Orthopedic Surgery in Osteogenesis Imperfecta in Adults

Calcif Tissue Int. 2024 Dec;115(6):976-988. doi: 10.1007/s00223-024-01306-0. Epub 2024 Nov 16.

Abstract

Osteogenesis imperfecta is a genetic disorder, mainly characterized by bone fragility. In adult with osteogenesis imperfecta, surgical treatment may be indicated as an emergency procedure in the event of a fracture, or as a scheduled procedure in case of patient functional impairment due to many causes, such as persistent pain, complex bone deformities, and degenerative arthropathies. In all cases, a multidisciplinary approach is recommended. It is essential to consider the patient's characteristics and expectations regarding treatment, as well as the local conditions, which may be complex due to the poor bone quality, bone deformity, and the presence of hardware implanted during childhood. The procedure can be very complex and challenging and each patient is a unique case. An accurate preoperative planning is mandatory to select the appropriate surgical technique, which can be either conservative or prosthetic, and to evaluate its feasibility and the risk-benefit ratio. The aim is to improve the patient's quality of life and autonomy as long as possible.

Keywords: Arthroplasty (in OI); Fracture osteosynthesis (in OI); Orthopedic surgery (in OI); Preoperative planning in OI.

Publication types

  • Review

MeSH terms

  • Adult
  • Fractures, Bone / surgery
  • Humans
  • Orthopedic Procedures / methods
  • Osteogenesis Imperfecta* / complications
  • Osteogenesis Imperfecta* / surgery
  • Quality of Life