Oral manifestations of McCune-Albright syndrome

Abstract

McCune- Albright Syndrome (MAS) is a rare fibrosseous lesion, characterized by a classic triad of polyostotic fibrous dysplasia (PFD), café –au-lait macules (CALM) and underlying endocrinopathies. We present the oral findings of an interesting case of MAS with relevant review of literature. A 30-year-old male presented to us with swelling of both jaws over a period of two years. Cutaneous examination revealed café - au – lait macule over the back, crossing the midline. Skeletal survey showed expansile, osteolytic, mixed radiolucent- radiopaque lesions in skull and jaw bones. Serum alkaline phosphatase was elevated (388 IU/L), with normal calcium, phosphorus, parathyroid hormone and 25 hydroxy vitamin D levels. Diagnosis of McCune- Albright syndrome was made and he was treated with parenteral bisphosphonates (intravenous Zoledronate 4 mg) and is under follow up for surgical recontouring of the jaws. Early recognition facilitates better treatment and improves prognosis by reducing the morbidity.

Metabolic Bone Diseases Affecting Tooth Eruption: A Narrative Review

“…It disrupts skeletal stem cell differentiation due to excess cAMP, leading to skeletal deformities, café-au-lait spots, and endocrine gland hyperfunction. MAS is commonly presented with fibrous dysplasia of the craniofacial bones and supernumerary teeth, leading to impacted TE, DTE, and abnormal bone development [ 111 , 112 , 113 ].…”

Clinical and translational pharmacological aspects of the management of fibrous dysplasia of bone

“…Since then, a number of publications reported variable beneficial effects of different bisphosphonates on pain, bone turnover markers and radiological features of FD/MAS lesions largely in adults and children with polyostotic FD/MAS. These publications include 18 case reports , 20 observational case series and a single randomized controlled study (RCT) using oral alendronate .…”

“…Case reports were predominantly on women (11/18), seven on children and six reporting the effects of treatment with zoledronate and pamidronate in patients with isolated craniofacial FD (CFD) . Positive outcome of treatment in the form of decreased pain was reported in all patients, associated by a concomitant normalization in bone turnover markers in six of the 17 patients in whom these markers were measured before and after treatment .…”