Onset, Progression, and Plateau of Skeletal Lesions in Fibrous Dysplasia and the Relationship to Functional Outcome

Hart, Elizabeth S; Kelly, Marilyn H.; Brillante, Beth A; Chen, Clara C.; Ziran, Navid; Lee, Janice S.; Feuillan, Penelope; Leet, Arabella I.; Kushner, Harvey; Robey, Pamela Gehron; Collins, Michael T.

doi:10.1359/jbmr.070511

Cited by 130 publications

(108 citation statements)

References 23 publications

(24 reference statements)

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“…The important point is that the patient was symptom free during clinical examinations and panoramic radiography did not reveal any abnormalities either, therefore the trigger for more evaluations was the CBCT for dental implant placement. This reveals the importance of comprehensive and meticulous examinations, before any dental treatment (7,8). In this patient we would prefer following up.…”

Section: Discussionmentioning

confidence: 99%

“…There is no specific treatment for McCune-Albright syndrome and due to the great variety of lesions in this syndrome, the treatments are almost specific to each patient. Generally, the disease stabilizes with the skeletal maturity and for this reason, surgical interventions can be delayed until the skeletal maturity occurs in the adult age (7). Surgery is recommended when there is a probability of a functional cranial nerve deficit, to prevent or limit the permanent loss of function (8).…”

Section: Discussionmentioning

confidence: 99%

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Uncommon Cone-Beam Computerized Tomography Findings in McCune-Albright Syndrome in an Implant Candidate Patient: A Case Report

Khoshhal¹,

Rabienejad²,

Shokri³

et al. 2013

Avicenna J Dent Res

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Introduction:McCune-Albright syndrome is a rare disease, characterized by triad of cafe-au-lait spots, endocrinopathies and fibrous dysplasia. These bone lesions are usually revealed during the first decade of life, together with pain, pathological fractures and secondary deformities. Case Presentation: A 40-year-old female patient presented an opaque lesion at the left mandibular side of face, in a cone-beam computerized tomography (CBCT) view, during the implant placement evaluations. The patient had experienced precocious puberty and had undergone hysterectomy. Unilateral cafe-au-lait spots were present on patient's left side of the face. There was no expansion in intraoral examination. The oral mucosa was also normal. No asymmetry was detected. The analysis of sample histopathology confirmed fibrous dysplasia. Discussion: In this patient we preferred following up. Afterwards, total surgical lesion resection can be performed. After a long-term follow-up, the area may receive an implant.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Uncommon Cone-Beam Computerized Tomography Findings in McCune-Albright Syndrome in an Implant Candidate Patient: A Case Report

Khoshhal¹,

Rabienejad²,

Shokri³

et al. 2013

Avicenna J Dent Res

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“…Other progression forms display rapid growth with bone pain and paresthesia occurring in the region of the inferior alveolar nerve, but also a compression of the optic nerve when the orbit is involved. The lesions typically manifest in early childhood, often appearing as significant bony lesions as early as age 5 [13]. They display progressive growth in youth and less activity in adults due to the apoptosis of the bone marrow stroma cells [14].…”

