Focal Fibrocartilaginous Dysplasia: Site-Specific Patterns of Presentation and Management-A Narrative Review

Background/Objectives: Focal fibrocartilaginous dysplasia (FFCD) is a rare, benign developmental disorder of the growing skeleton first described 40 years ago. It is characterised by a fibrocartilaginous tether adjacent to the physis, which disrupts symmetrical growth and leads to progressive angular deformity. The aim of this review was to define site-specific clinical patterns and management principles for FFCD to optimise patient outcomes. Methods: We conducted a narrative review of more than four decades of published literature on FFCD. All identified English-language case reports, case series, and review articles were analysed to synthesise evidence on clinical presentation, anatomical location, natural history and treatment strategies. Results: To date, 169 cases have been reported, with approximately two-thirds involving the proximal tibia. Tibial lesions typically present in toddlers as unilateral genu varum and, in most cases, demonstrate spontaneous remodelling and complete resolution. In contrast, femoral and upper-limb lesions rarely resolve spontaneously and often progress, thereby warranting earlier and more invasive management. Radiographic findings are highly characteristic, most commonly showing a cortically based metaphyseal lucency with a sclerotic rim. These features are generally considered sufficient for diagnosis, usually eliminating the need for biopsy. Management has evolved towards a tailored approach, consisting of observation for tibial lesions with potential for spontaneous resolution and timely surgical intervention for femoral or upper-limb lesions at risk of progression or joint compromise. Conclusions: Despite the advances made in recent decades, FFCD remains a distinctive yet heterogeneous condition in paediatric orthopaedics. This narrative review summarises more than four decades of published literature, including case reports, case series, and review articles, with particular attention to site-specific clinical patterns and their implications for optimising management.