PYCNODYSOSTOSIS: SURGICAL CHALLENGES OF INTRAMEDULLARY NAILING FOR LONG BONE FRACTURES: A CASE REPORT

Authors : İbrahim CANBEYLİ, Meriç ÇIRPAR, Birhan OKTAŞ, Cüneyt Emre ÖKKEŞİM

Pages : 125-130

Doi:10.24938/kutfd.317075

View : 33 | Download : 7

Publication Date : 2017-08-31

Article Type : Other Papers

Abstract :Pycnodysostosis, known as osteopetrosis acro-osteolytica, is a rare sclerotic bone disease with an autosomal recessive heritage pattern. Characteristic features of the disease are short stature, delayed closure of cranial sutures, partial or total dysplasia of the phalanges, obtuse mandibular angle, skeletal abnormalities and increased bone density. Type I collagen catabolism and bone resorption are defective in these patient due to lysosomal cysteine protease and cathepsin K enzyme defects resulting in dense and more brittle bones. Herein, we report a 45-year-old pycnodysostozis patient with a subtrochanteric transverse fracture of the left femur. We aim to reveal the difficulties encountered during intramedullary nailing of long bone fractures of patients with pycnodysostosis.
Keywords : Pycnodisostosis, long bone fractures, intramedullary nailing

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