Contemporary Surgical Management of Craniofacial Fibrous Dysplasia Using Computer-Assisted Surgery and Intraoperative Navigation.

Craniofacial fibrous dysplasia (CFD) is a rare developmental disease of bone, which typically presents as a painless, expansile mass causing deformity of the craniofacial skeleton. In rare circumstances, compression of neurovascular structures may arise, causing symptoms such as pain, visual impairment, and hearing loss. Traditionally, CFD debulking has been performed with "freehand" techniques using preoperative imaging and anthropometric norms to determine the ideal amount of tissue removal. The advent of computer-assisted surgery, computer-aided design, and computer-aided manufacturing (CAD/CAM) has revolutionized the management of CFD. Surgeons can now fabricate patient-specific osteotomy/ostectomy guides, allowing for increased accuracy in bone removal and improved cosmetic outcomes. This series of 3 cases describe our institution's technique using patient-specific ostectomy "depth guides", which allow for maximum removal of fibro-osseous tissue while sparing deep and adjacent critical structures. These techniques can be widely applied to the craniofacial skeleton to assist in the surgical management of CFD.

Craniofacial Fibrous Dysplasia: Experience at San José Hospital, Bogotá, Colombia.

INTRODUCTION: Fibrous dysplasia is a disorder in which normal bone is gradually replaced by immature fibro-osseous tissue, with an incidence of less than 7% of all benign bone tumors. The management of this disease is a challenge for plastic surgeons and neurosurgeons. GOAL: To describe the diagnostic, therapeutic, and outcome approach of patients with craniofacial fibrous dysplasia seen at the Plastic Surgery Service of the Hospital San José in Bogotá, Colombia. METHODS: This is a descriptive and retrospective case series study of patients diagnosed with monostotic and polyostotic fibrous dysplasia treated at the Plastic Surgery Department of Hospital San José during the period from January 1, 2010, to July 31, 2023. RESULTS: All (n=10) of the patients had monostotic craniofacial fibrous dysplasia. The most affected bones in patients with monostotic fibrous dysplasia were zone I bones (n=10, 100%), followed by zone II bones (n=2, 20%). Patients with zone I and II involvement manifested throbbing headaches associated with phosphenes and tinnitus (n=8, 80%) and pain during occlusion associated with edema in the affected cheek (n=5, 50%). Physical examination showed that patients with orbital wall involvement (zone I bone) had ocular dystopia (n=7, 70%).Regarding the treatment received by the patients, 90% (n=9) of the patients received surgical management as primary treatment, with orbitotomy, replacement, and/or remodeling of the roof and lateral wall of the orbit with bone graft, drilling, canthoplasty, ciliary suspension being the most frequently performed procedure (n=6, 60%). Of the patients, 20% (n=2) required reintervention. CONCLUSIONS: FD is a slowly progressive benign fibro-osseous disease that requires a timely, individualized, and multidisciplinary diagnosis and treatment to obtain favorable clinical and surgical results.The mainstay of treatment is surgery as a preventive measure since it is important to avoid future functional alterations that, depending on the location of the dysplasia, would cause a high risk of alteration of adjacent structures.

Functional outcomes and recurrence determinants in craniofacial fibrous dysplasia: Insights from 3D computed tomography and comprehensive clinical evaluation.

BACKGROUND: This study aimed to identify risk factors for postoperative lesion regrowth and to assess functional outcomes in craniofacial fibrous dysplasia, using a three-dimensional computed tomographic volumetric analysis. METHODS: A retrospective analysis was conducted on 47 patients with craniofacial fibrous dysplasia who were treated from July 2005 to December 2020. Patients were treated with either conservative shaving or radical excision followed by reconstruction. Demographic data, surgical details, lesion recurrence, and functional outcomes were assessed. Lesion volume changes and recurrence were evaluated clinically and through a computed tomographic volumetric analysis. RESULTS: Among the patients, 16 underwent conservative treatment, whereas 31 received radical treatment. The radical group showed more significant improvements in functional outcomes, particularly in orbital dystopia and facial asymmetry. Postoperative lesion volume was notably lower in the radical group (41.94 ± 38.13 cm3) compared with the conservative group (78.3 ± 47.3 cm3, p = 0.008). This reduction was maintained over an average follow-up of 3 years. Lesion growth rates were similar between the groups (8.17 ± 5.85% in radical vs. 5.84 ± 6.43% in conservative, p = 0.268). Multivariate analysis indicated that patients aged ≤20 years and those with multifocal involvement had significantly higher risks of recurrence, with adjusted odds ratios of 11.269 (p = 0.039) and 6.914 (p = 0.046), respectively. CONCLUSIONS: Our findings suggest that both conservative and radical treatments for craniofacial fibrous dysplasia provide benefits, with the radical approach notably enhancing functional outcomes. However, neither method definitively reduces lesion recurrence, highlighting the necessity for an individualized treatment strategy. This approach should balance functional enhancement with recurrence risks, tailored to each patient's specific clinical scenario.

