Abstract
Craniofacial fibrous dysplasia (CFD) is an asymptomatic disease that can have oral manifestations with severe effects on a patient's wellbeing. It is usually diagnosed based on patient concerns, including poor appearance due to spacing and functional difficulties in eating and speaking, as well as the presenting features, such as facial asymmetry and corresponding malocclusion, delayed or abnormal tooth eruption, and painless swelling or bony expansion. General dental practitioners (GDPs) will often see patients presenting with these multiple symptoms which can be overwhelming. An awareness of the oral and dental features is thus important to make an early diagnosis and to ensure that the patient is given the best advice and guidance early on what is available to address their concerns. This paper provides an overview of the oral and dental features associated with CFD and presents two cases in whom delayed diagnosis resulted in complex treatment needs to address their concerns. Both cases highlight the importance of early diagnosis and the essential role of GDPs in early recognition of the condition to optimise patient care and wellbeing.
Key points
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Early recognition of the oral and dental features of fibrous dysplasia is important to initiate early management.
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General dental practitioners play an essential role in the early recognition of these features.
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Patients with craniofacial fibrous dysplasia have complex integrated treatment needs which need early recognition, intervention and referral if necessary.
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Introduction
Fibrous dysplasia (FD) is a rare, developmental, benign fibro-osseous disease in which normal bone is replaced with fibrous tissue and structurally weak bone due to a change in normal bone metabolism by somatic gene mutation during embryogenesis.1 The clinical symptoms of the disease depend on when the mutation occurs and the level of differentiation of the primary pluripotent cells.2 Its incidence is estimated to be 1-in-5,000–10,0003 and constitutes 5–7% of all benign bone lesions.4,5 A 2:1 female-to-male ratio has been suggested with manifestation usually in the first few years of life.6
FD can be monostotic involving one bone, polyostotic involving multiple bones, or syndromic being associated with Jaffe-Lichtenstein disease, or Mazabraud and McCune-Albright syndromes.7 Those with the McCune-Albright syndrome also present with skin pigmentation, with light brown irregularly shaped patches with jagged edges known as ‘café-au-lait spots'. Other features related to endocrine abnormalities include hyperthyroidism, excess cortisol production (Cushing's syndrome), enlarged facial features, hands and feet (acromegaly)8 and proptosis (bulging eyes). Hearing loss or optic nerve damage caused by cranial nerve compression may also be seen.9 Monostotic FD accounts for 70% of all cases and involves the jaw bones, especially the posterior maxilla, in which painless expansion of the bone occurs, with the term craniofacial fibrous dysplasia (CFD) used to describe cranial bone involvement.5 Its prevalence in the craniofacial region ranges from 10–25% in monostotic disease, and up to 90% in polyostotic disease.10,11 The clinical features of FD are influenced by the type and can be isolated to small areas or may involve extensive spread to wider areas. CFD lesions are some of the earliest that can be detected, and the maxilla is affected almost twice as often as the mandible, with the lesions usually expanding during childhood/adolescence and becoming less active in adulthood. Expansion of the alveolar ridge cortices may extend to the hard palate.12,13 Other facial and dental features include facial asymmetry and deformity caused by the bone expansion within the jaw; malocclusion; tooth displacement; dental crowding and spacing; enamel hypoplasia; odontomas and retained primary teeth; diminution of the maxillary sinus; dentine dysplasia; and taurodont pulp chambers.14 Patients with CFD may also have a high caries incidence and when present with jaw deformities and café-au-lait lesions, a diagnosis of CFD should be suspected.
Diagnosis of CFD is based on the patient's history and clinical presentation; however, radiographic findings can be variable depending on the extent and severity of the disease. The classical radiographic appearance of the bone is described as ‘ground-glass' or ‘mixed mottled' with interspersed radiolucency and radiopacity and ill-defined lamina dura.15 Displacement of the inferior dental canal in the mandible due to abnormal bone formation within the fibrous tissue matrix is also seen.16 CFD can be misdiagnosed due to similarities of the clinical and radiographic features with other conditions17 (Table 1); however, the use of high-resolution imaging, such as cone beam computed tomography (CBCT) providing 3-dimensional images of the bone structure18 and magnetic resonance imaging useful for evaluating bone marrow and soft tissue components, have been invaluable in aiding diagnosis. Recent diagnostic tools, such as molecular and genetic testing with GNAS mutation analysis, are more sensitive19 and diagnostic for CFD and diphosphonate bone scanning (scintigraphy) used in conjunction with these other tests can help in determining the extent of the disease and additional sites of activity.20
Biopsy with a histological analysis, however, remains the gold standard and shows irregularly shaped immature woven bone trabeculae, often described as Chinese characters,21 in loosely organised fibrous connective tissue matrix containing spindle-shaped fibroblasts with inactive osteoblasts and a lack of osteoblastic rimming. As the fibrous tissue progressively replaces normal bone, the medullary cavity enlarges, with thinning of the cortical bone, and the fibrous component can be highly cellular or relatively sparse, exhibiting a whorled or storiform pattern.16
The most common oral complaints with CFD include poor appearance, discomfort and difficulty in function related to the spacing of the teeth and progressive changes in occlusion as the jaws expand. The primary goal in the management of these patients is to improve aesthetics, function and comfort, with preventive measures underpinning any intervention and the treatment options being influenced by the age at presentation and the extent and behaviour of the disease. Early recognition and diagnosis of the oral features of CFD are essential to minimise protracted courses of intervention and help raise the patient's awareness of the need for prevention to minimise progressive deterioration.
