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Proximal focal femoral deficiency (PFFD): management options and controversies
1Shriners Hospitals For Children: Greenville, 950 West Faris Road, Greenville, SC 29605, USA. dwestberry@shrinenet.org
Insights
Proximal focal femoral deficiency (PFFD) is a congenital hip anomaly with varied severity. Management for PFFD requires individualized treatment, focusing on hip and knee stability before considering limb lengthening or reconstructive surgery.
Area of Science:
- Orthopedics
- Pediatric Orthopedics
- Congenital Anomalies
Background:
- Proximal focal femoral deficiency (PFFD) is a rare congenital anomaly.
- It involves the underdevelopment of the proximal femur and hip joint.
- PFFD presents with significant variability in clinical presentation and deficiency degree.
Purpose of the Study:
- To outline current management strategies for PFFD.
- To emphasize the individualized approach required for PFFD treatment.
- To discuss surgical and non-surgical options based on PFFD severity.
Main Methods:
- Review of current literature and clinical practices for PFFD.
- Analysis of treatment strategies based on the degree of femoral shortening.
- Evaluation of hip and knee joint status in PFFD management.
Main Results:
- Management strategies are dictated by femoral shortening and hip/knee joint status.
- Mild PFFD cases may benefit from reconstruction with pelvic and femoral osteotomies.
- Achieving hip and knee stability is crucial before limb lengthening.
Conclusions:
- Individualized treatment is essential for acetabular deficiency and proximal femoral deformity in PFFD.
- Severe PFFD cases may require rotationplasty or amputation for prosthetic fitting.
- Treatment decisions must prioritize functional ambulation and joint stability.
Abstract:
Proximal focal femoral deficiency (PFFD) is a rare congenital anomaly characterised by failure of normal development of the proximal femur and hip joint. Significant variability in the clinical presentation and degree of deficiency is common. Current management strategies aimed at improving functional ambulation are largely dependent on the degree of femoral shortening and the status of the hip and knee joint. Treatment of acetabular deficiency and proximal femoral deformity in cases of PFFD must be individualised. Reconstruction of the hip joint with pelvic and femoral osteotomies may be possible in mild cases of PFFD. Stability of the hip and knee joint must be achieved prior to consideration for limb lengthening strategies. Severe cases of PFFD may be beyond surgical correction and warrant alternative strategies such as rotationplasty or selective amputation to facilitate prosthetic fitting.
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