Coexistence of meningioma and craniofacial fibrous dysplasia: a case series of clinicopathological study and literature review.

BACKGROUND: The co-existence of meningioma and craniofacial fibrous dysplasia (CFD) is rare. Due to the similar radiological characteristics, it is challenging to differentiate such co-existence from solitary hyperostotic meningioma resulting in a dilemma of prompt diagnosis and appropriate intervention. METHOD: We conducted a retrospective review of the data from 21 patients with concomitant meningioma and CFD who were treated at Beijing Tiantan Hospital from 2003 to 2021. We summarized their clinicopathological features and performed a comprehensive literature review. Additionally, we tested the characteristic pathogenic variants in exon 8 and 9 of GNAS gene and the expression of corresponding α-subunit of the stimulatory G protein (Gαs) related to CFD to explore the potential interactions between these two diseases. RESULTS: The cohort comprised 4 men and 17 women (mean age, 45.14 years). CFD most commonly involved the sphenoid bone (n = 10) and meningiomas were predominantly located at the skull base (n = 12). Surgical treatment was performed in 4 CFD lesions and 14 meningiomas. Simpson grade I-II resection was achieved in 12 out of the 14 resected meningiomas and almost all of them were classified as WHO I grade (n = 13). The mean follow-up duration was 56.89 months and recurrence was noticed in 2 cases. Genetic study was conducted in 7 tumor specimens and immunohistochemistry was accomplished in 8 samples showing that though GNAS variant was not detected, Gαs protein were positively expressed in different degrees. CONCLUSIONS: We presented an uncommon case series of co-diagnosed meningioma and CFD and provided a detailed description of its clinicopathological features, treatment strategy and prognosis. Although a definite causative relationship had not been established, possible genetic or environmental interplay between these two diseases could not be excluded. It was challenging to initiate prompt diagnosis and appropriate treatment for concomitant meningioma and CFD because of its similar radiological manifestations to meningioma with reactive hyperostosis. Personalized and multi-disciplinary management strategies should be adopted for the co-existence of meningioma and CFD.

Craniofacial Fibrous Dysplasia in Fronto-Orbital Region: A Single-Center Retrospective Study of 38 Cases.

OBJECTIVE: This study presents the clinical characteristics, imaging manifestations, and surgical experience in 38 patients diagnosed with craniofacial fibrous dysplasia in fronto-orbital region (foFD). METHODS: We retrospectively analyzed the clinical data from 38 patients who had surgery for foFD. The surgical procedure typically involved extensive tumor removal, followed by immediate reconstruction of the frontal bone and orbit using synthetic materials. Additionally, 9 patients underwent simultaneous microscopic decompression of the optic canal. RESULTS: Common clinical manifestations included progressive fronto-orbital bone deformity (35), proptosis (28), orbital dystopia (21), and visual impairment (9). The disease primarily affecting the frontal bone (38), the sphenoid bone (28), and the ethmoid bone (24). The optic canal was involved in 9 patients with functional impairment. Computed tomography scans in all 38 cases revealed satisfactory repair material positioning and complete resolution of frontal deformities. Among the 9 patients who underwent optic canal decompression, 7 experienced partial recovery of visual acuity after surgery. CONCLUSIONS: In the surgical treatment of foFD, it is crucial to achieve maximal bone resection and repair skull defects, while decompressing the optic canal can provide significant benefits for patients with decreased visual function preoperatively. The use of preformed artificial materials offers advantages in aesthetic restoration after lesion excision.

RANK-L inhibitor as a promising agent for refractory extensive craniofacial fibrous dysplasia: A case report.

BACKGROUND: McCune-Albright syndrome is a rare disorder characterized by polyostotic fibrous dysplasia (FD), café-au-lait skin pigmentation, and endocrine dysfunction. Extensive FD in the craniofacial region can present significant challenges in terms of disease control and carries a high risk of permanent visual impairment. METHODS: We present a case of medically and surgically resistant FD that required nine optic nerve decompressions. RESULTS: The condition was ultimately controlled with the use of the denosumab agent. CONCLUSION: The case highlights the importance and potential efficacy of denosumab in resistant FD management, particularly in cases involving sensitive organs.

