A Rare Osseous Dystrophy (Morquio)

Abstract

The excuse for reporting a single case lies, in this instance, in the twofold fact that it is unique in our experience, and that we have been able to find but one other apparently identical case in the literature.For about eight years, or since he was 10 months old, a boy with a striking general deformity of the body has been in and out of our clinic at the Children's Memorial Hospital. From the start an object of curiosity, he became increasingly so as he grew older and the deformities became more evident ( fig. 1). The early diagnosis of rickets\p=m-\madeeven then with much reservation\p=m-\wassoon abandoned.His diet and hygiene and his general care by an intelligent mother had all been unexceptionable. Beyond the period of infancy the whole clinical picture was unlike anything that could be attributed to rickets. Free consultation together with sporadic attempts at a more intensive study of the condition and a search through the literature left us no nearer a solution as to the nature and classification of this strange multiple deformity. While looking through the March, 1929, number of the Archives de médecine des enfants, one of us (Dr. Brennemann) came across a report by L. Morquio 1 of Montevideo, of four children out of five in one family all presenting an identical, widespread, osseous deformity.

A case of morquio's disease

“…Ruggles (1931) found thyroid and pituitary extracts to be of no value and in the present case, as in the one reported by Meyer and Brenneman (1932), vitamin D preparations caused no improvement. In Barnett's case (1933) osteotomy resulted in some improvement in walking.…”

“…Since 1929 reports of nine families have appeared in each of which two or more children have been affected in the same way (Ruggles, 1931 ;Barnett, 1933;Coward and Nemir, 1933 ;Davis and Currier, 1934 ;Valentin, 1930 ;Brown, 1933;Giraud and Bert, 1935 ;Summerfeldt and Brown, 1936). In addition six nonfamilial cases have been reported by Ruggles (1931), Meyer and Brenneman (1932), Sainz de los Terreros (1934), Freeman (1938 and Guerin and Lachapele (1938).…”