1709 COMPUTERIZED CRANIAL TOMOGRAPHY IN PATIENTS WITH SOTOS' SYNDROME

Abstract

Detailed assessment of patients with Sotos' Syndrome using current neuroradiographic procedures has not been widely reported. Twenty-one patients meeting the characteristics of this syndrome (Sotos, J. AJDC, Vol. 131, 1977) were studied. Seventeen had computerized cranial tomography, of which five were normal. Of the remaining twelve, nine had ventricular dilatation, two had cerebral atrophy, and two had agenesis of the corpus callosum. In eight of the patients the dilatation was mild, but one had significant dilatation requiring a V.P. shunt.

Several clinical findings not described previously were noted: two patients had atrial septal defects, three had tracheomalacia, and two had strabismus. In addition one had hypothyroidism, a finding previously described.

This is the first study to present the findings of computerized cranial tomography from a large number of patients with Sotos' Syndrome. The role of these findings in the pathogenesis is unclear, but the significant incidence of ventricular dilatation, cerebral atrophy, and agenesis of the corpus callosum makes it evident that C.T. scan of the head is an important part of the initial evaluation of patients with Sotos' Syndrome. Consideration must also be given to concurrent or associated disorders, such as cardiac anomalies, tracheomalacia, strabismus and hypothyroidism.

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