Department of Pediatric Orthopedics, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
J Med Life. 2020 Oct-Dec;13(4):543-547. doi: 10.25122/jml-2020-0161.
Osteogenesis imperfecta (OI) is a genetically determined systemic pathology that involves a disturbance in the synthesis of type one collagen and is mainly characterized by bone brittleness and other abnormalities. The musculoskeletal system is the most affected by bone fracture after mild mechanical stress occurs. Pathologic bowing appears without trauma, and hyperlaxity is jeopardizing joint stability. Having such an abrupt debut, some patients report in-utero fractures, and treatment is very challenging since surgery has to be postponed until the children grow and become good candidates for intramedullary nailing. In this paper, we discuss the experience of "Grigore Alexandrescu" Hospital for Children in treating these patients and the results we obtained regarding the revision surgeries that we had done. Revision surgery is mandatory for OI patients, and there are multiple factors in deciding to use an implant. We looked back at the data collected for those cases where revision was not related to infection, trauma, or disengagement of the male-female system due to the overlengthening of the implant. The conclusions made are comparable to other centers worldwide with respect to lower limb surgery, and we changed the management protocol according to the problems encountered.
成骨不全症(OI)是一种由基因决定的系统性疾病,涉及到 I 型胶原合成的紊乱,主要表现为骨骼脆弱和其他异常。在轻微机械应激后,骨骼系统最容易受到骨折的影响。病理性弯曲在没有外伤的情况下出现,关节过度松弛会危及关节稳定性。有些患者在子宫内就发生骨折,病情进展迅速,治疗极具挑战性,因为手术必须推迟到儿童生长并成为髓内钉内固定的合适人选。本文讨论了“Grigore Alexandrescu”儿童医院治疗这些患者的经验以及我们在修正手术方面取得的成果。修正手术对于 OI 患者来说是强制性的,在决定使用植入物时有多个因素。我们回顾了那些不是由于感染、外伤或由于植入物过度延长导致男女系统分离而导致修正手术的病例的数据。与其他全球中心相比,下肢手术的结论是相似的,我们根据遇到的问题改变了管理方案。