Clínica, epidemiología e imagenología del fibroma osificante y la displasia fibrosa del territorio cráneo-maxilofacial: revisión narrativa / Clinical, epidemiology and imaging of ossifiying fibroma and fibrous dysplasia of the cranio-maxillofacial territory: narrative review

Introducción: El fibroma osificante (FO) y la displasia fibrosa (DF) tienen características imagenológicas e histopatológicas similares que dificultan el diagnóstico diferencial. El propósito de la presente revisión narrativa es analizar las características clínicas, epidemiológicas e imagenológicas del FO y la DF, y evaluar la relación entre las características imagenológicas y las variantes histopatológicas del FO, en reportes y series de casos publicados.Materiales y métodos:Se realizó una búsqueda de reportes y series de casos de FO y DF entre 2017-2021 en PubMed, Scopus y Web of Science. Los casos debían tener suficiente información clínica, epidemiológica, histopatológica e imagenológica.Resultados:Se incluyeron 23 artículos con 25 lesiones: 17 FO y 8 DF. El tiempo de evolución de DF es más prolongado que FO. FO se ubica con mayor tendencia en complejo osteomeatal en comparación con DF. Sólo FO presentó alteraciones en piel, radiolucidez/hipodensidad periférica, perforación ósea y rizalisis externa, además, tuvo mayor tendencia al avance a espacios anatómicos adyacentes y a generar asimetría facial y/o craneal. FO psammomatoide y FO trabecular comparten patrones imagenológicos y tienen límites definidos corticalizados. FO convencional tuvo mayor tendencia a perforar corticales. DF puede tener límites mal definidos y definidos corticalizados. El FO mostró características imagenológicas de mayor agresividad que la DF.Conclusiones:La histología e imagenología por sí solas no son decisivas en el diagnóstico de FO y DF. Es imprescindible considerar conjuntamente clínica, imagenología e histopatología, enfatizando en las características que orienten el diagnóstico diferencial.(AU)

Introduction: Ossifying fibroma (OF) and fibrous dysplasia (FD) have similar imaging and histopathological characteristics, which make differential diagnosis difficult. The aim of this narrative review was to analyze the clinical, epidemiological and imaging characteristics of OF and FD, as well as to evaluate the relationship between imaging characteristics and histopathologic variants of OF, in reports and case series published.Materials and methods:A search of reports and case series of OF and FD between 2017-2021 in PubMed, Scopus and Web of Science was performed. The cases had to have enough clinical, epidemiological, histopathological and imaging information.Results:23 articles with 25 lesions were included: 17 OF and 8 FD. FD had a longer time of evolution than OF. OF is more likely to be in osteomeatal complex compared to FD. Only OF had skin alterations, peripheral radiolucency/hypodensity, bone perforation and external root resorption, in addition, it had a greater tendency to advance to adjacent anatomical spaces and generate facial and/or cranial asymmetry. Psammomatoid OF and trabecular OF share imaging patterns and have defined and corticated margins. Conventional OF had a greater tendency to cortical perforation. DF can have ill-defined and defined and corticated margins. OF has imaging characteristics of greater aggressiveness than FD.Conclusion:Histology and imaging alone are not decisive in OF and FD diagnosis. It is essential to consider clinical, imaging and histopathological evaluations as a whole, emphasizing in the characteristics that guide the differential diagnosis.(AU)

Craniofacial fibrous dysplasia: Systematic review of facial management.

