OSTEOCHONDRODYSTROPHIA DEFORMANS (MORQUIO'S DISEASE)

Abstract

IN 1941, we reported 3 cases of osteochondrodystrophia deformans¹ (eccentro-osteochondrodysplasia). In 2 of these the disease was far advanced, and in 1 it was thought to represent an early stage. No detailed report of an autopsy on a patient with osteochondrodystrophia deformans exists in the literature. Recently 1 of these 3 patients (case 2, C. P.) died, and observations at autopsy were made available for this report. Osteochondrodystrophia deformans, or Morquio's disease, is a skeletal disease characterized by (1) dwarfism, (2) deformities of the bones of the trunk and the extremities and (3) changes observed roentgenologically consisting of absence of centers of ossification and destruction, rarefaction and proliferation involving all of the bones of the body, including, directly or indirectly, those of the skull. In 1929, Morquio² described a form of osseous dystrophy occurring in 2 of a family of 5 children, and in 1935 he reported 2