Vlaić Josip, Ribičić Tomislav, Bojić Davor, Antičević Darko
Division of Paediatric Orthopaedic Surgery, Children's Hospital Zagreb, Zagreb, Croatia.
School of Medicine, University of Zagreb, Zagreb, Croatia.
Acta Clin Croat. 2023 Aug;62(Suppl3):18-24. doi: 10.20471/acc.2023.62.s3.2.
Spondyloepiphysal dysplasia congenita (SEDc) is a rare autosomal dominant genetic disorder. Femoral head ossification delay and the proximal femur varus deformity i.e. coxa vara (CV) are the major features of SEDc. The accepted treatment is a valgus femoral osteotomy. The data on hip surgery in SEDc are scarce. In our database from 2006 to 2020, there were 6 SEDc patients. Four patients had surgery on 8 hips. Surgical treatment was indicated due to progressive CV deformity i.e. a decreasing neck-shaft angle (NSA), pain, limited hip abduction, and gait disturbances. In three patients, a novel surgical treatment was applied - a greater trochanter apo-physiodesis using "Eight-plate". The patients were evaluated clinically and radiologically. The median age at first surgery was 6.3 years (range, 3.2 to 9.5 y) and the median follow-up period was 7 years (range, 5.6 to 14 y). The postoperative NSA was significantly improved with a mean increase of 13 degrees (P<0.001). Additional surgeries were needed in two patients. Overall, our results showed improved clinical and radiological parameters. The purpose of this study was to determine whether a method using an "Eight- plate" applied early to greater trochanter apophysis in SEDc patients with bilateral CV could reduce the need for more aggressive surgery in near future.
先天性脊椎骨骺发育不良（SEDc）是一种罕见的常染色体显性遗传病。股骨头骨化延迟和股骨近端内翻畸形即髋内翻（CV）是SEDc的主要特征。公认的治疗方法是股骨外翻截骨术。关于SEDc患者髋关节手术的数据很少。在我们2006年至2020年的数据库中，有6例SEDc患者。4例患者的8个髋关节接受了手术。手术治疗的指征是CV畸形进展，即颈干角（NSA）减小、疼痛、髋关节外展受限和步态障碍。在3例患者中，采用了一种新的手术治疗方法——使用“八钢板”进行大转子骨骺固定术。对患者进行了临床和影像学评估。首次手术的中位年龄为6.3岁（范围3.2至9.5岁），中位随访期为7年（范围5.6至14年）。术后NSA显著改善，平均增加13度（P<0.001）。2例患者需要再次手术。总体而言，我们的结果显示临床和影像学参数有所改善。本研究的目的是确定在双侧CV的SEDc患者中，早期将“八钢板”应用于大转子骨骺的方法是否能在不久的将来减少更积极手术的需求。
