Addressing surgical challenges in patients with severe form of osteogenesis imperfecta and with prolonged bisphosphonate treatment: intramedullary sclerosis and technical solutions.

IF 2.6 3区医学 Q2 ORTHOPEDICS

International Orthopaedics Pub Date : 2025-10-01 Epub Date: 2025-09-03 DOI:10.1007/s00264-025-06645-7

Željko Jeleč, Dragan Primorac, Vilim Molnar, Darko Antičević

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引用次数: 0

Abstract

Purpose: Osteogenesis imperfecta (OI) is commonly managed with bisphosphonates (BPs) which are associated with significant side effects. This study aimed to investigate intramedullary sclerosis as a potential side effect of prolonged BP use in paediatric patients.

Methods: Thirteen children with OI underwent surgery at our hospital. We assessed 21 non-operated bone segments from 13 paediatric patients using a tailored protocol to measure sclerosis severity and its impact on surgical outcomes.

Results: Among 21 non-operated segments, 17 exhibited intramedullary sclerosis. Pamidronate cycles inversely correlated with the percentage of the sclerosing area and varus deformity, plateauing after 15 cycles. Angulation in the sagittal plane decreased progressively.

Conclusions: Our study introduces intramedullary sclerosis as a possible novel BP-related side-effect in pediatric OI patients. Our findings suggest a critical review of prolonged BP therapy in OI patients, advocating for a surgical intervention threshold after fifteen BP cycles to mitigate complications and optimize treatment outcomes.

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解决严重成骨不全和长期双膦酸盐治疗患者的手术挑战：髓内硬化和技术解决方案。

目的：成骨不全症（OI）通常使用双膦酸盐（bp）治疗，其副作用明显。本研究旨在探讨儿科患者长期使用降压药的潜在副作用——髓内硬化。方法：13例成骨不全患儿在我院接受手术治疗。我们评估了来自13名儿科患者的21个未手术骨段，使用量身定制的方案来测量硬化严重程度及其对手术结果的影响。结果：21节段未手术，17节段出现髓内硬化。帕米膦酸盐周期与硬化面积和内翻畸形的百分比呈负相关，15个周期后趋于平稳。矢状面成角逐渐减少。结论：我们的研究将髓内硬化作为儿童成骨不全患者可能出现的一种新的bp相关副作用。我们的研究结果建议对成骨不全患者的延长血压治疗进行重要回顾，主张在15个血压周期后进行手术干预阈值，以减轻并发症并优化治疗结果。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

International Orthopaedics 医学-整形外科

CiteScore

5.50

自引率

7.40%

发文量

360

审稿时长

1 months

期刊介绍： International Orthopaedics, the Official Journal of the Société Internationale de Chirurgie Orthopédique et de Traumatologie (SICOT) , publishes original papers from all over the world. The articles deal with clinical orthopaedic surgery or basic research directly connected with orthopaedic surgery. International Orthopaedics will also link all the members of SICOT by means of an insert that will be concerned with SICOT matters. Finally, it is expected that news and information regarding all aspects of orthopaedic surgery, including meetings, panels, instructional courses, etc. will be brought to the attention of the readers. Manuscripts submitted for publication must contain a statement to the effect that all human studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study. Details that might disclose the identity of the subjects under study should be omitted. Reports of animal experiments must state that the "Principles of laboratory animal care" (NIH publication No. 85-23, revised 1985) were followed, as well as specific national laws (e.g. the current version of the German Law on the Protection of Animals) where applicable. The editors reserve the right to reject manuscripts that do not comply with the above-mentioned requirements. The author will be held responsible for false statements or for failure to fulfil the above-mentioned requirements.