Two patients in whom the early diagnosis of CFD was overlooked resulting in advanced dental challenges necessitating extensive and protracted restorative management are presented.
Case 1
A 28-year-old white male patient presented complaining of poor appearance and mobility associated with his anterior teeth causing difficulty with chewing.
A diagnosis of polyostotic CFD affecting both jaws had been made at the age of 15. He had given up smoking one year ago and reported high stress levels. There was a history of orthodontic treatment between 11–16 years of age, with difficulty encountered with tooth movement. He was an irregular dental attender as he was told ‘nothing could be done'. His extra-oral appearance is seen in Figure 1.
Case 1: extra-oral smiling appearance
Figure 2 shows the intra-oral presentation. The anterior maxilla and mandible were enlarged with reduced sulcus depth, plus poor oral hygiene with generalised inflammation of the gingival tissues with bleeding and probing depths of 3–5 mm. Teeth 11 and 21 were grade III mobile and 42 was grade II mobile. The gingival tissues were of thin biotype.
Case 1: intra-oral appearance of the teeth. (A) Cross bite and deep overbite and missing teeth. (B) Expansion of the anterior maxilla with spacing. (C) Expansion of the anterior mandible with spacing and crowding. (D, E) Right and left buccal views
Figure 3 and Figure 4 show the radiographic appearance and CBCT of the jaws, confirming the bony appearance and expansion.
Case 1: orthopantomogram showing the typical ‘ground-glass' and mixed mottled appearance (indicated with arrows), unerupted teeth, taurodont molars, short roots and poorly defined lamina dura and inferior dental canal
Case 1: cone-beam computed tomography scan. (A, B) Axial views of the maxilla and mandible showing the bone expansion and the position of the impacted canine teeth. (C) Sagittal view of the anterior maxilla and mandible showing the bone expansion and the position of the impacted canine tooth
Diagnoses of localised periodontitis stage III, grade C, currently unstable with the smoking history and stress as the risk factors, impacted teeth and CFD causing the spacing and malocclusion of the dentition, were made. The prognosis of the upper incisor teeth was poor. The occlusion and the underlying CFD, associated with the unpredictability in its response to extraction and dental implant surgery, posed challenges in addressing his main concerns, complicated by his expectations, attitude and compliance.
The dental management was phased and the primary challenges of treatment, including the unpredictability of the outcome, the risk of bleeding, and the issues with quality of the bone and its influence on the provision of dental implants, were discussed with the patient. Further engagement with the patient focused on ensuring that he understood these challenges and his commitment and attitude to the treatment needed to address his concerns. Treatment was executed as follows.
Initial phase
The S3 Treatment Guidelines for Periodontitis22 were followed to manage the periodontal issues. A diagnostic wax-up to show the feasibility of space closure in the anterior region on loss of 12 and 11 and 21 and 22 was shown to the patient. A decision was made to proceed with a transitional denture due to the uncertainty of the healing response to the extraction due to the underlying CFD. At review, patient compliance had improved with resolution of the periodontal problems. The patient was reminded of the previously discussed risks and challenges and consented to proceed with the next step.
Interim phase
An immediate tooth-supported upper partial denture with no anterior flange was fitted following extraction of the teeth (Fig. 5) and 42 was also extracted and ongoing maintenance was implemented. Healing was uneventful at one week and the denture was relined with tissue conditioner and the lower spaces were maintained. He was delighted with the improved comfort and enhanced aesthetic outcome. The definitive tooth replacement options were revisited, and dental implants were considered as the fixed option. The associated risks, including the uncertainty with vascularity and increased risk of bleeding, the quality of bone due to CFD, the possible impact on the wound healing following surgery and the issues with osseointegration of the implants,12 were discussed, and detailed planning was undertaken, taking into consideration the management of the unerupted canines.