Craniofacial fibrous dysplasia (CFD) may be associated with major cosmetic or functional consequences. However, management recommendations for CFD are currently unavailable. Therefore, this systematic literature review aimed to review the existing approaches for CFD management and propose a management algorithm. The focus question was "What are the different options for CFD treatment and their complication rates?" The MEDLINE database was searched, and 33 articles evaluating a total of 1154 patients were reviewed. The bias assessment showed that 20 of the 33 studies had a high or intermediate risk of bias, mainly because of retrospective data collection and small patient numbers. Radical surgery showed a lower recurrence rate than debulking, but its use should be weighed against the morbidity caused by the reconstruction performed in this technique. Orbital decompression using a radical technique or debulking is effective in cases showing exophthalmos or dystopia. Surveillance is a viable option for asymptomatic and/or non-progressive lesions. In cases showing optic nerve compression, prophylactic decompression should be avoided, and decompression should be performed only when patients show diminished visual acuity or visual field defect. Although bisphosphonates have shown efficacy in pain management, their posology requires further discussion. A management algorithm is presented.

Long-term Outcomes Following Endoscopic Transnasal Surgery for Optic Neuropathy Due to Craniofacial Fibrous Dysplasia.

OBJECTIVE: To investigate the feasibility of endoscopic transnasal optic canal decompression (ETOCD) guided by a navigation surgical system (NSS) for vision recovery in patients with compressive optic neuropathy (CON) caused by craniofacial fibrous dysplasia (CFD), and to explore the underlying cause of visual impairment. METHODS: All patients underwent unilateral NSS-guided ETOCD and were followed up periodically for at least six months. Paired sample t-test and Pearson correlation analyses were used to compare continuous variables of the visual outcomes at the final review. A histopathological test of abnormal bone specimens was performed postoperatively. RESULTS: Thirty-four patients were finally included, and all surgeries were uneventful. The best corrected visual acuity (BCVA) (logMAR units) decreased from 1.29 ± 0.80 preoperatively to 0.97 ± 0.78 at the last follow-up (p = 0.0012), improving in 28 patients (82.35%). The absolute value of mean defect (MD) significantly decreased (p < 0.001). Color vision was impaired in 17 patients preoperatively and improved in 6 patients. BCVA at the last follow-up was significantly correlated with preoperative BCVA, onset time, preoperative retinal nerve fibril layer thickness, and MD (all p < 0.05). Among 34 patients, 26 had a blunt bony process near the anterior foot of the optic chiasm. Of the total patients, 73.53% patients experienced bony fiber recurrence 6 months or earlier after surgery without visual loss. CONCLUSION: NSS-guided ETOCD appeared to be safe and effective for visual recovery in patients with CON due to CFD, and early surgical intervention was critical for long-term recovery. Unbalanced compression of the optic canal by the blunt bony process may be a major cause of visual impairment. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1857-1866, 2023.

Computer-guided contouring of craniofacial fibrous dysplasia involving fronto-orbital or fronto-cranial region using patient specific surgical depth guide: A prospective case series.

The aim of this study was to introduce a new computer guided technique for debulking and contouring the craniofacial fibrous dysplasia involving the fronto-orbital and fronto-cranial regions. Computer-guided contouring was performed using a modified patient-specific surgical depth guide for six patients with craniofacial fibrous dysplasia involving the fronto-orbital and fronto-cranial regions. Virtual planning was performed to determine the desired amount of bone removal and construct the patient-specific surgical depth guide. Then, the guide was printed using rapid prototyping. In the surgical theatre, the guide was seated in position. Implant drills were inserted through the created depth holes according to the planned fixed depth to create depth holes. Finally, the bone in between the created holes was removed using cutting discs, bone chisels and surgical burs. Satisfaction with facial aesthetics was evaluated by the patients using a Likert scale, and by the surgeons using the Whitaker rating scale. The surgical procedures were uneventful for all the patients. All the patients were satisfied with the post-operative facial esthetics and categorized as category I Whitaker rating scale. Patient-specific surgical guide technique for recontouring of fronto-orbital and fronto-cranial fibrous dysplasia can be considered an accurate substitution technique that overcomes the drawbacks of the unpredictable conventional one. Further investigations are required.

