Vol.95 No.9 contentsPRACTICA OTO-RHINO-LARYNGOLOGICA
Vol. 95 No. 9 September 2002
A Case of McCune-Albright Syndrome with
Surgical Treatment
Ryuichiro Saijo, Takeshi Shimizu, Syuichiro Hayashi,
Takatsugu Tsunoda and Yuichi Majima
(Mie University)
We report a 23-year-old female diagnosed with McCune-Albright syndrome (MCAS), which is a rare disease consisting of polyostotic fibrous dysplasia of the bone associated with brown pigmented areas of the skin and several endocrine dysfunctions. The diagnosis was made at the age of 5. Surgical treatment was performed at the ages of 13 and 17, to correct asymmetric lesions of her face. After the first and second surgery, the resected maxillary bone regrew rapidly for 3～12 months postoperatively. We recommend that surgical treatment should be performed after the arrest of bony growth on CT, arrest of growth in height and in weight and normalization of ALP. Patients with MCAS should be reviewed at 6～12 month intervals.
Key words : McCune-Albright syndrome, surgical therapy for McCune-Albright syndrome, fibrous dysplasia