ENAMEL HYPOPLASIA, BILATERAL CATARACTS, AND AQUEDUCTAL STENOSIS - A NEW SYNDROME

We report on a 12-year-old girl who presented with generalized enamel hypoplasia, cataracts, and enlargement of the cerebral ventricles seco ndary to aqueductal stenosis. Previously described syndromes of enamel defects with or without cataracts were excluded on the basis of clini cal criteria and appearance of the dentition. Metabolic conditions whi ch could have caused cataracts were excluded clinically and by biochem ical tests. The combination of signs in this patient may represent a n ew syndrome. (C) 1995 Wiley Liss, Inc.