PTHrP Modulates the Proliferation and Osteogenic Differentiation of Craniofacial Fibrous Dysplasia-Derived BMSCs.

Fibrous dysplasia (FD) is a skeletal stem cell disease caused by mutations in the guanine nucleotide-binding protein, alpha-stimulating activity polypeptide (GNAS) gene, which results in the abnormal accumulation of cyclic adenosine monophosphate (cAMP) and hyperactivation of downstream signaling pathways. Parathyroid hormone-related protein (PTHrP) is secreted by the osteoblast lineage and is involved in various physiological and pathological activities of bone. However, the association between the abnormal expression of PTHrP and FD, as well as its underlying mechanism, remains unclear. In this study, we discovered that FD patient-derived bone marrow stromal cells (FD BMSCs) expressed significantly higher levels of PTHrP during osteogenic differentiation and exhibited greater proliferation capacity but impaired osteogenic ability compared to normal control patient-derived BMSCs (NC BMSCs). Continuous exogenous PTHrP exposure on the NC BMSCs promoted the FD phenotype in both in vitro and in vivo experiments. Through the PTHrP/cAMP/PKA axis, PTHrP could partially influence the proliferation and osteogenesis capacity of FD BMSCs via the overactivation of the Wnt/ß-Catenin signaling pathway. Furthermore, PTHrP not only directly modulated cAMP/PKA/CREB transduction but was also demonstrated as a transcriptional target of CREB. This study provides novel insight into the possible pathogenesis involved in the FD phenotype and enhances the understanding of its molecular signaling pathways, offering theoretical evidence for the feasibility of potential therapeutic targets for FD.

Frequency of spontaneous osteosarcomatous transformation of craniofacial fibrous dysplasia: a systematic review.

OBJECTIVE: A systematic review was undertaken to examine the spontaneous of craniofacial fibrous dysplasia (FD) into osteosarcoma (OS). STUDY DESIGN: Five databases were searched, and data were evaluated in 2 subsets: (1) frequency analysis involving only cross-sectional studies that specifically examined patients for osteosarcomatous transformation, thereby allowing a calculation of the frequency of transformation of craniofacial FD, and (2) case analysis of case reports and case series of FD that underwent osteosarcomatous transformation. Quality assessment of the studies in the frequency and case analyses was performed. RESULTS: Seven cross-sectional studies involving 779 patients in the frequency analysis documented transformation of craniofacial FD into OS in 13 patients, for a frequency of 1.67%. Of the 18 separate cases of osteosarcomatous transformation in the case analysis, 11 (61.1%) occurred in females, 11 (61.1%) developed from monostotic FD, and 7 (38.9%) arose in the mandible, with 5 in the maxilla (27.8%) and 6 (33.3%) in other craniofacial sites. The mean age of the patients at the time of malignant diagnosis was 36.4 years, with an average of 11.3 years from FD diagnosis to the appearance of OS. The quality of the articles ranged from fair to excellent. CONCLUSIONS: The risk of osteosarcomatous transformation of craniofacial FD is low, but the possibility of transformation should be taken into consideration by clinicians, as this will guide treatment and follow-up regimens.

[Aesthetic rehabilitation of a case of maxillary fibrous dysplasia in a child]. / Réhabilitation esthétique d'un cas de dysplasie fibreuse maxillaire chez l'enfant.

Fibrous dysplasia (FD) is a rare, congenital, benign bone disorder in which healthy bone tissue is replaced by abnormal scar-like (fibrous) connective tissue. The lesions may affect one or more bones, including the jawbones. A 13-year-old girl visited the dentist for failed eruption of her left maxillary permanent teeth and facial asymmetry. Radiological analysis revealed impactions of the permanent teeth in hypertrophic, hyperdense, weakly trabeculated bone tissue. To restore the aesthetics of the smile, a treatment combining decoronation and bonded restorations was performed. FD enlargement would slow down after puberty, allowing for long-term therapies. But there is no information about implantology in this type of bone lesion. The aesthetic and functional consequences of a maxillary FD should be managed as soon as possible. Treatment involves a multidisciplinary team and follow-up care into adulthood.

