A Case of Hypertelorism Without Mental Defect

Abstract

The name " ocular hypertelorisrn " has been given bv Greig to a cranic fticial deforimitv wvhose main feature is a wide separation of the orbits an(d their contents. He described two eases (both females) which he had had uinder observation for .a nuinber of years, and also the anatomical findings in the dried skull(2). Thce only other case in the literattirev which Greig couldl find wvas one de.seribed by Htitehison(3" a.s a ease of oxycephaly, but which the photograph showed to be one of hypertelorism. Recently, Muir'4) recorded a. case oceurring in a female child of 13 months, and he discussed the anatomical findings of Greig's cases and his theory of production. Morc reeentlv, Cockavne(l) described two ca.ses, one, 'a fernale of 7 months, and the othler, a boy of 6 years and 9 months. Cockayne camne to the conclusion that there wans nothing in the fa-mily historv of the-se six eases to support Greig's theory as to a hereditary fault. It is noted that of these six eases five were females with gross mental defect. The sixth case (C. F. J. of Cocka.yne) is a male and it is state(l that " mentally he is backward."A. W., nmale, aged 4-years. The present case shows no signs of mental backwardness. He is the elder of two children and was referred to my Out-Patient Department cn account of bronchitis. His facial peculiarity was noticed; also that the mother had rather widely separated eyes. The father was seen some time later and it was noticed that he also had widely separated eyes with some flattening of the bridge of the nose. An old " family group " of the father's family was consulted and here the same tendency was obvious. A portion of this is reproduced (Fig. 1) showving the, boy's father, ulncle, aunt and grandfather.T'lbe pregnancy and birth were normal. Although the parents noticed the boy's peculiar face, they did not pay any attention to it as he developed normally, sat up, walked and talked at the usual times.Physically there is no defect except that the scrotum is empty and qne testicle is in the inguinal canal. The fingers are rather short and tapering.He is also "pigeon breasted."There is no mental defect, in fact, he is more than a match for children of his own age. The greatest horizontal circumference of the head is 505 mms., the greatest lenath from glabella to occipuit 297 mms., and the greatest breadth from the level of the upper border of the pinna to a similar point on the other side 275 mms. T'he length of the head as measured by cephalonieter is 176 mms. and the breadth 136 mms.