Treatment Protocol and Long-Term Outcomes in Craniofacial Fibrous Dysplasia

Study Overview
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Brief Summary
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Craniofacial fibrous dysplasia (CFD) is a nonneoplastic disease characterized by fibro-osseous lesions in the affected bones. Treatment is mainly surgical and is stratified based on four different anatomical zones. This study aimed to evaluate the long-term outcomes and refine the algorithm for CFD.

Detailed Description
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According to Chen et. al for the appropriate surgical management of FD the craniofacial skeleton can be divided into four zones according to the consideration of facial cosmesis, vital anatomical region and function preserving. The aim of the study was to evaluate the outcome of nearly 40 years long-term follow up cohort group and refine the algorithm of surgical intervention of FD under 4 zones classification.

Official Title
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Long-term Follow-up and Treatment Protocol for Craniofacial Fibrous Dysplasia From 40-year Data at Chang Gung Memorial Hospital: A Retrospective Case Series

Conditions
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Craniofacial Fibrous Dysplasia

Intervention / Treatment
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* Procedure: Craniofacial reconstruction


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Received CFD surgical intervention between 1972 and 2019 Personal interviews: between late 2019 and 2020, covering a postoperative period of over 20 years All the surgery were performed by a senior surgeon (Yu-Ray Chen) at Chang Gung Craniofacial Center Exclusion Criteria: Patients who underwent surgical treatment at other institutions during the follow-up period or those who did not return to the craniofacial outpatient department for follow-up and receive computed tomography scans between 2019 and 2020 will be excluded.
Sexes Eligible for Study
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All

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Craniofacial fibrous dysplasia<br>Fibro-osseous lesions within the affected bones based on image, and surgical intervention based on 4 different anatomical zones. | Procedure: Craniofacial reconstruction<br>* Zone 1 lesions: radical excision and immediate reconstruction with bone grafts for fibrous dysplasia lesions. Zone 2 lesions: trimming & recontouring. Zone 3 lesions: observation or conservative recontouring; optic nerve decompression performed when continuous deterioration of vision, and external auditory canal surgery performed when cholesteatoma/near total ear canal stenosis. Zone 4: observation or conservative recontouring; orthognathic surgery performed when malocclusion or midface retrusion noted.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Recurrence | Recurrence based on facial bone computed tomography | Personal interviews with facial bone computed tomography obtained on late 2019 and 2020 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Craniofacial fibrous dysplasia, Optic nerve decompression, Hormone therapy