Craniofacial Fibrous Dysplasia: Clinical and Therapeutic Implications.

PURPOSE OF REVIEW: This study aims to review diagnosis, potential complications, and clinical management in craniofacial fibrous dysplasia. RECENT FINDINGS: Fibrous dysplasia (FD) is a rare mosaic disorder in which normal bone and marrow are replaced with expansile fibro-osseous lesions. Disease presents along a broad spectrum and may be associated with extraskeletal features as part of McCune-Albright syndrome (MAS). The craniofacial skeleton is one of the most commonly impacted areas in FD, and its functional and anatomical complexities create unique challenges for diagnosis and management. This review summarizes current approaches to diagnosis and management in FD/MAS, with emphasis on the clinical and therapeutic implications for the craniofacial skeleton.

Severe craniofacial fibrous dysplasia associated with decreased visual acuity: a case report.

Fibrous dysplasia is a developmental abnormality characterized by the replacement of normal bone tissue by fibrous connective tissue with poorly organized bone trabeculae. This disorder rarely occurs in the craniofacial region, but in such cases it causes facial asymmetries and has severe clinical implications for the patient. This case report describes the treatment of an 18-year-old man who presented with complaints of facial deformity and decreased visual acuity. Cone beam computed tomography revealed a diffuse bone lesion affecting the region of the maxillary, frontal, and nasal bones on the left side of the face. After microscopic examination, the diagnosis of craniofacial fibrous dysplasia was made. The patient underwent a bilateral temporal craniotomy to perform decompression of the orbital apices and correct the loss of visual acuity. In addition, surgical cosmetic contouring of the facial bones was performed. The patient has been followed up by a multidisciplinary team; at his most recent examination, 18 months after the last surgical intervention, his clinical condition remained stable.

A Novel Bone Contouring Technique Using Multiple Tangential Shaving for Conservative Management of Craniofacial Fibrous Dysplasia.

This study aimed to propose a novel surgical technique, named multiple tangential shaving of bone contour, for the conservative management of craniofacial fibrous dysplasia. We retrospectively reviewed 17 patients who underwent conservative management of craniofacial fibrous dysplasia using multiple tangential shaving technique between July 2005 and December 2020. Demographics, tumor characteristics, and surgery-related factors were investigated. All patients underwent preoperative (T0) and postoperative computed tomography scans taken at least twice within 1 month for immediate assessment (T1) and at least 12 months postoperatively for long-term assessment (T2). Clinical outcomes, including tumor recurrence, perioperative complications, and physician measure of esthetic outcomes (Whitaker score), were investigated. This technique was applied for contouring of the zygomatic-maxillary and calvarial bone for patients aged between 16 and 60 years (mean age: 26 y). The mean±SD tumor volume reduction was 15.5±8.95 cm 3 , and the postoperative mean±SD tumor growth rate was 5.52±6.26% per year. Satisfactory outcome was obtained in terms of esthetics with a mean±SD Whitaker score of 1.41±0.62. Patients required a mean operation time of 1.67±0.43 hours and a mean number of shaving operations of 1.35±0.61 during the follow-up period. Five of 17 patients required reoperation because of the tumor recurrence (N=4) and to correct new-onset diplopia after surgery (N=1). In conclusion, the multiple tangential shaving technique allows an easy approach for conservative management of craniofacial fibrous dysplasia. An acceptable rate of tumor recurrence and esthetic outcomes can be obtained by selecting the appropriate candidate for a conservative approach.

A Novel Technique for the Correction of Unilateral Craniofacial Fibrous Dysplasia Using Multiplanar Sequential Cutting Guides.

