Department of Pediatric Orthopaedics Surgery, Necker Hospital, Assistance Publique Hopitaux de Paris, Université Paris-Cité, 149 rue de Sèvres, 75015, Paris, France.
Department of Pediatric Genetics, Necker Hospital, Assistance Publique Hopitaux de Paris, Université Paris-Cité, Paris, France.
Int Orthop. 2023 Jul;47(7):1863-1869. doi: 10.1007/s00264-023-05818-6. Epub 2023 May 12.
Osteogenesis imperfecta (OI) is a genetic disorder responsible for various symptoms including deformities and frequent fractures. Bone allografting is poorly documented in this condition. The objective of this study was to describe our experience and assessments in a consecutive series of OI patients.
Thirty-nine lower limb allograft procedures (28 femurs, 11 tibias) were performed in 26OI patients (mean age, 12.9 years). They were classified as type III of Sillence (17), type IV (6), and 3 recessive forms. The indications for surgery were correction of deformity (19), fracture (16), and non-union (4). In all cases, bone allografting was added to reinforce areas of fragility and in 28 cases for osteosynthesis to lock the rotations at the osteotomy site and to avoid screwed metallic plate. The duration of bone consolidation and allograft fusion was assessed. Complications and Gillette functional score were reported.
The mean follow-up was 6.7years (range, 2 to 10 years). On average, bone consolidation was achieved after 3.3 months and graft fusion after 7.7 months. No bone allograft-related complications were observed and there was any secondary displacement. The Gillette functional score was improved in 23 patients and stable in three cases. Complications were reported in two cases: one partial allograft resorption and one delayed consolidation of a non-union. One refracture was observed but after a significant trauma in a child who had regained significant physical activity.
Bone allografting in children with OI is a reliable method of biological fixation, allowing efficient fusion and contributing to increased bone capital and functional outcome.
成骨不全症(OI)是一种遗传性疾病,可导致多种症状,包括畸形和频繁骨折。骨同种异体移植在这种情况下的记录很少。本研究的目的是描述我们在一系列连续的 OI 患者中的经验和评估。
在 26 例 OI 患者(平均年龄 12.9 岁)中进行了 39 例下肢同种异体移植手术(28 例股骨,11 例胫骨)。他们被分为 Sillence Ⅲ型(17 例)、Ⅳ型(6 例)和 3 种隐性形式。手术指征为矫正畸形(19 例)、骨折(16 例)和骨不连(4 例)。在所有情况下,同种异体移植均用于强化脆弱区域,在 28 例中用于锁定截骨部位旋转的骨内固定,并避免金属板螺钉固定。评估骨整合和同种异体融合的持续时间。报告并发症和吉尔伯特功能评分。
平均随访 6.7 年(范围 2 至 10 年)。平均而言,骨整合在 3.3 个月后实现,移植物融合在 7.7 个月后实现。未观察到与同种异体移植相关的并发症,也未发生任何二次移位。23 例患者的吉尔伯特功能评分得到改善,3 例稳定。报告了 2 例并发症:1 例部分同种异体吸收和 1 例非骨不连延迟融合。在 1 例儿童中观察到 1 例再骨折,但该儿童在恢复了显著的体力活动后遭受了严重的创伤。
在 OI 儿童中进行骨同种异体移植是一种可靠的生物固定方法,可实现有效的融合,并有助于增加骨量和功能结果。