Fibrous Dysplasia - Polyostotic

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waldron classification, brannon and fowler classification, fibroosseous classification, familial gigantiform cementoma, mccune albright syndrome

Text of Fibrous Dysplasia - Polyostotic

Polyostotic Fibrous DysplasiaD. Rama Raju : Annals and Essences of Dentistry; volume II. Issue 1 Jan March 2010

Presented by :

Dr. Kush Pathak

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Contents Case report Discussion Pathology & Pathogenesis Polyostotic fibrous dysplasia Clinical course & Progression Roentogenographic features Monostotic Fibrous Dysplasia Histological features2

Biochemical findings Conclusion Critical Evaluation Review of Article References

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Case Report A 20 year old patient came with a complaint of hard growth on the right side of the upper jaw since 6 months. Growth was small and painless, when first observed by the patient. At the same time he also observed growth on his chest which was first observed by his friends. It was also slow growing.4

Both lesions were asymptomatic.

On General Examination Skin was completely normal, without any pigmentation. No endocrinal dysfunction. Face was slightly asymmetrical, due to growth on right side of the maxilla.

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Patient had good oral hygiene with full set of teeth. Growth was extending mesially from right upper canine to distally up to second molar. Size of the lesion was approximately 4 x 2.5 cm. It was extending superio - inferiorly from cervical level of first molar to beyond the buccal sulcus.6

Growth was irregular in shape and bony hard in consistency. Gingiva over the lesion is normal. An another lesion was present on the right side of the chest on second rib extending up to the fourth rib, medially from mediastinum to 1 cm mesial to mid clavicular live

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Provisional diagnosis : Polyostotic Fibrous Dysplasia

Differential Diagnosis : Osteoma Ossifying Fibroma

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Investigations : IOPA Occlusal view of right maxilla PNS view of skull PA view of chest Serum Calcium level Serum Alkaline Phosphatase slightly raised Biopsy right buccal region

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Radiographic features : IOPA & Occlusal view ground glass pattern Radiopacity at the 2nd rib.

Final Diagnosis Fibrous Dysplasia10

Discussion Fibrous dysplasia usually affects young people and tends to become static after puberty. Without treatment or careful management of the diagnosed cases, severe skeletal deformity can result. There are 2 main clinical types of this disease

Monostotic Involves one bone Polyostotic Involves multiple bones11

Polyostotic is sub classified as : a) Jaffes type, in which several bones of the skeleton are involved, pigmented lesions of the skin (cafau-lait spots). b) Albrights syndrome, a polyostotic form accompanied by pigmented skin lesions and endocrine dysfunction presenting as precocious puberty in females and sometimes other anomalies (Albright et al,1937)12

Pathology & Pathogenesis Histopathological appearance can vary according to the relative maturity of the lesion. It consists of well vascularised, cellular fibrous connective tissue containing irregular trabeculae of immature bone sometimes having a Chinese character appearance. Bone in some lesions or some parts of lesion can show osteoclastic activity that are lined by osteoblasts.13

Moth eaten spicules devoid of osteoblastic activity which can be seen in some lesions, may represent a burned out phase. Calcifications as seen in ossifying fibroma, and minute basophilic and laminated calcifications may be seen. Schlumburger (1946) suggested that infection or trauma might play a role in some cases. But it is possible that there is genetic predisposition to this defect involving osteogenic mesenchymal tissue.

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Greco and Steiner (1996) examined fibrous dysplasia cases and suggested that abnormal osteoblastic maturation of the bone forming mesenchyme has one of the most important alterations in this condition.

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Polyostotic Fibrous Dysplasia fibrous The first recognized case of polyostoticdysplasia with skin and endocrine lesions was in 1922 by WELL.

Types : Jaffes type fibrous dysplasia involving a variable number of bones, where most of the skeletal bones are normal and are accompanied by pigmented lesions of the skin or Caf au lait spots. Albrights type more severe type involving nearly all bones in the skeleton accompanied by 16 pigmented skin lesions and in addition having

Warrick, in 1973, evaluated some endocrinal disorders with polyostotic fibrous dysplasia Sexual Precocity Accelerated skeletal growth with premature fusion of epiphyses Goiter & hyperparathyroidism Cushings syndrome Gynaecomastia Diabetes mellitus Acromegaly17

Clinical Features : Occurs early in life. Recurrent bone pain is most common. Bowing and thickening of long bones with leg length discrepancies. Deformities such as coxavara, shaephars crook, deformity of femur, bowing of tibia, Harvisons groove and intra pelvic protrusions of acetabula 18 were also seen.