In this unique case report, the authors have described a new method for the correction of unilateral craniofacial fibrous dysplasia by using sequential cutting guides. Due to the complex 3-dimensional anatomy of zygoma, it needs to be chiseled in multiple planes to mimic the normal contralateral side. To achieve this, 3 different guides were used one after the other to perform osteotomies in different planes and remove the excess fibrous bone.

Pearls & Oy-sters: Sequential Bilateral Hearing and Vision Loss With Optic Disc Swelling Due to Sphenoid Bone Craniofacial Fibrous Dysplasia.

Bilateral optic disc swelling is a common finding but rarely associated with multiple cranial neuropathies. In this case, an 18-year-old man presented with subacute sequential hearing loss followed by subacute sequential visual loss. Clinical examination revealed bilateral optic disc swelling. Lumbar puncture revealed a normal opening pressure of 15 cmH2o. This case discusses a rare but important cause of bilateral optic disc swelling in the context of hearing loss, disequilibrium, and a normal CSF opening pressure. An overview of the literature is provided, and treatment options are discussed to guide further management of similar cases.

Comparison of the histopathological characteristics of diffuse sclerosing osteomyelitis of the mandible, chronic suppurative osteomyelitis, and craniofacial fibrous dysplasia.

BACKGROUND: There are relatively few reports on the histopathological characteristics of diffuse sclerosing osteomyelitis of the mandible (DSOM), which is difficult to distinguish from chronic suppurative osteomyelitis (CSO) and craniofacial fibrous dysplasia (CFD). This study aimed to summarize and compare the histopathological characteristics of DSOM, CFD, and CSO. MATERIALS AND METHODS: In this study, hematoxylin and eosin-stained sections of patients with DSOM, CSO, and CFD at the Peking University Hospital of Stomatology from 2015 to 2020 were retrieved. The histopathological characteristics were summarized, including new bone formation, inflammatory cell infiltration, bone trabecular morphology, osteoclasts, sequestrum, bacterial mass, and calcified spherules, similar to cementicles. The histopathological characteristics of DSOM, CSO, and CFD were compared, and the results were statistically analyzed. RESULTS: In total, 50, 13, and 10 patients with DSOM, CSO, and CFD were included in this study, respectively. In terms of new bone formation, both DSOM and CSO showed reactive bone formation (p = 1), whereas CFD mainly showed fiber osteogenesis (p < 0.001). The inflammatory cells of DSOM were mainly lymphocytes and plasma cells, whereas those of CSO were mainly lymphocytes and neutrophils (p < 0.001), and there was usually no inflammatory cell infiltration in the CFD specimens (p < 0.001). DSOM, CSO, and CFD showed irregular bone trabeculae (p = 0.045, p = 0.703) and active osteoclasts (p1 = 0.189, p2 = 0.256). DSOM showed a small amount of bacterial mass but no sequestrum; neither of which was found in CFD (p = 1, p = 1), but it was common in CSO (p = 0.011 and p = 0.025). DSOM and CSO showed smooth and regular basophilic lines (p = 0.308), whereas CFD showed a rough and irregular basophilic line (p < 0.001). CONCLUSIONS: The histopathological characteristics of the three diseases were partly similar, but there were evident differences. The main differences are the type of new bone formation, types and distribution of inflammatory cells, and presence of sequestrum and bacterial masses. These differences will help clinicians diagnose DSOM.

Malignant Sarcomatous Degeneration of Craniofacial Fibrous Dysplasia.