Case 1: improved appearance following fit of the immediate partial denture. (A) Smiling. (B) Retracted
Definitive phase
Six months later, restoratively driven implant placement with four implants in the maxilla and two in the mandible at an insertion torque of 35 Ncm was undertaken (Fig. 6). Despite the fibrous nature of the bone, high primary stability was achieved and healing one week later was uneventful. A provisional bridge was fitted five months after the surgery in the maxilla and crowns fitted in the mandible (Fig. 7). The radiographs taken post-fit (Fig. 8) showed the ground-glass bone appearance. The radiolucency seen on the distal of the lateral incisor implants could be the healing response of the CFD as clinically there was no pocketing, bleeding or inflammation noted around the implants. The patient was delighted with the outcome.
Case 1: at first stage implant surgery. (A) Fibrous appearance of the alveolar bone with expansion. (B) Implant fixtures in situ
Case 1: upper implant-supported bridge and lower right and left implant-supported crowns in situ
(A, B) Case 1: peri-apical radiographs showing the implants placed in the anterior maxilla
The patient has been kept under regular follow-up to ensure his compliance is maintained. His attitude to dental treatment has improved and the gingival tissues around the implants continue to remain stable despite the lack of keratinised tissue which is unlikely to be related to the CFD. The patient has requested a break from treatment and the provision of the final prosthesis has thus been delayed. When he is ready to embark on the final stage of treatment, the site will be reassessed and the need to increase the keratinised gingival tissue will be considered.
Table 2 summarises the key challenges encountered in the management of this case and outcomes achieved.
Case 2
A 78-year-old Afro-Caribbean female patient presented complaining of poor appearance due to a failing bridge and loose teeth causing difficulty with chewing.
A diagnosis of monostotic CFD affecting the right maxilla managed with three-monthly intravenous infusions of pamidronate was made in 2015. Hypertension, osteoarthritis, schizophrenia, and well-controlled type II diabetes mellitus were noted. The patient reported to be a non-smoker and an irregular dental attender.
The extra-oral appearance with a severe cant on the right side is seen in Figure 9.
Case 2: Extra-oral appearance showing facial asymmetry, high smile line, and cant of the maxillary occlusal plane
The intra-oral appearance is shown in Figure 10, with the expansion of the right maxillary alveolus causing the occlusal cant. The oral hygiene was poor, with inflamed gingival tissues with bleeding and probing depths of up to 7 mm. The upper anterior bridge was failing with extrusion of the grade III incisor teeth and missing molars with a traumatic overbite.
Case 2: intra-oral views. (A) Right cant with teeth in occlusion. (B) Palatal view showing the bony expansion of the right maxilla. (C, D) Right and left lateral views showing the lack of interocclusal space on the right side
The ‘ground-glass' radiographic appearance of the bone in the right maxilla and mottled appearance in the lower right mandible are evident in Figure 11.
Case 2: orthopantomogram showing the ground-glass and mottled appearance of the alveolar bone (indicated with arrows) and generalised horizontal bone loss
Diagnoses of generalised periodontitis stage IV, grade C, currently unstable with controlled diabetes, CFD, acquired tooth loss and failing upper anterior bridge were made. The prognosis of the upper teeth and the lower right molar teeth was poor and that of the remaining teeth was questionable.
The dental management was complicated by the differential size of the maxilla due to the bony expansion, the maxillomandibular relationship, deep overbite, intravenous drug infusions and patient compliance. To correct the malocclusion and facial asymmetry, the options of surgical bone recontouring and orthognathic surgery23 were considered; however, due to the risks with the intravenous drug infusion, risks of recurrence following surgery24 and the patient's intolerance to extensive surgical procedures, these were discarded. The patient's focus was the maxillary teeth only and treatment was thus planned with a phased approach aimed at the upper jaw keeping status quo in the mandible. The associated challenges with the failing teeth in line with her expectations and compliance was discussed and consent was obtained for treatment executed as follows.
Initial phase
The S3 Treatment Guidelines for Periodontitis22 were followed to manage the periodontal disease and her role highlighted while planning removal of all the upper teeth and their replacement with an immediate upper denture in conjunction with the medical practitioner and the laboratory technician. The latter was essential to establish how much the cant could be corrected (Fig. 12).