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.

Modification of LeFort I Osteotomy for Correction of Severe Maxillary Cant Associated With Fibrous Dysplasia.

Differential superior reposition of maxilla following LeFort I osteotomy in the correction of maxillary cant poses a greater challenge especially when associated with the pathology like fibrous dysplasia which completely obliterates the antrum. Purpose of this paper is to highlight the modification of LeFort I osteotomy and hypothesis is to assess its difficulty index in modifying the standard steps, in executing the maxillary separation at various to correct the gross facial asymmetry to achieve a favorable outcome. Multiphased management involved scrupulous clinical planning, advanced imaging by computed tomgraphy scans, stereolithographic models to debulk the lesion. The second phase included pre surgical orthodontic evaluation along with correction of severe maxillary cant adopting a modified LeFort 1 osteotomy technique and standard bilateral sagittal split osteotomy, thereby simultaneously attaining functional stability and esthetic harmony.

Augmented reality navigation method for recontouring surgery of craniofacial fibrous dysplasia.

The objective of this study is to introduce the application of augmented reality (AR) navigation system developed by the authors in recontouring surgery of craniofacial fibrous dysplasia. Five consecutive patients with craniofacial fibrous dysplasia were enrolled. Through three-dimensional (3D) simulation, a virtual plan was designed to reconstruct the normal anatomical contour of the deformed region. Surgical recontouring was achieved with the assistance of the AR navigation system. The accuracy of the surgical procedure was assessed by superimposing the post-operative 3D craniomaxillofacial model onto the virtual plan. The pre-operative preparation time and operation time were also counted. In all patients, AR navigation was performed successfully, with a mean ± SD of the errors of 1.442 ± 0.234 mm. The operative time of the patients ranged from 60 to 80 min. The pre-operative preparation time was 20 min for each patient. All the patients showed uneventful healing without any complications, in addition to satisfaction with the post-operative aesthetics. Using our AR navigation system in recontouring surgery can provide surgeons with a comprehensive and intuitive view of the recontouring border, as well as the depth, in real time. This method could improve the efficiency and safety of craniofacial fibrous dysplasia recontouring procedures.

[Craniofacial fibrous dysplasia: about six cases]. / Dysplasie fibreuse osseuse crânio-faciale: à propos de six observations.

Fibrous dysplasia (FD) of bone is a benign, congenital and rare disease in which normal bone is replaced by fibrous bone tissue, resulting in bone deformities. It can affect any bone in the body, however craniofacial fibrous dysplasia is characterized by specific clinical manifestations, progression and therapeutic issues. The purpose of our study was to describe the diagnostic, therapeutic and evolutionary features of craniofacial FD. This study involved six patients with craniofacial FD followed up in the Department of Otolaryngology at the Principal Hospital of Dakar. The average age of patients was 26.16 years, ranging from 11 to 58 years. Sex ratio favoured women (83% of the cases). Bone deformity was the main feature of craniofacial FD leading to diagnosis. One patient presented with unilateral nasal obstruction with epistaxis. In all cases, scanner enabled diagnosis and topographic balance. Two female patients underwent surgery. One case of recurrence was reported. Craniofacial FD is a rare bone disease that can manifest as serious sensory and functional disorders. It poses real therapeutic issues; hence adequate interdisciplinar management is essential.

Serie de Casos Clínicos: Displasia Osteofibrosa Craneofacial. Resolución Quirúrgica / Case Series: Craniofacial Fibrous Dysplasia. Surgical Treatment

INTRODUCCIÓN: La Displasia Fibrosa Ósea Craneofacial es una lesión ósea benigna en la que se da una sustitución de tejido óseo normal por tejido fibro-óseo. Desarrollada a partir de una mutación genética. Subordinada en variantes: monostósica y polistósica. Escasos casos son reportados sobre malignización de la patología. El Diagnóstico se realiza con la sospecha clínica y se confirma mediante exámenes de imagen y anatomopatológicos. CASO CLÍNICO: El presente trabajo presenta una serie de tres casos diagnosticados de Displasia Osteofibrosa Craneofacial en el Hospital José Carrasco Arteaga y Hospital del Río, Cuenca-Ecuador, en los que por el cuadro clínico de los pacientes se decidió tratamiento quirúrgico. EVOLUCIÓN: En todos los casos, la evolución fue favorable. Se logró resultados positivos, reducción de síntomas y mejoría estética general en todos los casos. CONCLUSIÓN: Se obtienen mejores resultados con los tratamientos quirúrgicos actuales (remodelado más congelamiento óseo), entre ellos: buenos resultados estéticos, mejor calidad de vida, menor riesgo de infecciones. En el presente trabajo los tres participantes sometidos a resolución quirúrgica señalaron que el tratamiento reflejó positivamente en el ámbito funcional, estético y emocional. (au)