BACKGROUND: Fibrous dysplasia (FD) is an uncommon bone disease characterized by the replacement of normal bone architecture with abnormal fibro-osseous connective tissue. Here, we discuss 2 cases of craniofacial FD, with malignant sarcomatous degeneration - a rare and morbid complication of the disease. CASE HISTORY: Two cases of craniofacial FD with malignant degeneration are presented. In the first, a 68-year-old male with a history of FD presented with acutely worsening left-sided facial pain and V2 and V3 hypoesthesia. Imaging findings suggested a large infratemporal fossa mass with biopsy demonstrating sarcomatous degeneration. Radical craniofacial resection achieved a gross total resection with likely microscopic disease. The patient was unable to tolerate adjuvant chemotherapy or radiation and succumbed to his disease 13 months following surgery.In the second case, a 36-year-old male with McCune-Albright Syndrome and craniofacial FD presented with acutely worsening left-sided headaches and midface hypoesthesia. Imaging revealed a heterogenous and expansile lesion with erosive changes in the left nasal cavity and infratemporal fossa. Pathology was suggestive of low grade sarcomatous degeneration. Given the extensive involvement of the skull base, the tumor was deemed unresectable, and the patient soon died following initiation of chemotherapy. CLINICAL RELEVANCE: Malignant sarcomatous transformation is a rare and challenging complication of craniofacial FD. Indolent onset, advanced spread at time of presentation, and close relationship with vital neurovascular structures are all hurdles for the treating clinician. The entity poses a diagnostic dilemma, as pathological analysis can be equivocal and may mimic nonmalignant processes, such as locally aggressive FD.

Craniofacial Fibrous Dysplasia of the Skull Assisted by Virtual Surgical Planning.

Craniofacial fibrous dysplasia (FD) involves thickening of the skull and facial bones, causing asymmetry and distortion of overlying soft tissues. Surgical contouring is often performed with rotary bur or osteotome, with the goal of matching contralateral unaffected anatomy. This is made technically challenging by having no direct visualization of contralateral structures, and the desire to control depth of resection to match the contour of the unaffected side. In our report, a 13-year-old male presented for surgical evaluation of craniofacial FD affecting the right parietal/temporal bones. A novel virtual surgical planning approach of premade drilling template with numerous pilot guide holes was used to assist bone debulking. The pilot holes allowed precise burring of the dysplastic bone. The patient achieved excellent calvarial contour symmetry without unintended intracranial extension. We believe that virtual surgical planning and drilling depth guides are effective tools in the reconstruction of craniofacial FD.

Surgical management of syndromic versus non-syndromic craniofacial fibrous dysplasia: a systematic review and meta-analysis.

The main purpose of this study was to identify an algorithm for the surgical management of fibrous dysplasia in syndromic (McCune-Albright syndrome) and non-syndromic patients (monostotic and polyostotic subtypes). The secondary objectives were to assess the prevalence of affected craniofacial bones and the main clinical presentation. The authors performed a systematic review and meta-analysis by conducting a comprehensive electronic search from 1 January 2000 to 31 December 2019. A total of 1260 patients were included. The maxilla was the most affected facial bone (41%) (p<0.001, CI 38.3 to 43.8) and facial asymmetry was the chief complaint (p<0.001, CI 31.7 to 37.1). Conservative surgery registered higher recurrence rates than radical resection in both syndromic (84%) (p<0.001, CI 70.9 to 92.8) and non-syndromic patients (26%) (p<0.001, CI 21.8 to 30.6). Compared with prophylactic decompression, therapeutic optic nerve decompression (OND) showed better postoperative outcomes in both syndromic (p=0.9, CI 18.6 to 55.9) and non-syndromic patients (p=0.09, CI 9.3 to 28.4). Watchful waiting showed excellent results in both subgroups when asymptomatic (p<0.001). Syndromic and non-syndromic patients share the same treatment strategies. Radical resection is the preferred surgical technique to eradicate the disease, but it is often difficult to perform due to the extent and location of the disease. Furthermore, the authors advise early therapeutic over prophylactic OND to prevent optic nerve atrophy. Asymptomatic patients should be managed expectantly. Finally, medical management helps reduce the symptoms of bone pain (p=0.02 in non-syndromic and p<0.001 in syndromic patients).