Treatment of Fibrous Dysplasia of Bone with Craniofacial Involvement and Functional Impairment
When fibrous dysplasia affects the craniofacial skeleton and causes functional impairment or significant physical disfigurement, a specialist surgical approach is required. The key clinical goals are prevention of functional loss — particularly hearing and vision — arrest or reduction of physical disfigurement, prevention of secondary deformity, and minimisation of long-term morbidity.
Clinical Scenario
Craniofacial fibrous dysplasia (CFFD) with functional impairment or significant physical disfigurement. Patients identified with CFFD at baseline or at subsequent monitoring evaluations should be referred for formal assessment to a craniofacial service with experience in the care of this condition.
Treatment Goals
- Prevention of functional loss — especially hearing and vision
- Arrest or reduction of physical disfigurement
- Prevention of secondary deformity
- Minimisation of long-term morbidity from CFFD and its treatment
Approach (partial overview)
Management is surgical, delivered by a multidisciplinary craniofacial team. The approach may involve lesion bulk reduction for symmetry, or more extensive excision with reconstruction — selected according to disease extent and functional impact. Advanced planning technologies, including 3-dimensional imaging analysis and computer-aided design, are considered standard of care… the complete protocol specifies the full algorithm, indications, and surgical options.
References
DOI: 10.1186/s13023-019-1102-9
- The goals of treatment are: a) Prevention of functional loss – especially hearing and vision; b) Arrest or reduction of physical disfigurement; c) Prevention of secondary deformity; d) Minimisation of long-term morbidity from CFFD and its treatment.
- If CFFD is identified at baseline or at subsequent monitoring evaluations, the patient should be referred for a formal assessment to a craniofacial service with experience in the care of patients with CFFD.
- Treatment options include: burring of the lesion to reduce bulk and achieve symmetry; subtotal excision and reconstruction; complete excision of the lesion and reconstruction of the calvarial defect.
- Advanced imaging techniques and 3-dimensional analysis of scans together with virtual surgical planning and computer-aided manufacturing and design of patient-specific implants should be regarded as the standard of care in surgery of FD of the craniofacial skeleton.
- Simple curettage is not recommended as it is ineffective and may increase the risk of complications.
View source ↗