The knee joint in diastrophic dysplasia - A clinical and radiological study

We examined clinically and radiologically the knees of 46 patients (27 fema les and 19 males) with diastrophic dysplasia, The age of the patients varie d from newborn to 38 years. A total of 18 patients was followed during thei r growth until adolescence, The knees of two legally aborted fetuses appear ed on examination to be macroscopically normal and congruous. Excessive val gus deformity of the tibiofemoral weight-bearing angle with a mean of 14 de grees was noted in infancy Most of the patients had marked instability of t he knees. The range of movement of the knee began to decrease before the ag e of five years. There were signs of early degeneration and deformation of the bony epiphyses before the age of six gears, The patellofemoral joint wa s abnormal from an early age. A marked patella infera, often associated wit h a lateral position of the patella with bony fragmentation, was noted, The knee in diastrophic dysplasia is basically unstable, showing early deforma tion of the subchondral bone and degeneration of the joint.