Vijayan Sandeep, Pai Harish, Shah Hitesh, Bhaskaranand Nalini
Department of Orthopaedics, Kasturba Medical College, Manipal University, Manipal, Udupi, Karnataka, India.
Department of Orthopaedics, Subbiah Medical College, Shimoga, Karnataka, India.
J Family Med Prim Care. 2017 Jul-Sep;6(3):651-653. doi: 10.4103/2249-4863.222011.
The fracture in a Factor XIII deficiency patient is being reported for the first time in the literature. We report a displaced fracture neck of femur in a 17-year-old boy with Factor XIII deficiency. Open reduction and internal fixation was done 8 days after the following the initial injury. Two units each of fresh frozen plasma and cryoprecipitate were given perioperatively to prevent excessive bleeding. No perioperative bleeding complications were encountered. At 18-months follow-up, the fracture had united with evidence of avascular necrosis. The fracture neck of femur in a child or young adult needs to be reduced and stabilized at the earliest to prevent devastating complications. Its occurrence in a patient with Factor XIII deficiency is to be managed like in a normal patient, but with extra perioperative care. Undue delay in fixation as happened in this case should be avoided for a better outcome.
因子 XIII 缺乏症患者发生骨折的情况在文献中首次被报道。我们报告了一名 17 岁患有因子 XIII 缺乏症的男孩发生股骨颈移位骨折。在初次受伤后 8 天进行了切开复位内固定术。术中给予两单位新鲜冰冻血浆和冷沉淀以预防过度出血。未遇到围手术期出血并发症。在 18 个月的随访中，骨折已愈合，但有缺血性坏死迹象。儿童或年轻成人的股骨颈骨折需要尽早复位并固定，以预防严重并发症。因子 XIII 缺乏症患者发生此类骨折的处理方式与正常患者相同，但围手术期需格外护理。应避免像本病例中那样的固定过度延迟，以获得更好的治疗效果。