Case 2. (A) Immediate maxillary complete denture on articulated working models. (B) Maxillary arch clearance. (C) The immediate maxillary denture relined with temporary soft denture liner. (D) Appearance following fit of the denture fitting of the immediate maxillary denture
Interim phase
The upper teeth were removed and the denture was fitted. The intravenous drug infusion was stopped for three months after the extractions. One week later, healing was uneventful and the denture was relined with tissue conditioner. The patient was delighted with the outcome.
Definitive phase
The definitive denture, constructed using copy denture technique, was fitted and the patient was reviewed for 18 months (Fig. 13). The patient remained compliant and showed a new level of confidence with the outcome.
Case 2: definitive maxillary complete denture. (A) Fit appointment. (B) Smile at review 15 months. (C) Facial view
Table 3 summarises the key challenges encountered in the management of this case and outcomes achieved.
Discussion
Both cases had a diagnosis of CFD but posed different challenges in their dental management; however, both were complicated by the poor attitude/compliance due to erroneous information that ‘nothing could be done' given to them. The effect of oral health on patients' quality of life has been reported25 and this was evident in both patients in whom as soon as the pain was resolved and the appearance improved, there was an increased confidence and positivity seen towards their dental and oral health.
The clinical presentation of the first case is in line with the features published for polyostotic CFD.10 The key challenge in this case was the patient's attitude and behaviour. A phased treatment approach allowed for the exploration of these issues while simultaneously assessing the stability of the CFD and evaluating the patient's expectations. The decision to opt for implant treatment was influenced by the patient's age and his desire for a fixed option, and was only undertaken after careful evaluation of the healing and post-extraction bone quality. It is reported that endo-osseous implants in patients with CFD are at a higher risk of failure due to a poorer bone quality, rate of bone healing and osseointegration.26 In our case, there was good vascularity and healing which was evident following the extraction, and high primary stability of the implants had been obtained at high insertion torque, thus giving the confidence of a good outcome. A five-year successful outcome has been reported for implants in patients with CFD27 and others have reported similar success with complete re-ossification of the bone and integration of implants.28,29,30 The risk of failure is reported to be reduced if the implants are placed after skeletal maturity when the growth of CFD lesions have diminished.28 Implant outcomes in both CFD bone and normal bone have been reported to be comparable;29 although, one study with a full arch maxillary rehabilitation reported a failure of one implant due to peri-implantitis after six years.31 One study reported successful osseointegration of an implant placed in the maxilla subsequent to surgical CFD lesion removal with histologically confirmed healing, with one-year peri-implant bone level stability.32 Another study reported successful surgical recontouring of CFD that developed around a dental implant in the maxilla, with preservation of peri-implant soft and hard tissues over a six-month follow-up.33 In the case presented, the implants have been in situ for just over three years with no complications. This outcome was underpinned by integrated treatment planning with careful consideration of the bone quantity and quality using a phased treatment approach with atraumatic surgical techniques used for implant placement. In this case orthodontic treatment had been attempted; however, this was unsuccessful due to lack of tooth movement, which is contrary to published evidence which states that orthodontic tooth movement can be undertaken in CFD cases; although, a high tendency of relapse has been reported.11 The root shortening associated with the central incisor teeth could be due to the orthodontic treatment and subsequent tooth mobility related to the unstable periodontal disease and deep overbite. The quality of the gingival tissues was difficult to establish at the outset; however, once the periodontal health was stabilised, thin gingival biotype was noted. The remodelling changes post-extraction may have been contributory to the change in the gingival tissues and the lack of keratinised tissue is often reported around dental implants. In this case, this is unlikely to be related to the CFD; however, before the definitive implant provision, creating a band of keratinised tissues should be considered to minimise the risk of future peri-implant disease.34 The effect of CFD on keratinised tissues and periodontal disease remains unanswered with little to no evidence published. In this case, the cause of the periodontal disease was primarily his plaque control with his smoking history as the risk factor, and if the CFD had been a major issue, the outcome to the debridement would not have been successful. The treatment executed up to the provision of the denture is straightforward and falls within the remit of a general dental practitioner (GDP), who needs to be mindful of the challenges in patient management due to the stigma. It is important to recognise the benefits of early recognition, stage the treatment to address concerns promptly, and refer the patient early when necessary.
