From the Research
Proximal focal femoral deficiency (PFFD) management should prioritize a multidisciplinary approach, with treatment options depending on the severity of the deficiency, and considering the patient and family's preferences and priorities, as stated in the most recent study 1. The condition is characterized by underdevelopment of the upper part of the femur bone, resulting in a shortened thigh and functional limb length discrepancy. Key aspects of management include:
- Limb equalization and deformity correction
- Nonsurgical management using prosthetics
- Amputation and limb lengthening
- Complex limb reconstruction
- Physical therapy to maintain muscle strength, joint mobility, and functional independence The most recent study 1 emphasizes the importance of maximizing function and considering the overall severity of the condition, as well as the patient and family's preferences and priorities, in making management decisions. In terms of specific treatment options, limb lengthening using external fixation devices like the Ilizarov apparatus may be performed in stages during childhood and adolescence for mild cases, while rotationplasty (Van Nes procedure) or amputation with prosthetic fitting might provide better functional outcomes in more severe cases 2, 1. Early intervention is crucial, with treatment typically beginning when the child starts to stand or walk, and long-term follow-up is necessary as growth continues, with adjustments to prosthetics or additional surgeries often required during developmental milestones.