Supportive prosthetic treatment of adolecent patient with Gorlin-Goltz syndrome
Lek. dent. Karolina Chmura-Kmiecik, Dr. Wojciech Stós, Dr Krzysztof Gronkiewicz
Department of Prosthodontics and Orthodontics, Dental Institute, Jagiellonian University Medical College, Krakow, Poland;
Introduction
Gorlin Goltz’s syndrome is a rare autosomally inherited genetic disease, that is also known as nevoid basal cell carcinoma syndrome. Its diagnosis is based on the presence of major and minor criteria. Symptoms occur with variable intensity in nervus system, skin, endocrinal system, eyes and some of them manifest with skeletal and stomatognathic malformations.
Case Description
This work presents a case of a 10-year-old patient with a family history, diagnosed with disorder in the PTCH suppressor gene, confirming the occurrence of Gorlin Goltz syndrome. Manifestations within the stomatognathic system include occlusion disorders related to the occurrence of cystic tumors in mandible body on the right side and the mandible body and angle on the left side. The patient underwent prosthetic treatment using obturators during a two-stage surgical treatment of extensive bilateral mandible cysts.
Discussion
Gorlin-Goltz syndrome among others lead to occurrence of functional and aesthetic disorders within stomatognathic system. Apart from role in syndrome treatment radiological surveillance used in dentistry can play an important part in its early detection.