990. p. 203-261.
  • 2. Herring JA. Metabolic and endocrine bone diseases. In Herring JA, editors. Tachdjian’s pediatric orthopaedics. 4th ed. Philadelphia: Elsevier; 2008. p. 1917-1982.
  • 3. Pitt MJ. Rachitic and osteomalacic syndromes. Radiol Clin North Am 1981;19:581.
  • 4. Mankin HJ. Metabolic bone disease. Instr Course Lect 1995;44:3-29.
  • 5. Peterson BR. Augmenting vitamin D to combat genetic disease. Chem Biol 2002;9(12):1265-6.
  • 6. Fucentese SF, Neuhaus TJ, Ramseier LE, Exner GU. Metabolic and orthopedic management of X-linked vitamin-D resistant hypophosphatemic rickets. J Child Orthop 2008;2:285-91.
  • 7. Kocaoglu M, Bilen FE, Sen C, Eralp L, Balci HI. Combined technique for the correction of lower-limb deformities resulting from metabolic bone disease. J Bone Joint Surg Br 2011;93B:52-6.
  • 8. Kanel JS, Price CT. Unilateral external fixation for corrective osteotomies in patients with hypophosphatemic rickets. J Pediatr Orthop 1995;15:232-7.
  • 9. Rubinovitch M, Said SE, Glorieux FH, Cruess RL, Rogala E. Principles and results of corrective lower limb osteotomies for patients with vitamin D-resistant hypophosphatemic rickets. Clin Orthop Relat Res 1988;237:264-70.
  • 10. Petje G, Meizer R, Radler C, Aigner N, Grill F. Deformity correction in children with hereditary hypophosphatemic rickets. Clin Orthop Relat Res 2008;466:3078-85.
  • 11. Song HR, Soma Raju WJ, Kumar S, Lee SH, Suh SW, Kim JR, Hong JS. Deformity correction by external fixation and/ or intramedullary nailing in hypophosphatemic rickets. Acta Orthop 2006;77(2):307-14.
  • 12. Eralp L, Kocaoglu M, Cakmak M, Ozden VE. A correction of windswept deformity by fixator assisted nailing. A report of two cases. J Bone Joint Surg Br 2004;86(7):1065-8.