BACKGROUND: Craniofacial Bone Fibrous Dysplasia is a benign bone lesion where normal bone tissue is replaced with fibrous tissue. Developed from a genetic mutation. Subordinated in variants: monostotic and polyistotic. Few cases are reported on malignancy of the pathology. The diagnosis is determined with clinical suspicion and confirmed by imaging and pathology tests. CASE REPORT: In this paper, we present a series of three cases diagnosed with Craniofacial Fibrous Dysplasia at José Carrasco Arteaga Hospital and Hospital del Río, Cuenca-Ecuador. All of patients were treated with surgery, because of the clinical features. EVOLUTION: In all the cases, the patient evolution was favorable. Reduction of symptoms and general aesthetic improvement were achieved in all cases. CONCLUSION: Better results are obtained with the current surgical treatments (bone remodeling plus bone freezing), among them: good aesthetic results, better quality of life, lower risk of infections. In the present paper, the three patients treated with the surgical procedure indicated that the treatment reflected positively in the functional, aesthetic and emotional aspects.(au)

Fibrous Dysplasia treated with virtual planning ­ report of two cases

Fibrous dysplasia (FD) is part of a rare group of bone dysplasia. It exhibits benign behavior and can lead to osteolytic lesions, deformities, and fractures. The treatment is challenging, and accurate removal of the lesion is necessary to restore function and esthetics. Here we present two cases of FD where virtual planning with presurgical computed tomography (CT) was used for the production of a surgical guide for bone contouring. First, CT image reconstruction was performed to mirror the patient's original anatomy. Then, three surgical guides that determined the area and depth of bone wear were prepared and used in the relevant sequence during the actual surgeries, which were successfully performed in both patients. This technique is termed the template guide holes (TGH) technique. The findings from this report suggest that presurgical virtual planning and guide preparation allows direct and objective measurement of the level of bone wear and improves the functional and esthetic outcomes of surgery for FD. In particular, the TGH technique is safe and allows adequate preoperative surgical simulation, reduces the surgical duration, and increases the predictability of the final result.

Craniofacial fibrous dysplasia associated with McCune-Albright syndrome: challenges in diagnosis and treatment: case reports.

BACKGROUND: McCune-Albright syndrome (MAS) is a rare multisystem disorder that classically was defined by the triad of polyostotic fibrous dysplasia of bone, café-au-lait skin pigmentation, and precocious puberty. It is a condition that has a gradual onset, slow growth rate and remain painless throughout. The clinical phenotype of MAS is highly variable and no definite treatment is available. CASE PRESENTATION: This article describes two cases, a 10-year-old girl and an 11-year-old boy, both with MAS comprising deforming craniofacial FD. Challenges related to diagnosis and management included late reporting with big lesions, involvement of multiple craniofacial bones, mutilating surgeries and ultimately high degree of morbidity. CONCLUSION: Delayed diagnosis and management of MAS results in devastating physical disabilities and severe morbidity after treatment.

Navigation-assisted endonasal endoscopic optic nerve decompression in fibrous dysplasia.

A 12-year-old girl presented with left-sided decreased vision of 2-month duration. Clinical evaluation and imaging revealed fibrous dysplasia compressing the left optic nerve with no underlying endocrinological abnormalities. Best-corrected visual acuity showed progressive deterioration of vision over 2-month follow-up. She underwent navigation-assisted endonasal endoscopic optic nerve decompression. Post-surgery there was improvement in vision and it became normal (6/6). Six-month follow-up showed stable vision with no further complications.

Cystic Degeneration of Craniofacial Fibrous Dysplasia.

BACKGROUND: Fibrous dysplasia (FD) is most often a slowly progressive benign disease in which the normal bone structure is replaced by fibrous and osteoid tissue. CASE DESCRIPTION: A 16-year-old adolescent, known with FD in the sphenoid bone, suffered an acute decreased visual acuity with papilledema on the left eye. The radiologic images were best compatible with cystic degeneration of the known FD with optic nerve compression in the optic canal. Decompression of the optic nerve was performed through an endoscopic exploration of the left sphenoid sinus. The visual acuity recovered completely. CONCLUSIONS: In FD with cystic changes, leading to acute signs of optic nerve compression, early aggressive surgical decompression is strongly recommended. Cystic degeneration of the FD, although rare, should be considered.

Fibrous dysplasia imitating malignancy.

