Pediatric Orthopedics, Nemours Children's Clinic, Pensacola, FL, USA.
Orphanet J Rare Dis. 2012 May 24;7 Suppl 1(Suppl 1):S1. doi: 10.1186/1750-1172-7-S1-S1.
The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon's approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and "lessons learned" are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.
骨多骨性纤维结构不良(FD)的手术治疗具有一定难度。目前,仍不清楚治疗相关骨畸形的最有效方法。受累骨的程度和模式存在明显差异,这使得获取数据来指导外科医生治疗这些患者变得困难。鉴于数据不足,但需要指导,马里兰州贝塞斯达国立卫生研究院召集了这些患者管理方面的公认专家,参加了一次国际会议,以解决与骨纤维结构不良相关的问题,讨论并完善他们关于这些具有挑战性患者的手术适应证和首选治疗方法的建议。本文介绍了具体的挑战、推荐方法和“经验教训”,希望面对典型畸形的外科医生能够在 FD 儿童和成人的手术重建中得到指导。