Bonneau Soline, Georges Samuel, Fraisse Bernard, Haumont Edouard, Lefèvre Yan, Bremond Nicolas, Pejin Zagorka, Violas Philippe
Service de Chirurgie Pédiatrique, CHU Angers, 4 Rue Larrey, 49100 Angers, France.
Service de Chirurgie orthopédique et traumatologie pédiatrique, Hôpital Necker Enfants malades - AP-HP, 149 Rue de Sèvres, 75015 Paris, France.
SICOT J. 2024;10:43. doi: 10.1051/sicotj/2024035. Epub 2024 Oct 25.
Leg length discrepancy (LLD) and malalignment of long bones are frequent orthopedic problems encountered in Maffucci syndrome and Ollier disease (OD). Orthopedic surgeons used historically external fixators to address the deformities. In this multicentric case series, we propose the use of motorized intramedullary nails.
We retrospectively reviewed for 9 years, in four different centers, patients with OD and Maffucci's syndrome that had lengthening nails for LLD with or without associated deformities. The minimum follow-up period was 24 months. We reported complications, clinical tolerance of lengthening, lengthening rate and target, bone healing index, and EQ-5D-Y functional and visual analog scores (VAS). We also saw on X-rays the whole lengthened bone and its regenerate zone to assess the evolution of the enchondromas.
we used the nailing technique in 8 femurs and 2 tibias in 8 patients (mean age: 13.3 years, range: 11-16, mean follow-up time: 53.8 months, range: 26-108). The mean correction amount was 6.44 cm for the femur over 76.8 days and 3.75 cm over 44 days for the tibia with a mean VAS score of 6.63/15 and mean EQ-5D-Y of 81/100. The lengthening goal was achieved in all patients. No mechanical complications were noted. The medullary canal of the operated bones showed improvement and healing in 8 out of 10 segments.
Besides achieving the goals of surgery with good functional outcomes, lengthening nails has a therapeutic effect on enchondromas with fewer complications than traditional correction methods.
肢体长度不等(LLD)和长骨排列不齐是马富西综合征和 Ollier 病(OD)中常见的骨科问题。骨科医生过去一直使用外固定器来矫正畸形。在这个多中心病例系列中,我们建议使用电动髓内钉。
我们回顾性分析了 4 个不同中心 9 年来患有 OD 和马富西综合征且因 LLD 伴或不伴相关畸形而接受延长钉治疗的患者。最短随访期为 24 个月。我们报告了并发症、延长的临床耐受性、延长率和目标、骨愈合指数以及 EQ-5D-Y 功能和视觉模拟评分(VAS)。我们还通过 X 光观察整个延长的骨骼及其再生区域,以评估内生软骨瘤的演变。
我们对 8 例患者的 8 根股骨和 2 根胫骨采用了髓内钉技术(平均年龄:13.3 岁,范围:11 - 16 岁,平均随访时间:53.8 个月,范围:26 - 108 个月)。股骨平均矫正量为 6.44 厘米,历时 76.8 天;胫骨平均矫正量为 3.75 厘米,历时 44 天,平均 VAS 评分为 6.63/15,平均 EQ-5D-Y 评分为 81/100。所有患者均达到延长目标。未发现机械并发症。10 个节段中有 8 个手术骨的髓腔显示有改善和愈合。
除了实现手术目标并获得良好的功能结果外,延长钉对内生软骨瘤具有治疗作用,且并发症比传统矫正方法少。