[Craniofacial fibrous dysplasia: about six cases]

Area of Science:

• Medical Sciences
• Pathology
• Genetics

Background:

• Fibrous dysplasia (FD) of bone is a rare, benign congenital disorder characterized by the replacement of normal bone with fibrous tissue.
• This pathological process can lead to significant bone deformities, affecting any bone in the body.
• Craniofacial fibrous dysplasia (cFD) presents distinct clinical manifestations, progression patterns, and therapeutic challenges compared to FD in other skeletal sites.

Observation:

• This study retrospectively analyzed six patients with craniofacial FD treated at the Principal Hospital of Dakar.
• The patient cohort had an average age of 26.16 years (range: 11–58 years), with a strong female predominance (83%).
• The primary clinical presentation leading to diagnosis was bone deformity, with one patient experiencing unilateral nasal obstruction and epistaxis.

Findings:

• Computed tomography (CT) scans were instrumental in diagnosing craniofacial FD and assessing its topographic extent in all patients.
• Surgical intervention was performed in two female patients.
• A single case of recurrence was documented within the study group.

Implications:

• Craniofacial FD is a rare condition with the potential to cause severe sensory and functional impairments.
• Effective management of cFD necessitates a comprehensive, interdisciplinary approach due to its complex therapeutic landscape.
• Further research into the pathogenesis and long-term outcomes of cFD is warranted to optimize patient care.