Ruck Joanne, Dahan-Oliel Noémi, Montpetit Kathleen, Rauch Frank, Fassier François
J Child Orthop. 2011 Jun;5(3):217-24. doi: 10.1007/s11832-011-0341-7. Epub 2011 May 8.
To examine the functional outcomes of children with osteogenesis imperfecta (OI) following initial Fassier-Duval (FD) rodding to the femur at 1 year, and to determine which factors are associated with change in gross motor function, ambulation, and functional performance.
Approval from our Institutional Review Board was obtained. A retrospective chart review identified 60 children (28 males, 32 females) with OI who underwent initial FD femoral rodding (101 rods) and who were receiving bisphosphonates. The mean age of the children was 3 years, 11 months at the initial femoral FD rodding. Two had type I OI, 30 type III, 27 type IV, and one type VI. The maximum length of follow-up was 4 years. Telescoping FD rods were used for the femurs, with surgeries performed one leg at a time, with a 1-week interval. The active range of motion (AROM) of the hips and knees in flexion was measured 4-5 weeks post-initial rodding. Outcomes on the Gillette Functional Assessment Questionnaire (FAQ) Ambulation Scale, the Gross Motor Function Measure (GMFM), and the Pediatric Evaluation of Disability Inventory (PEDI) were compared pre-operatively and at 1 year post-surgery using t-tests and multivariate linear regression.
Pre-operatively, the mean FAQ score was 2.0, and this increased to 5.8 at 1 year post-surgery. Statistically significant improvements (P ≤ 0.05) were found on the FAQ, crawling, standing, walking and running, and total domains of the GMFM, and PEDI mobility and self-care from baseline to 1 year. The results from the multivariate linear regression indicate that older age (P = 0.0045) and higher weight (P = 0.0164) are significantly associated with lower scores in the self-care domain of the PEDI, and that OI type III compared to type IV is significantly associated (P = 0.0457) with greater improvement on the crawling domain of the GMFM. Higher weight was also associated (P = 0.0289) with lower scores in the standing domain of the GMFM, as well as with the total GMFM score (P = 0.0398).
Our findings indicate that initial FD femoral rodding resulted in benefits in ambulation, gross motor function, self-care, and mobility for children with OI beyond physiological expectations due to developmental growth. FD rodding is a procedure which can improve the overall mobility in children with OI with significant femoral deformities.
研究成骨不全症（OI）患儿初次接受法西耶 - 杜瓦尔（FD）股骨内固定术后1年的功能结局，并确定哪些因素与粗大运动功能、步行能力及功能表现的变化相关。
获得了我们机构审查委员会的批准。通过回顾性病历审查，确定了60例患有OI的儿童（28例男性，32例女性），他们接受了初次FD股骨内固定术（共101根棒）且正在接受双膦酸盐治疗。这些儿童初次进行股骨FD内固定术时的平均年龄为3岁11个月。其中2例为I型OI，30例为III型，27例为IV型，1例为VI型。最长随访时间为4年。股骨使用可伸缩的FD棒，手术每次只做一条腿，间隔1周。初次内固定术后4 - 5周测量髋、膝关节屈曲的主动活动范围（AROM）。使用t检验和多元线性回归比较术前和术后1年在吉列功能评估问卷（FAQ）步行量表、粗大运动功能测量（GMFM）以及儿童残疾评定量表（PEDI）上的结果。
术前，FAQ平均评分为2.0，术后1年增至5.8。从基线到术后1年，在FAQ、爬行、站立、行走和跑步、GMFM的各个领域以及PEDI的移动性和自我护理方面均发现了具有统计学意义的改善（P≤0.05）。多元线性回归结果表明，年龄较大（P = 0.0045）和体重较高（P = 0.0164）与PEDI自我护理领域得分较低显著相关，与IV型相比，III型OI与GMFM爬行领域更大的改善显著相关（P = 0.0457）。体重较高还与GMFM站立领域得分较低（P = 0.0289）以及GMFM总分较低（P = 0.0398）相关。
我们的研究结果表明，初次FD股骨内固定术为患有OI的儿童在步行、粗大运动功能、自我护理和移动性方面带来了超出因发育生长而产生的生理预期的益处。FD内固定术是一种可以改善患有严重股骨畸形的OI儿童整体移动性的手术。
