Craniofacial fibrous dysplasia associated with McCune-Albright syndrome.

摘要

An 18-year-old boy presented to our hospital in August 2006 for management of an extremely large lesion that was causing considerable distortion of his face (Fig 1). The patient was an orphan raised by his grandmother who had suffered from dementia for years. Therefore, part of the patient's clinical history was described by his neighbors, and the information was not as accurate as desired. The patient was said to have had a rigid tumor-like lesion protruding from the palatal vault into his oral cavity about 10 years previously. The lesion was estimated to be as large as 3 cm in diameter in the beginning. According to the neighbors' accounts, the lesion may have originated from the left maxilla. The lesion was painless, and increased very slowly in the early years, but it had been swelling rapidly since 2002 (Fig 2), especially in the previous 3 months, and the maximum diameter of the lesion had reached 20 cm when he visited our hospital. The giant lesion had caused the patient great difficulties in speaking, eating, and even breathing. The patient also stated that the lesion had recently become painful, and the surface of the lesion often ulcerated. For financial reasons, he had not received any therapy before he visited our hospital.