[Fronto-ethmoidal fibrous dysplasia: a case-report]

Area of Science:

• Orthopedics
• Genetics
• Pathology

Background:

• Fibrous dysplasia is a congenital bone disorder characterized by the replacement of normal bone with fibrous tissue.
• Craniofacial involvement occurs in approximately one-third of cases, necessitating specialized evaluation.
• Understanding the pathogenesis and clinical spectrum is crucial for effective management.

Observation:

• A case of isolated fibrous dysplasia in the paranasal sinuses presented with headache and palpebral edema in a 12-year-old.
• Imaging revealed a fronto-ethmoidal lesion with epidural empyema, without other systemic abnormalities.
• The patient's condition improved with intravenous antibiotics, indicating a favorable response to medical management.

Findings:

• Computed tomography (CT) scans are pivotal for diagnosing fibrous dysplasia, often obviating the need for biopsy.
• Initial workup should include axial skeleton X-rays and hormonal assessments to rule out multifocal disease or associated endocrine abnormalities.
• Biphosphonate therapy has shown efficacy in reducing the need for surgical interventions.

Implications:

• Fibrous dysplasia is frequently a multifocal condition, underscoring the importance of a comprehensive diagnostic approach.
• Medical management, particularly with biphosphonates, is the preferred initial treatment strategy.
• Optimal patient outcomes are achieved through a collaborative, multidisciplinary team approach involving various medical specialists.