Westacott Daniel, Kannu Peter, Stimec Jennifer, Hopyan Sevan, Howard Andrew
The Hospital for Sick Children, Toronto, ON, Canada.
J Pediatr Orthop. 2019 Sep;39(8):e614-e621. doi: 10.1097/BPO.0000000000001116.
The proposed association between osteofibrous dysplasia and adamantinoma has led some to advocate resection of the entire lesion, which can require major subsequent reconstruction. However, this link remains unproven and there is some support in more recent literature for a less aggressive approach. This study aims to describe our experience managing pediatric tibial osteofibrous dysplasia with such an approach and to report functional outcomes in children treated thus.
A total of 28 cases of osteofibrous dysplasia in 25 patients were managed at a referral center for pediatric bone tumors with observation in the first instance, then limited surgical intervention if required to address pain and deformity. Surgery aimed to restore stability and alignment without excising the lesion. Clinical records provided basic clinical outcome measures involving walking, recreation, orthoses and school/work participation and patients provided a Musculoskeletal Tumour Society score (MSTS) where contactable.
Mean age at presentation was 6.0 years and mean follow-up was 8.3 years. Only 8 patients required surgery. According to basic outcome measures, 13 patients were symptom-free. About 15 patients (17 cases) provided a MSTS and the mean score was 24 of 30. No transformation to adamantinoma was observed. Those who presented at a younger age and with bilateral disease more often required surgery and remained symptomatic.
A less aggressive approach to pediatric tibial osteofibrous dysplasia achieves good functional outcomes and patient satisfaction in most cases. Surgery is required in the minority of cases. Transformation to adamantinoma was not observed in this series. We recommend patient education, clinical observation and reactive intervention if required, rather than proactive resection and reconstruction.
Level IV-case series.
骨纤维结构不良与造釉细胞瘤之间可能存在的关联使得一些人主张切除整个病灶，这可能需要后续进行大型重建手术。然而，这种关联尚未得到证实，并且最近的文献中有一些支持采取较保守方法的观点。本研究旨在描述我们采用这种方法治疗小儿胫骨骨纤维结构不良的经验，并报告接受该治疗的儿童的功能结局。
在一家小儿骨肿瘤转诊中心，共对25例患者的28例骨纤维结构不良病例进行了管理，首先进行观察，然后根据需要进行有限的手术干预以解决疼痛和畸形问题。手术旨在恢复稳定性和对线，而不切除病灶。临床记录提供了包括行走、娱乐、矫形器以及上学/工作参与情况等基本临床结局指标，并且在能够联系到患者的情况下，患者提供了肌肉骨骼肿瘤学会评分（MSTS）。
就诊时的平均年龄为6.0岁，平均随访时间为8.3年。只有8例患者需要手术。根据基本结局指标，13例患者无症状。约15例患者（17例病例）提供了MSTS评分，平均得分为30分中的24分。未观察到向造釉细胞瘤的转变。那些发病年龄较小且患有双侧疾病的患者更常需要手术，并且仍有症状。
对于小儿胫骨骨纤维结构不良，采用较保守的方法在大多数情况下可取得良好的功能结局和患者满意度。少数病例需要手术。本系列中未观察到向造釉细胞瘤的转变。我们建议对患者进行教育、临床观察，并在需要时进行反应性干预，而不是积极地进行切除和重建。
IV级——病例系列。
