• Suparat Thamrat D.D.S., Grad. Dip. (Oral Medicine)
• Pannipa Thumasombut D.D.S., Grad. Dip. (Oral Medicine)
• Samornroj Arporniem D.D.S., Grad. Dip. (Oral Medicine)
• Wichitsak Cholitgul B.Sc., D.D.S., Diplomate, Thai Board of Oral Diagnostic Science

This literature review aims to report various defects appeared in patients with McCune-
Albright Syndrome which is a rare, multisystem disorder caused by GNAS 1 gene mutation.
This gene code for the stimulatory subunit of G protein involved in cyclic adenosine monophosphate
synthesis, resulting in persistently elevated cyclic adenosine monophosphate levels. The clinical
manifestation of the disorder is characterized by the triad of café-au-lait cutaneous spot, polyostotic
fibrous dysplasia and endocrinopatheis. Fibrous dysplasia frequently affects the craniofacial bones and
dental tissue causing disfigurement and developmental anomalies of teeth. These symptoms can be
early detected by the first sign of fibrous dysplasia during an oral examination and leading to approppriate
diagnosis of McCune-Albright Syndrome. The diagnosis usually relies on clinical informations
as facial asymetry including radiographic features as ground glass appearance. There is no specific
treatment for this syndrome, usually the symptomatic treatment with long term medical follow-up for
the endocrine disturbances is recommmended.

(CU Dent J. 2014;37:113-22)