Westberry D E, Davids J R
Shriners Hospitals For Children: Greenville, 950 West Faris Road, Greenville, SC 29605, USA.
Hip Int. 2009 Jan-Mar;19 Suppl 6:S18-25. doi: 10.1177/112070000901906s05.
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.
近端股骨局灶性缺损(PFFD)是一种罕见的先天性异常,其特征为近端股骨和髋关节正常发育失败。临床表现和缺损程度存在显著差异是常见的。目前旨在改善功能性行走的管理策略很大程度上取决于股骨缩短的程度以及髋关节和膝关节的状况。PFFD病例中髋臼缺损和近端股骨畸形的治疗必须个体化。在PFFD轻度病例中,通过骨盆和股骨截骨术重建髋关节可能是可行的。在考虑肢体延长策略之前,必须实现髋关节和膝关节的稳定性。严重的PFFD病例可能无法通过手术矫正,需要采用诸如旋转成形术或选择性截肢等替代策略以方便假肢安装。