Golz-Gorlin or Basal cell naevus syndrome

Description: The syndrome is an autosomal dominant with incomplete penetrance. It is an ectodermal disorder and is characterised by: Basal cell naevi, over upper torso and shoulders with a risk of malignant change, particularly over the face. Odontogenic cysts. Bifid ribs and occasionally spina bifida calcification of the falx and sometimes in other soft tissues There is an associated increased incidence of ovarian fibromas.