Early detection and management of Gorlin-Goltz syndrome: The orthodontist’ s role

AIM: The aim of this work is to underline the importance of the orthodontists in the contribution to the early diagnosis of Gorlin-Goltz Syndrome (GGS), avoiding and preventing so the orofacial and systemic complications SUBJECTS AND METHODS: 11 GGS patients, 5-18 years of age, 7 females, 4 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 CONCLUSIONS: The orthodontists have a great responsability to detect GGS , finding signs through the radiographs used in orthodontics. The approach must be multidisciplinary between different specialists of Dentistry and Medicine, for prevention of severe malocclusions, craniofacial and systemic tumors.