Medical ConditionsEndocrine, GI & renal: Fibrous dysplasia and McCune-Albright syndrome
Medical ConditionsEndocrine, GI & renal: Fibrous dysplasia and McCune-Albright syndrome
Fibrous dysplasia and McCune-Albright syndrome
Overview
Board takeaway: Assess jaw lesions, endocrine control, and antiresorptive exposure before dental surgery; imaging should answer a specific clinical question.
What causes fibrous dysplasia and McCune-Albright syndrome?
A postzygotic activating GNAS variant causes mosaic disease. Fibrous dysplasia replaces normal bone with fibro-osseous tissue and may affect one or multiple bones. McCune-Albright manifestations include characteristic café-au-lait pigmentation and endocrine hyperfunction, such as precocious puberty, hyperthyroidism, or growth hormone excess.
Which systemic findings affect dental planning in McCune-Albright syndrome?
Fractures, deformity, scoliosis, pain, hypophosphatemia, and endocrine disease may occur. Craniofacial lesions can affect hearing, vision, and facial symmetry. Current hormone control and bone medication history matter more than a label of overgrowth alone.
What oral findings occur with craniofacial fibrous dysplasia?
Jaw expansion, asymmetry, tooth displacement, malocclusion, and a ground-glass radiographic appearance may occur. Altered tooth number, enamel, or pulp anatomy is reported in some patients. Rapid growth, new pain, sensory change, or functional deterioration requires reassessment rather than attributing every change to known dysplasia.
How should dental treatment be planned in fibrous dysplasia or McCune-Albright syndrome?
- Define the lesion: review location, anatomy, imaging, and craniofacial-team assessment.
- Surgery: plan extraction or other invasive care around affected bone, adjacent structures, and healing risk.
- Medication: review bisphosphonate or other antiresorptive exposure and coordinate risk assessment.
- Endocrine status: uncontrolled hyperfunction or other significant disease changes procedural planning.
- Imaging and orthodontics: use clinically justified imaging; cone-beam computed tomography is not mandatory for every extraction. Discuss tooth movement, growth, and relapse risk with the specialty team.
What treatment and prognosis apply to McCune-Albright syndrome?
Care addresses fractures and deformity, endocrine disease, pain, and functional craniofacial problems. Antiresorptives may be used for selected bone pain, but should not be presented as proven prevention of all fractures or lesion progression. Disease burden varies; many patients have a normal lifespan with continuing specialist care.