Spontaneously, pathologic fractures were commonly seen. Caf au-lait spots on skin were seen most commonly on abdomen, back, thighs.(due to irregular melanin pigmentations) Albrights type Precocious puberty was seen in females. Vaginal bleeding with advent of menarche during 19 4th month of life have been seen.

Early development of breasts. Variety of disturbances involving various endocrine glands. Bones of face and skull are frequently involved causing an obvious facial asymmetry. Bony, non tender swelling, obstruction or displacement of adjacent structures.20 Expansion and deformities of maxilla and mandible

Roots were often displaced with loss of lamina dura around the teeth. Less frequently multiple jaw lesions with facial asymmetry and titled occlusal plane caused grotesque appearance.

Clinical Course & Progression Extension of existing lesions Appearance of new lesions Increasing the deformity of the involved bones

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Clinical basis behind the roentogenographic changes is, that they are due to rarefaction of medullary portion of the bone.

Roentogenographic Features

Pseudo De. Orange or Ground Glass appearance Alternating areas of granular density and lucency, giving a radiographic appearance resembling the rind of an orange, most common type 40% cases22

Whorled plaque likeThe matrix of the well circumscribed expansive lesion was composed of plaques of amorphous material of radio density, intermediate between bone and soft tissue which, on close examination, are seen to be arranged in a whorled, onion peel or whirl pool pattern and is seen in 20% cases.

Diffuse Sclerotic TypeThis type presented as a homogeneously dense area of involvement, with no clear view of lesion and normal bone.23

Cyst likeOn close examination faint ground glass matrix due to mineralization in fibrous dysplasia.

Pagetoid typeIn this radiologic type of lesion the affected area of bone was markedly expanded and showed alternating areas of radiopacity and radiolucency, such as seen in Pagets diseases of bone.24

Chalky WhiteThe matrix consisted of amorphous radio dense material with sharply, marginated lesions containing whorled plaque like densities rare type

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Monostotic variety of craniofacial region typically presents lesions without a clearly defined boundary. Periphery of the lesion often blends into normal bone and hence it is difficult to clearly delineate the lesion. Unlikely polyostotic variety, monostotic variety is diagnosed only after conducting biopsy.26

Monostotic fibrous dysplasia

Histological features Minimal collagen production Woven bone is arranged randomly with few osteoblasts on the bony trabeculae. Areas of calcification along with resorption cavities containing osteoblasts and few giant cells seen.27

Although resorption and formation of bone seen, it did not result in mature lamellar bone formation. This is characteristic difference between reparative bone and fibrous dysplasia histologically. Multiple islands of cartilage with fluid filled cysts may be seen Cortical thinning is evident28

Biochemical findings Serum alkaline phosphatase may be slightly raised due to increased bone activity.

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Conclusion Fibro-osseous lesions of jaw bones appear with a variety of clinical and radiographic characteristics. As they have varied radiographic appearance, the nature of the lesion can only be confirmed through biopsy. Many serious bone diseases and lesions are manifestated radiographically with characteristic fibro osseous bone changes. But differentiation between the various fibro-osseous lesions i.e., fibrous dysplasia, ossifying fibroma and cementifying fibroma is difficult on the biopsy results alone. A review of the clinical and radiographic features 30 together with the histopathologic changes and

Critical Evaluation No introduction Discussion part was insufficient No chest radiographs images Repetitive texts Hyperparathyroidism repeated on page 23, 1st column, last paragraph Shaephars crook 23 page, 2nd column

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Review Benign fibro osseous lesions are a collection of non neoplastic intraosseous lesions that replace normal bone and consist of a cellular fibrous connective tissue within which nonfunctional osseous structures form.

Contemporary Oral & Maxillofacial Pathology Sapp 32 & Eversole

Classification of Fibroosseous Lesions

Fibro Osseous Lesions of the Jaws; Charles Waldron; 33 J Oral Max. Surg. (51). 1993

Benign Fibro-osseous Diseases of the Maxillofacial Bones 34 A review & DD; Faizan Alawi; Am J Clin Pathol 2002;118

Fibrous dysplasia was first described by Lichtenstein in 1938 & is an asymptomatic regional alteration of bone in which the normal architecture is replaced by fibrous tissue and non functio