BRACHYDACTYLY TYPE A1 WITH ABNORMAL MENISCI AND SCOLIOSIS IN 3 GENERATIONS

We report a three-generation family in which four members had brachyda ctyly type Al, degenerative arthritis of the knee as a complication of abnormal menisci, and variable scoliosis. Nine of the 15 individuals in the two generations preceding the proband had brachydactyly. Three of these nine had degenerative arthritis of the knee including the pro band's father who had meniscal degeneration with tears. One other had radiologically confirmed discoid menisci. Of those with brachydactyly, five also had scoliosis. Although autosomal dominant inheritance of b rachydactyly Al and discoid menisci have been reported separately, cos egregation of these features in one family has not previously been des cribed and seems to comprise a unique autosomal dominant condition. Th e combination of brachydactyly, meniscal ab normalities including disc oid meniscus, and scoliosis suggests that this disorder represents a n ew osteochondrodysplasia syndrome.