Gorlin-Goltz Syndrome: early detection and multidisciplinary approach

Aim: the aim of this work is to highlight the importance of the dentist in contributing to early diagnosis of Gorlin-Goltz Syndrome (GGS), avoiding and preventing orofacial and systemic complications. The goal is also to establish a proper dental and surgical protocol. Methods: twenty-one patients affected by GGS, aged 5 to 14 years (mean age 8.2 years), including twelve females and nine males, underwent multidisciplinary treatment. In 8 cases GGS was detected by orthodontists. GGS or Basal Cell Naevus Syndrome is a generalized disorder with autosomal dominant inheritance and variable expressivity. The main symptoms are recurrent multiple jaw keratocysts and basal cell naevi of the skin. Other characteristics are Paget-like cranial appearance, wide flat nose, craniofacial asymmetry, hypertelorism, prognathism, costo-vertebral deformities, calcified falx-cerebri, hyphoscoliosis, palmo-plantar hyperkeratosis. Results: diagnosis of Gorlin-Goltz Syndrome in childhood has been crucial, because the early treatment helped to prevent and reduce jawbones' destruction and prevented severe complications in other organs. Conclusions: dentists have a great responsibility to detect GGS, by identifying signs in dental radiographs. The approach must be multidisciplinary involving specialists from both Dentistry and Medicine, to prevent severe malocclusions, as well as craniofacial anomalies and systemic tumors.