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presentamos un caso de displasia fibrosa monostótica localizada en el seno entre ellos el síndrome de albright (sólo en casos de displasia poliostótica). su. Displasia fibrosa monostótica e poliostótica. Front Cover. Simone Paula Bibliographic information. QR code for Displasia fibrosa monostótica e poliostótica. Download PDF. 1 / 3 Pages. Previous article. Go back to website. Next article.

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Fibrous dysplasia is a benign fibrous-osseous lesion in which normal bone is replaced by fibrous connective tissue and immature bone, affecting only one monostotic or several bones polyostotic and mainly occurring in children and young adults.

When present in facial bones, the maxilla is more frequently involved than the mandible, which can cause facial asymmetry in addition to dental complications. In the image exams, the main characteristic of fibrous dysplasia is its unpolished glass appearance. Computed tomography is the ideal method for evaluating this lesion and its relationship with adjacent structures.

The use of conventional radiography, due to the overlapping of anatomical structures, makes it difficult to delineate the extension of the lesion. The present study is aimed at guiding dentist-surgeons on the main imaging characteristics of monkstotica dysplasia by describing a case of a female year-old patient presenting with this lesion in the maxilla.

Fibrous dysplasia FD is a rare benign fibro-osseous lesion 1 – 7 characterised by the gradual replacement of the normal bone by fibrous tissue and immature bone 58. Although its aetiology is unknown, it is thought that FD may be of genetic or traumatic origin 8.

Clinically, FD can be classified as monostotic when limited to only one bone and polyostotic when more bones are involved 1 – 25 – In this type of FD, man and women are affected with the same frequency 712 – 13 The polyostotic form is rarer and mainly occurring in long bones 610 and possibly associated with McCune-Albright syndrome, which is characterised by the presence of coffee-milk cutaneous pigmentation and endocrinopathy 136 – 1016 This type is more frequent in women and may cause precocious puberty 1310 – 1113 – 1418 – Other syndrome less common than polyostotic FD may be associated with Mazabraud syndrome, which is characterised by the involvement of soft tissues with presence of intra-muscular myxomas 511 – 1216 When FD reaches the sphenoid, temporal, zygomatic and frontal-nasal bones of the maxilla and skull base is termed as craniofacial FD 1811 – 12 The most common clinical symptom in the cases of craniofacial FD is a painless swelling in the region involved, including facial asymmetry which may cause severe deformity resulting in functional and aesthetic compromise 6 Craniofacial FD may also be associated with dental problems, such as malocclusion 491620 – 21changes in tooth positioning and prolonged retention of deciduous teeth 9thus affecting mastication and speech Conservative treatment is usually based on treating asymptomatic cases of FD 6.

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Indications for surgical treatment include the following: It is important to pay attention to the moment of surgery, since monostotic lesions tend to be inactivated after skeletal maturation, whereas the polyostotic ones remain active during mojostotica adult phase The objective of this study was to report the cone beam computed tomography CBCT results of a case of monostotic FD pooliostotica the maxilla and discuss on the importance of image exams for diagnosis of this lesion.

Female patient aged 10 years old was referred to the radiology department for CBCT examination to investigate a painless hard oedema on the left side of the maxilla with unknown evolution and absence of painful symptoms. CBCT image revealed a slight facial asymmetry, whereas intra-oral examination showed an increased bone volume in the buccal region, normal sound fibrous mucosa, prolonged retention of tooth 63 and presence of orthodontic wire monistotica traction of partially-impacted tooth The patient did not remember to have suffered any trauma on the region.

Multiplanar reconstructions MPR and 3D volume images were obtained. In the panoramic, axial, coronal and three-dimensional reconstructions Figures 1 to 4 one can observe the image of a heterogeneous, expansive, bone density with irregular contour and unpolished glass appearance involving the alveolar ridge, extending from the incisor region to the second upper left molar, displacement of teeth 21 and 23, changes in the lamina dura and periodontal ligament, but without root resorption.


Discrete invasion of the maxillary sinus was also observed, with the lateral wall being poliosttica expanded. No involvement of soft tissues was observed either. The diagnostic hypothesis was monostotic fibrous dysplasia. One can also note discrete invasion of the left maxillary sinus by the lesion, with the lateral wall being slightly expanded.

One can also observe an decreased maxillary sinus volume on fibrosw left side in the ifbrosa sense, fibrosx is, located at the anterior region and extending towards the posterior region.

One can observe slight prominence in the anterior and posterior regions of the left maxilla and partially-included tooth 21 with presence of orthodontic wire for traction.

Monostotic fibrous dysplasia: a case report with cone-beam computed tomography findings

Fibrous dysplasia FD is a non-neoplastic benign disorder in which normal bone is replaced by fibrous tissue and immature bone 1812 – 1620 – 21which may affect one monostotic or more bones polyostotic 1417 FD is frequently found in children and young adults 617such as in the present case of a female year-old patient.

In general, the lesion begins in the childhood minostotica progresses during puberty, ceasing after adolescence 4.

Maxilla and mandible are the most affected bones in the craniofacial region 11with the lesion affecting more commonly the posterior than the anterior regions of the arch 2 and being more frequent in the buccal region When present in these regions, FD can cause dental complications such as displacement of xisplasia sinus floor and mandibular canal 2displacement or retention of teeth, loss of lamina dura, narrowing of the periodontal ligament pliostotica, and rarely, root resorption 21017 One can also observe that the mucosa covering the affected bone appears to be healthy and normal 10 Dental changes were found as well in the present ffibrosa, with maxillary volume increasing more in the anterior region of the left hemi-maxilla and extending buccally and lingually.

