MCCUNE-ALBRIGHT-SYNDROME PROGRESSING WITH SEVERE FIBROUS DYSPLASIA

We present the case of an 11-year-old girl with McCune-Albright syndro me associated with severe fibrous dysplasia. In addition to bone lesio ns, she has apparent manifestations of precocious puberty. In examinat ion, a mass at the mentum spreading to mandibular corpus bilaterally w as seen. This mass has affected the mandibular teeth. It was nearly 20 x 20 x 15 cm in size and had local necrotic regions on it. The lower lip was expanded too much by the mass. Another mass filled the left ma xillary sinus, expanding the left zygomatic region outwardly and closi ng the left nasal fossa completely. A rectangular skull shape was rela ted to the involvement of cranial bones. After stainless steel, custom -made mandibular prosthesis was prepared, the patient underwent surger y. A partial mandibulectomy was performed, and resulting mandibular bo ne defect was reconstructed by steel prosthesis. Craniofacial involvem ent occurs in 100% of disseminated cases. In the dental literature, ma ndibular involvement was found in 20% of cases. However, in studied li terature, we did not find a dramatic mandibular lesion as severe as th at presented here.