Craniofacial polyostotic fibrous dysplasia

Area of Science:

• Craniofacial Surgery
• Oral and Maxillofacial Surgery
• Skeletal Dysplasias

Background:

• Fibrous dysplasia is a benign bone disorder where normal bone is replaced by fibrous tissue.
• Polyostotic fibrous dysplasia affects multiple bones, and when involving the craniofacial skeleton, presents significant management challenges.
• Gross craniofacial polyostotic fibrous dysplasia requires careful consideration due to potential for extensive bone involvement and functional compromise.

Observation:

• Presents three cases of gross craniofacial polyostotic fibrous dysplasia.
• Highlights the challenges associated with complete surgical excision and immediate reconstruction in growing patients.
• Discusses the potential for accelerated lesion growth following partial excision during the active growth phase.

Findings:

• Advocates for conservative surgical resection aimed at protecting or restoring essential functions (e.g., vision) during the patient's growth period.
• Emphasizes that complete excision is often not feasible or possible in the initial stages.
• Suggests that partial excision can lead to increased lesion activity in pediatric and adolescent patients.

Implications:

• Recommends a phased surgical approach: initial functional preservation followed by definitive procedures after lesion quiescence.
• Stresses the importance of close, long-term follow-up to monitor lesion activity.
• Informs surgical strategies for managing craniofacial polyostotic fibrous dysplasia, prioritizing functional outcomes and long-term control.