Orthopaedics, All India Institute of Medical Sciences, New Delhi, Delhi, India.
Orthopedic Surgery, Indraprastha Apollo Hospital, New Delhi, India
BMJ Case Rep. 2023 Sep 18;16(9):e252667. doi: 10.1136/bcr-2022-252667.
Pycnodysostosis is a rare genetic condition that leads to generalised bony sclerosis and increased fracture risk. Orthopaedic specialists play a crucial role in managing affected children due to their susceptibility to frequent fractures. We had a case of a middle childhood female patient with pycnodysostosis and a femur fracture. Initially, an attempt using the Titanium Elastic Nailing System was made, but the sclerotic metaphyseal bone made it challenging. So, we opted for a 4.5 mm locked compressive plate, with multiple drill bits as a backup due to potential drill breakage. Though elastic nailing is preferred for paediatric long bone fractures, surgeons must be prepared for extremely sclerotic cortices and a narrow medullary canal when dealing with patients with pycnodysostosis. Open fixation and multiple drill bits in the toolkit are essential to overcome the potential obstacles during the procedure.
干骺型发育不全症是一种罕见的遗传性疾病,可导致全身性骨硬化和骨折风险增加。由于容易频繁骨折,矫形外科专家在治疗受影响的儿童方面发挥着至关重要的作用。我们有一例患有干骺型发育不全症和股骨骨折的中年女性患者。最初,尝试使用钛弹性钉系统,但硬化的干骺端骨质使操作变得具有挑战性。因此,我们选择了 4.5mm 锁定加压钢板,并准备了多个钻头作为备用,以防钻头断裂。虽然弹性钉是治疗儿童长骨骨折的首选方法,但对于患有干骺型发育不全症的患者,外科医生必须为极硬化的皮质和狭窄的髓腔做好准备。在手术过程中,开放式固定和工具包中的多个钻头对于克服潜在的障碍是至关重要的。