Familial osteochondritis dissecans of the knee

Abstract

The autosomal dominant pattern of osteochondritis dissecans and association with mild dwarfism were observed during a study of 4 generations of one family. Twelve of 31 family members had proven osteochondritis dissecans and 8 others appeared to have or (now deceased) have had it; 10 of these 20 patients were of short stature (less than 5%). At the onset, children under 11 years of age had bilateral irregularities of ossification. Three of the 11 adolescents (13-19 years) had osteochondritis dissecans with development of the condition from irregularities of ossification that could be traced in the roentgenograph of one. Two of the 3 surviving adults now in their sixth or seventh decade have osteoarthritis (one moderate, one severe). The younger adults, now in the 20's and 40's, have minimal knee sysmptoms.