Jaw Keratocysts and Sotos Syndrome

Sotos syndrome, described by Sotos et al. [1], is characterized by excessive growth during childhood, macrocephaly, distinctive facial appearance and learning disability. The disorder is largely caused by mutations or deletions in the NSD1 gene. The typical facial gestalt includes macrodolichocephaly with frontal bossing, front-parietal sparseness of hair, apparent hypertelorism, down slanting palpebral fissures, and facial flushing. This paper discusses a case of Sotos syndrome in an adolescent male with multiple odontogenic keratocysts in his jaw bones, a previously unreported oral manifestation, out with a syndromic context.