Year : 2005 | Volume
: 16 | Issue : 4 | Page : 151--152
Maxillofacial fibrous dysplasia
Sumita Mahajan, Mala Kamboj, Karen Baoz
Department of Oral Pathology and Microbiology, Manipal College of Pathology and Microbiology, U.P. King George's University of Dental Science, Lucknow, 226003-Uttar Pradesh, India
Department of Oral Pathology and Microbiology, Manipal College of Pathology and Microbiology, U.P. King George«SQ»s University of Dental Science, Lucknow, 226003-Uttar Pradesh
Fibrous dysplasia is a non- neoplastic hamartomatous developmental fibro osseous lesion of bone. This paper provides a brief overview of fibrous dysplasia in a historic perspective and highlights the controversies in fibrous dysplasia seen in the cranio- maxillofacial lesions. A case report of a young woman is presented who was diagnosed as having maxillofacial fibrous dysplasia, thereby making an attempt to use this term in cases with relatively limited facial bone disease.
|How to cite this article:|
Mahajan S, Kamboj M, Baoz K. Maxillofacial fibrous dysplasia.Indian J Dent Res 2005;16:151-152
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Mahajan S, Kamboj M, Baoz K. Maxillofacial fibrous dysplasia. Indian J Dent Res [serial online] 2005 [cited 2021 Oct 16 ];16:151-152
Available from: https://www.ijdr.in/text.asp?2005/16/4/151/29905
Fibrous Dysplasia is defined as "a benign lesion, presumably developmental in nature, characterized by the presence of fibrous connective tissue with a characteristic whorled pattern and containing trabeculae of immature non-lamellar bone". Eversole defines fibrous dysplasia of craniofacial bones as "a benign, non-neoplastic intranedullary cellular proliferation of fibroblasts, with formation of irregular trabeculac of bone or ovoid calcifications that shows indistinct, non-encapsulated borders".
As early as 1891, von Recklinghausen first described fibrous dysplasia ofbones under the term'Osteitis fibrosacystica'. Freund in 1934 named it as 'osteitis fibrosa localizata/disseminata'. Jacobson in 1937 in his extensive review of jaw bone diseases described it as 'Fibrous Dystrophy' whereas Albright (1937) described it as Albright's syndrome, which is a triad of polyostotic fibrous dysplasia, cutaneous pigmentation and endocrine disturbances. Lichtenstein in 1938 coined the tenn 'Fibrous Dysplasia of bone' and described it as a distinct entity of unknown etiology. Lichtenstein and Jaffe' in 1942, reported different clinical manifestations for fibrous dysplasia that is solitary (monostotic) and multiple (polyostotic) forms. In 1958, Jaffe' suggested the term 'Fibro-osseous Dysplasia' for fibrous dysplasia ,,,,,.
A 35-year-old woman visited the College of Dental Surgery Mangalore with a chief complaint of difficulty in placement of partial denture and pain while wearing the lower denture for the last one year. Patient was unaware of anything unusual until she visited a local dentist six months ago who observed a swelling at the denture site. The dentist took a biopsy from the mandibular lesion and referred the patient to our institution for expert management. The biopsy report came as cemento-osseous lesion.
Oral examination revealed the presence of a swollen edentulous area extending linearly from lower left second premolar to the right first molar. Another swelling was seen in the left posterior region of maxilla extending in buccal plate of upper left first molar up to upper left third molar. Both the swellings were painless in growth. The patient complained of pain in mandible on wearing the partial denture.
Orthopantomo graph revealed radio opacity in relation to the upper left posterior (extending in buccal plate of the left first molar up to the left third molar) region. Another radioluscency with ill-defined borders and areas ofradio opacity was seen between lower left second premolar and right first molar. Margins of this lesion in the radiographs appeared toben on-distinct [Figure 1]
Surgical recontoming of the maxilla and a segmental resection of the mandible was done and the resected bony specimen and spicules were sent to the Department of Oral Pathology Mampal College of Dental Sciences, Mangalore, India for histopathological examination. The histopathological picture of the decalcified sections revealed coarse bony trabeculae with osteocytes, absence of osteoblastic rimming around the trabeculae scattered in a cellular fibrous tissue stroma [Figure 2].
Thus based on the clinical, radiological and histological findings the patient could be diagnosed as having fibrous dysplasia
Historically, the concept of a monostotic form of fibrous dysplasia limited to maxilla has been widely accepted and published. But in reality, it is not true. There is said to be an insidious extension into adjacent facial bones, reminiscent of locally infiltrative spread of a neoplasm from its primary site , . hi this particular case it could be thought that the lesion extended from maxilla to mandible. This peculiar behaviour of fibrous dysplasia as reported by Lichtenstein, actually reflects a programmed "field effect" of abnormal osseous development in congenitally predisposed bone matrix .
Thus, it could be assumed that the abnormal maturation proceeds synchronously (though at varying rates) in the affected bones of the craniofacial complex, rather than centrifugally spreading outward from a point of origin. The silent or sub clinical disease, thus, likely exists in otherwise apparently normal appearing bones such as skull, facial or long bones as well.
Mina' supported a concept, which stated, "when a single bone (monostotic) is affected, it probably represents a forme fruste of the more severe form (polyostotic). He stated that the craniofacial bones affected by fibrous dysplasia are in the following order that is frontal> sphenoid> ethmoid> maxilla> mandible> zygoma> parietal> occipital> temporal .
Mandible, being an anatomically freestanding osseous unit, could be said to be affected by monostotic behaviour. But historical records do not go in favour of it, as several earlier diagnosed cases of monostotic fibrous dysplasiaof mandible were later diagnosed either as polyostotic fibrous dysplasiaorfewasossifying fibroma .
Thus, the term "maxillofacial fibrous dysplasia" could be said to be a more fitting designation for patients with typical, relatively limited facial bone disease.
Craniofacial fibrous dysplasia could be used when there is extensive involvement of facial bones and bones of the skull.This patient could be classified under maxillofacial fibrous dysplasia giving a more definitive picture ofthe area affected in the body.
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