COSTELLO-SYNDROME IN 2 BRAZILIAN CHILDREN

The increasing number of children with Costello syndrome described wor ld wide has helped in delineating further the characteristic features of this condition. We report here two children, seen in the Genetic Di vision of the Brasilia University Hospital, showing the main features of the syndrome: ''coarse'' face, redundant skin on the feet and hands , hyperextensible hand and finger joints, curly hair, growth and psych omotor retardation, and feeding problems. In addition, one of the pati ents developed hydrocephalus during the evolution of the disease, maki ng the third published patient with this complication. We discuss the appropriateness of serial brain imaging studies in Costello syndrome i n light of the relative frequency of central nervous system malformati ons.