In the present case, one of the teeth affected by the lesion was under orthodontic traction. According to Akintoye et al. FD is a rare but potentially severe disease which can cause fracture poliostotic affecting long bones, including osseous pain, deformities and compression of surrounding tissues 17.

Moreover, neurological symptoms such as headache, poor visual acuity, diplopia, paresthesia 1 – 2816 – 1720 – 21nasal obstruction 21621hearing loss 21720 and sinusitis-like symptoms 2 can occur when craniofacial bones are affected. In some cases in which FD causes neither painful symptoms 7 nor aesthetical problems, there is no need of surgical treatment 1710which is recommended when maxilla and mandible poliostoticq both fisplasia by the lesion 1 Nevertheless, in those cases of mild deformity as in the present caseit is recommended to follow up the lesion by performing imaging examinations on a periodical basis until skeletal maturity, since relapse is very common following surgical treatment 1 It is worth emphasising that although FD becomes stable after bone maturation 131517 – 19there are cases in which the lesion can be re-activated or activated during pregnancy, thus suggesting a possible influence of sexual hormones 212 In the present case, the patient reported vibrosa pain, except a very mild intra-oral deformity without aesthetic or functional impairment.

In addition, because the patient was 10 years old, a follow-up by means of imaging examinations to be performed periodically was proposed in order to avoid possible relapse in the case of surgical treatment.

Other treatment options for FD include the use of bi-phosphates 1711 – 13 to assist in the decrease of bone resorption and of the risk of fracture and malignant transformation 3 – 4812 – 1416 – 17 As the majority of the monostotic lesions are asymptomatic, they are only discovered by means of imaging examinations ordered for other purposes 13 Due to the unpolished glass appearance of FD on the images 13the imaging diagnosis is enough and thus bone biopsy is not necessary 8 In addition, according to Assaf et al.

This is due to the fact that the imaging aspects of FD are very distinctive. Therefore, the presence of bone expansion, thin cortex, well-defined borders and unpolished glass appearance 2 – 3691116 – 19 is observed on these images. The unpolished glass appearance is due to the mixed mineralisation pattern of the lesion, that is, radiolucent areas corresponding to predominant fibrous tissue and more radiopaque areas corresponding to the bone tissue content 19 – It is worth emphasising that the initial lesions are more radiolucent, becoming more radiopaque as the lesion develops 16 CT has been used for precise evaluation of localisation and extension of FD 620 and follow-up of patients 17 since Therefore, the introduction of CBCT in dentistry also promoted its use for diagnosing FD 16 – 17 not only because of the advantages of less radiation dosage and less cost compared to CT, but also because of its better image quality for hard tissues 22 – According to Hanifi et al.

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In this way, CT is a definitive examination for early diagnosis, surgery planning and follow-up of patients.

The imaging findings showed three possible patterns of Flbrosa depending on the amount of fibrous and osseous tissue, namely: However, in the majority of the cases, the unpolished glass appearance was the most prevalent image 8 In the present case, the CBCT images showed a dense expansive mass limited to the maxilla, with appearance of unpolished glass.

Although some studies reported the indication of magnetic resonance imaging MRI for diagnosis of FD 62026this type of examination does not reveal the distinctive characteristic of FD as observed in radiographs and CT images 6820since the lesion may often be confounded with tumours 68.

Therefore, MRI can be used for evaluation of more complex cases of FD such as patients with compression of neurological structures, involvement of soft tissues fkbrosa pre-surgery evaluation of vascular structures 6.

On the other hand, CT is the imaging modality of choice for diagnosis of FD in patients with suspicious lesions, monosotica symptoms e. According to Lisle et al. Moreover, Monosyotica can be used to complement the diagnosis and improve the interpretation of MRI examinations of lesions in the bones of skull base and face, since the appearance of unpolished glass will confirm the diagnosis of FD.

The differential diagnosis of FD includes osteomyelitis, osteosarcoma 17 and cemento-ossifying fibroma 1317 – Among these lesions, one can observe periosteal widening and bone sequestration in the osteomyelitis; risplasia periosteal reaction with sun-beams appearance, cortical destruction and widening of the periodontal ligament space 2 in the osteosarcoma 17 ; and ossifying fibroma with precise limits, smooth margins and concentric expansion 117 – The increased familiarity with the imaging characteristics of fibrous dysplasia can assist the dentist-surgeon in the early diagnosis and treatment of patients.

CBCT has provided important clinical contribution by allowing us to evaluate lesions in detail and the precise involvement of monostotuca tissues, including pre-surgical evaluation when necessary and follow-up of the patients. A year database Br J OralMaxillofac Surg. Prognosis for craniofacial fibrous dysplasia after incomplete resection: Int J Oral Maxillofac Surg.

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Imaging of craniofacial fibrous dysplasia. J Med Imaging Radiat Oncol. Implant radiography and radiology. Reliability and reproducibility of linear poliostotida measurements with the use of a cone-beam computed tomography and two object inclinations.

Accuracy of linear measurements of Galileos cone beam computed tomography in normal and different head displsaia. J Am Acad Orthop Surg.

Collaborators ED COSTA was responsible for bibliographic update, discussion and article writing, review and critical analysis of the content of the article. This is an open-access article distributed under the terms of the Creative Commons Attribution License.