Gorlin-Goltz Syndrome : early detection and multidisciplinary management

AIM: The aim of this work is to underline the importance of the dentist in the contribution to the early diagnosis of Gorlin-Goltz Syndrome (GGS), avoiding and preventing so the orofacial and systemic complications, and to determine a proper dental and surgical protocol. METHODS: 21 GGS patients, 5-14 years of age, (mean age of 8,2 years) 12 females, 9 males, had undergone to a multidisciplinary treatment. In 8 cases the GGS has been detected by orthodontists. The 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 characteristic signs are: paget-like cranial appearance, wide flat nose, craniofacial asymmetry, hypertelorism, prognatism, costo-vertebral deformities, falx-cerebri calcificated, hyphoscoliosis, palmo-plantar hyperkeratosis. RESULTS: Diagnosis of the syndrome in childhood has been of great importance, because the early treatment contributed to avoid and reduce destruction of the jawbones and prevented severe complications in other organs. CONCLUSION: The dentists have a great responsability to detect GGS, finding signs through the radiographs used in dentistry. The approach must be multidisciplinary between different specialists of Dentistry and Medicine, for prevention of severe malocclusions, craniofacial and systemic tumors.