1991 年 40 巻 1 号 p. 33-37
A 19 year-old male who had avascular necrosis in lateral condyle of the right femur with multiple epiphyseal dysplasia as well as flattening of the fourth lumbar vertebra was presented. Although the height was normal, the limbs were short. Three of his five siblings were also atypical epiphyseal dysplasia.
Fairbank (1947) described 20 cases of multiple epiphyseal dysplasia (MED) with the triad of dwarfism, stubby digits, dysplasia of epiphysis inherited by autosomal dominant trait. However, Ribbing (1955) reported arypical type of MED characterized by avascular necrosis of middle phalanx, osteochondritis dissecans, multiple flattening of spine, flattening of femoral heads, normal stature inherited by autosomal recessive trait. Our case representing avascular necrosis of lateral femoral condyle, flattening of femoral and humeral heads, flattening of lumbal spine was diagnosed as Ribbing type MED. There are a few reports describing MED with avascular necrosis of femoral head or osteochondritis dissecans of knee joint. However, no case of avascular necrosis of femoral chondyle has been reported. Histologically, osseous avascular necrosis with empyt lacunae was demonstrated by the specimen biopsied at the time of surgery. So, our case is supposedly a rare type of MED with avascular necrosis in femoral condyle described by Ribbing.