Oral manifestations of McCune-Albright syndrome
A-30-year old man presented with a slow growing painless swelling involving both jaws, over a period of 2 years. He stated that the swelling was initially small, but gradually enlarged to cause spacing between teeth and noticeable facial asymmetry. This swelling was not associated with pain, paresthesia or pus discharge. History taking from his parents revealed recurrent fractures since the age of 4 years, brown pigmented patches over his trunk since birth, attainment of puberty at the age of 12 years and no similar features in other family members. There was no history of visual impairment, nasal stuffiness or hearing disability. He was a product of non consanguineous marriage, born full term with normal birth weight and milestones. Physical examination showed short stature in comparison to parents (height-142 cm), multiple deformities due to recurrent fractures, shortened right leg, limping and a large café au lait macule over the back crossing the midline. Facial examination revealed frontal bossing, asymmetry on the right side, with an enlargement of the right malar and maxillary bone and chin button deviation towards the left side [Figures and ]. He had a prognathic mandible, with lengthening of the body on the right side [].\nIntraoral examination revealed well defined uniform expansion of the mandible up to the inferior border, extending from the third molar on the right side to the premolars on the left side. The labial and right buccal vestibules of the mandible were shallow due to the swelling. The mucosa over the swelling showed black patchy pigmentation and dilated superficial veins. The swelling was non tender and bony hard on palpation. The right maxilla was enlarged diffusely from the lateral incisor to the second molar with normal mucosa and was nontender with bony hard consistency [].\nThe midline of both maxillary and mandibular teeth had shifted to the left, with an anterior cross bite and spacing between maxillary and mandibular anterior teeth. Grade I mobility and labial tipping were seen in 41, 42. The overjet between the right maxillary and mandibular posterior teeth was reduced. He was diagnosed clinically as a case of MAS. Hormonal profile revealed normal thyroid, adrenal and gonadal axes evaluation. The serum alkaline phosphatase was elevated (388 IU/L), with normal calcium, phosphorus, parathyroid hormone and 25 hydroxy vitamin D levels.\nSkeletal survey by paranasal sinus view of the skull revealed expansile, ill defined mixed radiopaque- radiolucent lesions in the frontal bone, ground glass radiopaque lesion in the right maxilla causing obliteration of the maxillary sinus and an expansion of the right body of the mandible []. The OPG showed an extensive multilocular lesion, extending 1 cm below the sigmoid notch of the ascending ramus of the right side to mesial aspect of the mandibular left first molar []. The lesion caused an increase in width of the right body and symphysis with a loss of normal trabecular pattern. The mandibular canal was displaced inferiorly towards an intact lower border. The right maxillary sinus was occluded and the right maxillary alveolar process in the posterior region showed orange peel pattern. Loss of lamina dura was noted for teeth located within the lesion. Expansive, osteolytic lesions were also seen in right hip and shoulder. He was treated with parenteral bisphosphonates (intravenous Zoledronate 4 mg) and is under follow up for surgical recontouring of the jaws.