Department of Orthopaedic Surgery, Boston Children's Hospital (Harvard Teaching Hospital), Boston, Massachusetts.
Department of Orthopaedic Surgery, Rainbow Babies and Children's Hospital, Cleveland, Ohio.
J Bone Joint Surg Am. 2021 Apr 21;103(8):715-726. doi: 10.2106/JBJS.20.00600.
Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes.
Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression.
Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS.
Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population.
Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.
先天性多发性关节挛缩症（AMC）是一种描述新生儿出生时存在≥2 处先天性挛缩的病症。尽管 3%至 69%的患者存在脊柱畸形，但 AMC 中关于生长友好型器械（GFI）的数据不足。我们的研究目的是描述 AMC 伴早发性脊柱侧凸（EOS）患儿目前 GFI 趋势，并与匹配的特发性 EOS（IEOS）队列进行比较，以确定脊柱僵硬或四肢挛缩是否影响结果。
从多中心 EOS 数据库（1993 年至 2017 年）中确定了≥30°脊柱畸形且接受 GFI 治疗≥24 个月的 AMC 患儿。采用倾向评分法，根据年龄、性别、器械和曲线，将 35 例 AMC 患儿与 112 例 IEOS 患儿匹配。多变量线性混合模型比较了两组患儿的脊柱畸形变化和 24 项早发性脊柱侧凸问卷（EOSQ-24）评分。采用多变量泊松回归分析队列并发症和再次手术。
术前，两组患儿的年龄（p = 0.87）、性别（p = 0.96）、器械（p = 0.62）、非手术治疗率（p = 0.54）和主要冠状面曲线幅度（p = 0.96）无差异。在初次 GFI 后，AMC 患儿的冠状面矫正百分比降低（35%比 44%；p = 0.01），残余冠状面曲线更大（49°比 42°；p = 0.03），但矫正的胸腰段后凸百分比相似（17%比 21%；p = 0.52）。在 GFI 毕业的患儿（n = 81）中，最终冠状面曲线幅度（55°比 43°；p = 0.22）和最终矢状面曲线幅度（47°比 47°；p = 0.45）在最终确定性手术后的最新随访中无显著差异。AMC 患儿的 T1-S1 长度更短（p < 0.001），T1-S1 生长速度相似（0.66 比 0.85mm/月；p = 0.05），EOSQ-24 评分在最新随访时更差（64 比 83 分；p < 0.001）。在校正了步行状态和 GFI 持续时间后，AMC 患儿的并发症发生率增加了 51%（发生率比，1.51 [95%置信区间（CI），1.11 至 2.04]；p = 0.009），每年并发症增加 0.2 个（95%CI，0.02 至 0.33 个；p = 0.03）。
AMC 和 EOS 患儿在初次手术治疗后，初始脊柱畸形矫正程度较低，但最终 GFI 曲线幅度和治疗期间 T1-S1 总生长量与 IEOS 患儿相比在统计学和临床方面均无显著差异。接受 GFI 治疗时间较长且不能行走的 AMC 患儿发生的并发症最多。需要多学科围手术期管理来优化 GFI，并改善这一复杂人群的生活质量。
治疗性 III 级。欲了解完整的证据水平描述，请参见作者须知。
