Although rare, fibular hemimelia or congenital fibular deficiency (CFD), is the most common congenital long bone deficiency, with an approximate incidence of 7.4- 20 per 1 million live births. The clinical presentation of CFD represents a broad spectrum of manifestations, ranging from mild fibular deficiency with limb length discrepancy to a significantly short limb with multiple associated foot, ankle and knee deformities. Traditional FH classification such as Achterman and Kalamchi described the amount of fibular deficiency, which is today known to be unrelated to length discrepancy and foot deformity. Current classifications are based on the associated deformities of the ankle and subtalar joint, as the foot deformity is the main prognostic factor. Treatment should be tailored for each patient to maximize the lower limb function - this involves predicting the limb length discrepancy and then coming up with a surgical plan to correct these in the fewest number of surgeries spread out as much as possible throughout the child’s growing years, so that by skeletal maturity the child has achieved equal leg length, a functional plantigrade foot, excellent alignment of the hip, knee and ankle and, as needed, a stable knee joint. Multimodal imaging provides detailed evaluation of the osteochondral and extraosseous malformations. In-utero identification can be accomplished with prenatal ultrasonography. After birth, radiographs often show striking bony anomalies. Detailed information regarding associated crucial cartilaginous, articular, soft tissue, and vascular abnormalities required for preoperative planning necessitates the use of magnetic resonance (MR) imaging. The purpose of this poster is to: 1) review the various types of osteochondral and extra-osseous abnormalities of CFD as depicted by different imaging modalities, 2) describe the limitations of each of these modalities, 3) outline the image-based classification of CFD, 4) describe the options for treatment, and 5) discuss the post-operative imaging evaluation of CFD.
SPR 2019 Annual Meeting & Postgraduate Course
congenital fibular deficiency