PDF(Pubmed)
Abstract：
Focal fibrocartilaginous dysplasia (FFCD) is a rare disease that can cause angular deformities of long bones. The common pathologic finding is a thick fibrotic band extending from epiphysis to metaphysis on one side of the bone. The tethering effect of the fibrotic band around the growth plate is thought to be the main etiology for the development and progression of the deformity. FFCD mostly affects the proximal tibia and the distal femur. The literature contains different treatment options. Here, we present the case of a 20-month-old girl with FFCD on the medial side of the distal femur causing varus deformity. Our treatment protocol included excision of the fibrotic band from the medial side and application of a two-hole plate for guided growth on the lateral side of the distal femur. Deformity correction was achieved rapidly with no complications. A literature review is also presented along with pathologic and magnetic resonance imaging findings.
摘要：
局灶性纤维软骨发育不良（FFCD）是一种罕见的疾病，可引起长骨的角畸形。常见的病理发现是在骨的一侧从骨phy延伸到干phy端的厚纤维化带。生长板周围的纤维化带的束缚作用被认为是畸形发展和进展的主要病因。FFCD主要影响胫骨近端和股骨远端。文献包含不同的治疗选择。这里,我们介绍了一个20个月大的女孩，在股骨远端内侧患有FFCD，导致内翻畸形。我们的治疗方案包括从内侧切除纤维化带，并应用两孔板在股骨远端外侧引导生长。畸形矫正迅速，无并发症。还提供了文献综述以及病理和磁共振成像发现。