Fibrous dysplasia is a benign bone disease, presenting as monostotic or polyostotic lesions, or as part of a syndrome (McCune-Albright/Mazabraud). Its clinical course shows a variegated picture and the progression of its growth is unpredictable. In the workup of 39 fibrous dysplasia cases in the cranio-facial area, four cases presented fast growth tendencies, of which two patients with McCune-Albright syndrome showed malignant-like rapid growth. This local aggressive form is extremely rare, and the concept of this issue has not been clearly defined. With regard to the speed of growth a volumetric-time analysis in one of our cases demonstrated a 74 days tumor doubling rate with an exponential growth curve. According to the literature the aggressive form presented extra-cranially mainly at an adult age, whereas its appearance in our cranio-facial patient collective was much younger. Distinguishing nonmalignant and malignant aggressive forms is difficult and highly inconsistent in the literature. We therefore implemented a quantitative growth measure analysis to define aggressive forms based on progression and speed of growth and impartial of type of FD, localization or functional incapacity. Due to our study findings and literature review we state a prevalence of an aggressive form might be possibly about 5 %.

Abordagem cirúrgica no tratamento da displasia fibrosa craniofacial- experiência de 14 anos / Surgical approach to the treatment of craniofacial fibrous dysplasia - A 14-year experiencedysplasia - 14 years experience

INTRODUÇÃO: Dentre os tumores benignos do esqueleto craniofacial, a displasia fibrosa caracteriza-se pelo crescimento progressivo e acometimento de jovens, acarretando deformidade funcional e estética. Esse trabalho analisa aspectos clínicos e prognósticos de pacientes com essa patologia, submetidos a tratamento cirúrgico. MÉTODOS: Análise retrospectiva de 19 pacientes com displasia fibrosa craniofacial, acompanhados de janeiro de 1997 a dezembro de 2011, tratados com remodelamento ósseo e ressecção cirúrgica. Revisão da literatura sobre. RESULTADOS: A distribuição etária variou de 8 a 65 anos, com média de 21,75 anos. Dez pacientes (52,7%) eram do sexo feminino. A forma poliostótica predominou, com 15 casos (78,9%). Entre os ossos acometidos destacaram-se esfenóide, etmóide e frontal, nas formas poliostóticas; e maxila e zigoma, nas monostóticas. A principal queixa foi de assimetria da face. Um paciente evoluiu com diminuição de acuidade visual. O tratamento baseou-se na ressecção cirúrgica e reconstrução com enxerto, quando doença localizada, e no desgaste e remodelamento ósseo, em formas poliostóticas. Em apenas um caso (5,2%), com compressão de nervo óptico, foi necessário acesso intracraniano. Reabordagem cirúrgica, por crescimento tumoral, foi necessária em três pacientes. Como complicações, tivemos um caso de lagoftalmo e epicanto no pós-operatório de paciente tratado por cirurgia com acesso infraorbitário. Ausência de outras intercorrências no seguimento a curto e longo prazo. Os resultados de preservação de função e recuperação de contorno facial foram satisfatórios. CONCLUSÃO: Essa experiência, em concordância com a literatura, permite concluir que a cirurgia é eficaz na abordagem de casos selecionados de displasia fibrosa craniofacial.

INTRODUCTION: Fibrous dysplasia is benign tumor of the craniofacial skeleton that primarily affects young patients. It is characterized by the progressive growth of benign fibrous tumors with resulting functional and aesthetic deformities. This study assesses the clinical and prognostic features in patients with fibrous dysplasia who underwent surgical treatment at our institution. METHODS: Retrospective analysis of 19 patients with craniofacial fibrous dysplasia, treated between January 1997 and December 2011 with bone remodeling and surgical resection. We also review the literature regarding fibrous dysplasia. RESULTS: Patients ranged between 8-65 years old, with a mean age of 21.75 years. Ten patients (52.7%) were women. The polyostotic form was predominant and present in15 cases (78.9%). The sphenoid, ethmoid, and frontal bones were most commonly involved in the polyostotic form and the mandibular and zygomatic bones were most commonly involved in the monostotic form. The main complaint was asymmetry of the face. One patient developed decreased visual acuity. Treatment was based on surgical resection and graft reconstruction in the localized form of the disease, and bone abrasion and remodeling in the polyostotic form. Intracranial access was necessary in only one case (5.2%) where the optic nerve was compressed. Repeat surgical treatment due to recurrent tumor growth was necessary in three patients. The only complication occurred in a patient who developed lagophthalmos and epicanthus postoperatively after undergoing surgery using infraorbital access. No other complications occurred during short- and long-term follow-up. Functional preservation and facial contour recovery outcomes were satisfactory. CONCLUSION: Our experience, along with that of other investigators, demonstrates that surgery is effective in treating selected cases of craniofacial fibrous dysplasia.