Section: Discussionmentioning

confidence: 99%

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Angermair¹,

Fretwurst²,

Semper-Hogg³

et al. 2017

Dentistry

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and sclerotic areas. These variable radiographic presentations impede radiographic diagnostics and require for an experienced practitioner to plan correct patient management Therefore, the present case report demonstrates the strongly deviating clinical and radiological manifestations of monostotic and polyostotic forms of fibrous dysplasia (FD) in the facial area and its impact on dental and surgical therapy. Presentation of Case Patient 1A 14-year-old male patient was admitted to the Department of Oral-and Craniomaxillofacial Surgery presenting a painless distension in the anterior lower jaw which appeared approximately 4 month before dental visit. Clinical diagnostics revealed an inconspicuous, pale reddish gingiva in the concerned region. In the right incisor region, a hard, non-compressive, non-displaceable swelling of 4 × 3 cm (Figure 1) was detectable. The patient demonstrated a conservative adequately restored juvenile dentition and good oral hygiene behavior with slightly soft plaque caused by orthognathic multiband brackets. The teeth showed neither loosening nor percussive sensitivity. In Keywords: Fibrous dysplasia; Craniofacial fibrous dysplasia;McCune-Albright syndrome; Cone beam computer tomography; Radiographic diagnostics IntroductionFibrous dysplasia (FD) is a rare, congenital malformation of the bone tissue. The disease occurs as a monostotic form, in which only a single bone is affected, and a polyostotic form involving multiple skeletal locations. Fibrous dysplasia may also occur within the context of McCune-Albright Syndrome, a severe polyostotic disease in which endocrine disorders (e.g., puberty praecox, hyperthyreodism, Cushing's disease) and pigmentation of the skin ("cafe au lait" spots) are present [1,2]. While fibrous dysplasia accounts for approximately 7% of all benign osseous tumors, McCune-Albright syndrome occurs very rarely, with a prevalence of 1/100,000 to 1/1,000,000 [3]. In 70-80% of the cases the disease manifests in a monostatic form in the femur, tibia, and ribs, and in 20-30% as a polyostotic form [4].The pathology is a mutation of the GNAS gene on chromosome 20 in early embryogenesis [5]. This gene encodes a G protein, which leads to dysregulation of the adenylyl cyclase [6]. Overproduction of cyclic adenosine monophosphate (cAMP) leads to a deficient differentiation of bone stem cells [7] and finally to the replacement of the healthy bone matrix by proliferating bone marrow stromal cells. This leads to the formation of the characteristic fibrous lesions [8,9]. Histological lesions are comprised of irregular trabecular formation within a fibrous stroma with bone marrow stromal cells without differentiation in adipocytes and osteoblasts. The bony matrix is replaced by proliferating soft tissue and shows an irregular woven structure that is described to resemble "Chinese character" pattern [8].These changes in the bony structure lead to a characteristic radiological manifestation of the disease in juvenile patients where a "ground glass"-like radiolucency enables a tenta...

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“…Café-au-lait macules are supposed to be the first manifestation, shortly after birth. Nonetheless, FD lesions usually are not apparent before the first years of life (1,6). These lesions commonly lead to bone pain and insufficiency fractures (6).…”

Section: Case Reportmentioning

confidence: 99%

Secondary aneurysmal bone cyst in McCune-Albright syndrome

Tournis

Balanika

Megaloikonomos

et al. 2017

ccmbm

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SummaryPolyostotic fibrous dysplasia in combination with café-au-lait macules and hyperfunctioning endocrinopathies consists of a rare clinical condition termed as McCuneAlbright syndrome. Aneurysmal bone cysts are tumorlike cystic lesions, composed of blood-filled compartments. They may occur as primary lesions or secondary to other pathologies; most commonly giant cell tumors of bone. However, secondary aneurysmal bone cysts in McCune-Albright syndrome are exceptional. We present a 28-year-old female with McCune-Albright syndrome. She experienced precocious puberty at age 3 months. In childhood, she experienced multiple long bone fractures, facial deformity and progressive visual and hearing impairment. One year ago, she experienced a painful, gradually enlarging bone lesion involving the right ilium, pubic and ischial bone with groundglass appearance, septa, marginal sclerosis, endosteal scalloping and blow-out expansion resulting in localized thinning of the cortex. CT-guided needle biopsy of the pelvic lesion showed aneurysmal bone cyst. Selective arterial embolization was recommended, however, the patient and her relatives did not consent to proceed to treatment, and she remained in close surveillance thereafter.

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Onset, Progression, and Plateau of Skeletal Lesions in Fibrous Dysplasia and the Relationship to Functional Outcome

Cited by 130 publications

References 23 publications

Uncommon Cone-Beam Computerized Tomography Findings in McCune-Albright Syndrome in an Implant Candidate Patient: A Case Report

Uncommon Cone-Beam Computerized Tomography Findings in McCune-Albright Syndrome in an Implant Candidate Patient: A Case Report

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Secondary aneurysmal bone cyst in McCune-Albright syndrome

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