Management of long bone fractures in patients with pycnodysostosis

被引:1
作者
Sharma, Ajay [1 ]
Mittal, Samarth [1 ]
Trikha, Vivek [1 ]
Vatsya, Pulak [2 ]
机构
[1] All India Inst Med Sci, Orthopaed, New Delhi, India
[2] Indraprastha Apollo Hosp, Orthoped Surg, New Delhi, India
关键词
Orthopaedics; Congenital disorders; Medical education;
D O I
10.1136/bcr-2022-252667
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页数:4
相关论文
共 14 条
  • [1] "Challenges in the management of fractures in osteopetrosis"! Review of literature and technical tips learned from long-term management of seven patients
    Bhargava, Amit
    Vagela, Manesh
    Lennox, C. M. E.
    [J]. INJURY-INTERNATIONAL JOURNAL OF THE CARE OF THE INJURED, 2009, 40 (11): : 1167 - 1171
  • [2] Bisaccia M., 2020, Tibial diaphyseal fractures in children: indications and limitations of the treatment with monolateral and hybrid external fixator
  • [3] Bocchi MB, 2022, J Xiangya Med, V7, P29
  • [4] Fracture management in pycnodysostosis: 27 years of follow-up
    Bor, Noam
    Rubin, Guy
    Rozen, Nimrod
    [J]. JOURNAL OF PEDIATRIC ORTHOPAEDICS-PART B, 2011, 20 (02): : 97 - 101
  • [5] Pycnodysostosis, a lysosomal disease caused by cathepsin K deficiency
    Gelb, BD
    Shi, GP
    Chapman, HA
    Desnick, RJ
    [J]. SCIENCE, 1996, 273 (5279) : 1236 - 1238
  • [6] Jiya TU, 2006, Eur J Trauma, V32, P477, DOI DOI 10.1007/S00068-006-5085-6
  • [7] Subtrochanteric fracture managed by intramedullary nail in a patient with pycnodysostosis
    Kundu, ZS
    Marya, KM
    Devgan, A
    Yadav, V
    Rohilla, S
    [J]. JOINT BONE SPINE, 2004, 71 (02) : 154 - 156
  • [8] Images in medicine Pycnodysostosis presented with tibial shaft fracture
    Mahmoudi, Abdelhalim
    Bouabdallah, Youssef
    [J]. PAN AFRICAN MEDICAL JOURNAL, 2020, 35
  • [9] Matar Hosam E, 2014, BMJ Case Rep, V2014, DOI 10.1136/bcr-2014-207730
  • [10] Mori Satoshi, 2009, Clin Calcium, V19, P699, DOI CliCa0905699708