The second case presented the classical features of monostotic CFD, including a slow-growing and painless unilateral expansion of the posterior maxilla,13,35 with a late diagnosis made in the second decade of life noted as an incidental radiographic finding.28,36 The slow-growing nature of the lesion was confirmed in this case where the progressive increase in the spacing between the teeth had prompted her to seek help with the secondary periodontal issues that had led to pain. The intravenous infusion complicated the provision of the dental management due to the increased risk of the medication-related osteonecrosis of jaw (MRONJ).37 The case highlights the importance of close integrated working with medical practitioners to manage the risks and also emphasises the importance of controlling primary diseases, which in her case had exacerbated the ongoing discomfort and pain from the teeth.37,38 Dental practitioners should be aware of the Scottish Dental Clinical Effectiveness Programme guidelines which provide clear guidance on how to manage patients taking anti-resorptive drugs regarding preventive advice, understanding the risks of extraction, and undertaking close clinical and radiographic monitoring.39 While surgical correction of the excess bone could have been considered, due to the risk of MRONJ, this was deemed inappropriate. It has been reported that even after surgical correction, problems with denture retention and stability can be seen due to adaptability.23,40 The denture offered the flexibility of correcting the cant and is a more cost-effective alternative, especially in the presence of uncertainty related to the CFD activity. This patient was keen on staying with a denture and such patients can be managed effectively by GDPs; however, where complexity is driven by the presence of facial deformities, lack of sufficient restorative space, asymmetries and severe malocclusion with unstable jaw dimensions, a referral to a specialist should be considered.
Both cases highlight the importance of early recognition and intervention by GDPs. While the tooth drifting and mobility could be secondary to the CFD, the unstable periodontal disease, which is preventable, had led to the exacerbation and poor prognosis of the teeth, and early intervention could have minimised this risk. This is essential to ensure that patients are being given the appropriate guidance and advice at the outset and as most of the population are first encountered by the GDPs, they play a key role in early diagnosis and/or referral. The key areas that GDPs should be mindful of when seeing such patients are:
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Listening and hearing the patient's complaints, especially if the complaint is of poor appearance due to spacing or tooth movement or unusual facial asymmetry. Further questions should be asked about duration and timing and if it is worsening
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Patients complaining about ‘pigmentation'. Those with appearance of café-au-lait spots should be questioned further about their medical history and the syndromic CFD suspected
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Clinical examination and full mouth assessment should be undertaken to exclude other causes of tooth movement, e.g., periodontal disease, apical infection. Preventive care should be initiated, and the patient should be reassessed to evaluate the next steps in the event of no improvement or change
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Ensure communication with the patient is clear, informative and relevant, providing guidance on the treatment progress, and highlighting their role
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Discuss treatment options ensuring the patient understands the limitations
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When feeling overwhelmed, consider referral but initiate primary dental disease management with patient engagement.
GDPs, as the gatekeepers to patient care, play an essential role in the ongoing management of patients for their day-to-day oral health needs and even for patients who are referred.13,35
Figure 14 provides an overview of the patient journey in the management of patients with CFD bringing together the concepts covered in the cases presented.
Diagnostic and treatment pathway for patients with suspected craniofacial fibrous dysplasia
Conclusion
Craniofacial fibrous dysplasia is a rare condition that can affect patients who present to GDPs. In these patients, preventive care and early intervention will help towards maintaining the dentition and oral health; however, this must be an integrated and collaborative approach, with the patients at the core of the management. The cases presented have highlighted the importance of early intervention while emphasising the importance of a patient-centred individualised approach to managing their expectations and compliance. In both cases, the planning was undertaken with patient engagement and with a joint decision-making approach, taking into account factors such as the patient's age and skeletal maturity, as well as their compliance and the location, severity and behaviour of the lesions. All these have to be considered when planning a patient's management, recognising that dental procedures are safe to undertake in patients with CFD.
GDPs are proficient in managing a wide spectrum of dental diseases and conditions which should include CFD. On the other hand, some cases of CFD may exhibit intricacies and complex treatment needs and may need early referral management to a specialist or tertiary care. Even in these cases, the GDPs play a pivotal role, not just in the management of the primary disease stabilisation, but also in the provision of maintenance and supportive care needed by the patients afterwards to maintain a successful outcome.
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MWHM: conceptualisation (supporting); methodology (supporting); project administration (lead); investigation (equal); writing original draft (equal); review and editing (lead). RR: conceptualisation (supporting); methodology (supporting); writing original draft (equal); review and editing (supporting). JKB: conceptualisation (supporting); methodology (supporting); writing original draft (equal). KD: conceptualisation (supporting); investigation (equal). URD: conceptualisation (lead); methodology (lead); project administration (supporting); investigation (equal); review and editing (supporting). All authors gave final approval and agreed to be accountable for all aspects of the scientific work.
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Man, M., Ruparelia, R., Batra, J. et al. Craniofacial fibrous dysplasia: a challenge for general dental practitioners. Br Dent J 239, 693–702 (2025). https://doi.org/10.1038/s41415-025-9010-y
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DOI: https://doi.org/10.1038/s41415-025-9010-y
