GROWTH DISORDER OF SKULL IN MONGOLISM

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Abstract
The synchondrosis spheno-occipitalis of 6 mongoloid patients, ages between 8 mos. and 30 yrs.; the synchondrosis spheno-ethmoidalis of 2 mongoloid children; and vertebrae of 2 mongoloid children were prepared for a histologic study of bone and cartilage. In addition, the rib cartilage of an 8 mos. old mongoloid child was described. The study of the cartilage revealed lack of formation of preparatory columns and absence of proliferation while the boney ridges are heavily ossified and the boney border appeared perforated by primary marrow cavities. The synchondrosis spheno-ethmoidalis was found wide open with very little signs of ossification of the fibrous tissue. After evaluation of these observations, the difference between chondro-dysplasia, cretinism and mongolism is discussed. In chon-drodysplasia, a primary disease of the cartilage with early absorption and ossification is present while the membranous bones and the periosteal ossification are not involved. In cretinism, the transformation of cartilage into bones is affected, the ossification therefore being retarded. In mongolism, there is a definite deficiency of cartilage, this tissue being neither growing nor absorbed. The ossification, itself, appears normal. The transformation of fibrous tissue in the sutures of the skull was also found to be retarded and insufficient. An analysis of these observations indicated that the growth disorder in mongolism depends on a congenital absence or deficiency of those agents which, either from hypophyseal or extra hypophyseal sources, stimulate differentiation and growth.

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