Department of Orthopaedics and Traumatology, Hacettepe University, School of Medicine, Ankara, Turkey.
Department of Pathology, Hacettepe University, School of Medicine, Ankara, Turkey.
Acta Orthop Traumatol Turc. 2021 Mar;55(2):184-188. doi: 10.5152/j.aott.2021.20080.
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.
局灶性纤维软骨发育不良(FFCD)是一种罕见疾病,可导致长骨的成角畸形。常见的病理表现为一侧骨的骺端至干骺端有一条增厚的纤维带。纤维带围绕生长板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。文献中包含不同的治疗选择。在这里,我们介绍了一例 20 个月大的女孩,其股骨远端内侧发生 FFCD 导致内翻畸形。我们的治疗方案包括从内侧切除纤维带,并在股骨远端外侧应用两孔板进行引导生长。畸形矫正迅速,无并发症。还对文献进行了回顾,并介绍了病理和磁共振成像表现。