Persiani Pietro, Ranaldi Filippo Maria, Martini Lorena, Zambrano Anna, Celli Mauro, D'Eufemia Patrizia, Villani Ciro
Department of Anatomical, Histological, Forensic Medicine and Locomotive System Sciences.
Department of Pediatrics, Sapienza University of Rome, Rome, Italy.
J Pediatr Orthop B. 2019 Mar;28(2):179-185. doi: 10.1097/BPB.0000000000000536.
Osteogenesis imperfecta (OI) is a rare congenital osteodystrophy. Patients with OI present with osteoporosis, extreme bone fragility and severe deformities of the lower limbs, which predispose them to frequent fractures. The aim of our study is to describe the minimally invasive osteotomy technique to correct the tibial deformities in patients with OI type III, using the Fassier-Duval (FD) intramedullary nailing, which is considered the gold standard in this kind of surgery. We analyzed the results obtained from 14 patients with OI type III, treated for tibial deformities with the minimally invasive percutaneous osteotomy technique and osteosynthesis with the FD telescopic nail. The results were compared with that of a control group composed of 18 patients with OI type III, treated for tibial deformities with open technique osteotomies and osteosynthesis with FD telescopic nail. The follow-up was set at 18 months postoperatively. The data concerning the following were collected from the two groups: duration of surgery, number of osteotomies performed, postoperative pain, time required for functional recovery, and for the formation of bone callus. To analyze the variations in the quality of life, all the patients were given the Pediatric Outcomes Data Collection Instrument questionnaire, before surgery and at the end of the follow-up. In patients who underwent corrective surgery with the percutaneous technique, the average duration of surgery was inferior, the postoperative pain was significantly lower, the recovery of 90° range of motion of knee flexion was reached at an average of 37.8 days, and they ambulated bearing full weight on the leg without auxiliary aids on average 45 days after surgery. The Pediatric Outcomes Data Collection Instrument questionnaire values were satisfactory in both groups. The osteosynthesis with the FD telescopic nail, performed with the minimally invasive surgical technique, has improved the management of deformities in OI. The minimally invasive technique, however, requires the maturation of three distinct learning curves: surgery on patients with OI, open technique with the FD nail, and percutaneous technique with the FD nail.
成骨不全症(OI)是一种罕见的先天性骨发育不良。OI患者表现为骨质疏松、骨极度脆弱以及下肢严重畸形,这使他们容易频繁骨折。我们研究的目的是描述一种微创截骨技术,使用法西耶 - 杜瓦尔(FD)髓内钉来矫正III型OI患者的胫骨畸形,该技术被认为是此类手术的金标准。我们分析了14例III型OI患者采用微创经皮截骨技术及FD伸缩钉内固定治疗胫骨畸形所获得的结果。将结果与由18例III型OI患者组成的对照组进行比较,对照组采用开放技术截骨及FD伸缩钉内固定治疗胫骨畸形。随访设定为术后18个月。从两组收集了以下数据:手术时长、截骨数量、术后疼痛、功能恢复所需时间以及骨痂形成时间。为分析生活质量的变化,所有患者在手术前和随访结束时均接受了儿童结局数据收集工具问卷。接受经皮技术矫正手术的患者,平均手术时长较短,术后疼痛明显减轻,平均37.8天达到膝关节屈曲90°的活动范围恢复,平均术后45天即可在无辅助器具的情况下负重行走。两组的儿童结局数据收集工具问卷评分均令人满意。采用微创外科技术进行的FD伸缩钉内固定改善了OI畸形的治疗效果。然而,微创技术需要经历三个不同的学习曲线成熟阶段:对OI患者的手术、FD钉开放技术以及FD钉经皮技术。