Oral and Maxillofacial Pathology II: Bone Diseases

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Questions and Answers

What is the age range for the occurrence of fibrous dysplasia?

4-18 years (correct)

20-35 years

2-10 years

10-25 years

What is the characteristic radiographic pattern of fibrous dysplasia?

Ground-glass appearance

Smoke screen pattern

All of the above (correct)

Ill-defined radiolucent pattern with few faint trabeculae of bone

What is the effect of mutated osteoblasts in fibrous dysplasia?

Formation of abnormal fibrous tissue

Formation of lamellar bone in place of woven bone

Formation of woven bone in place of lamellar bone (correct)

Formation of normal bone marrow

What is the characteristic feature of teeth in fibrous dysplasia?

Narrow PDL space Signup and view all the answers

What is the complication of fibrous dysplasia that is associated with deformation of the orbit?

Deformity of the orbit Signup and view all the answers

What is the treatment approach for fibrous dysplasia?

Plastic surgery after 21 years of age Signup and view all the answers

What is the mode of inheritance of cherubism?

Autosomal dominant Signup and view all the answers

What is the classification of bone diseases that includes fibrous dysplasia?

Developmental Signup and view all the answers

What is the characteristic feature of cherubism?

Developmental jaw swelling that stops with cessation of skeletal growth Signup and view all the answers

What is the characteristic of fibrous dysplasia in the early stages of embryonic development?

Abnormalities in multiple cell types Signup and view all the answers

What is the name of the syndrome resulting from a mutation affecting osteoblasts, melanocytes, and endocrine cells?

McCune-Albright syndrome Signup and view all the answers

What is the term for fibrous dysplasia affecting a single bone?

Monostotic fibrous dysplasia Signup and view all the answers

What is the characteristic of facial fibrous dysplasia?

Affects a single bone in the maxilla Signup and view all the answers

What is the result of a mutation occurring during postnatal life?

Monostotic fibrous dysplasia affecting a single bone Signup and view all the answers

What is the characteristic of Jaffe type fibrous dysplasia?

Multiple bone lesions with cutaneous pigmentation Signup and view all the answers

What is the term for fibrous dysplasia affecting multiple bones?

Polyostotic fibrous dysplasia Signup and view all the answers

Study Notes

Classification of Bone Diseases

Bone diseases can be classified into 7 categories: Developmental, Metabolic, Inflammatory, Cystic, Tumors, Hormonal, and Deficiency

Developmental Bone Diseases

Fibrous Dysplasia: a developmental tumorlike condition characterized by replacement of normal bone by fibrous connective tissue with irregular bony trabeculae (fibro-osseous lesion)

Fibrous Dysplasia

Can occur in three forms: Monostotic, Polyostotic, and Syndrome-related
Monostotic FD: affecting a single bone
Polyostotic FD: affecting multiple bones
Syndrome-related: associated with McCune-Albright Syndrome or Jaffe Type

McCune-Albright Syndrome

Results from mutation of a pluripotent stem cell in the early stages of embryonic development
Characterized by multiple bone lesions (polyostotic), cutaneous (café au lait) pigmentation, and endocrine disturbances

Jaffe Type

Results from mutation of osteoblasts and melanocytes
Characterized by multiple bone lesions (polyostotic) and cutaneous (café au lait) pigmentation

Facial Fibrous Dysplasia

Monostotic fibrous dysplasia affecting the maxilla
Etiopathogenesis: Non-heritable post zygotic somatic mutation in GNAS1 gene
Clinical features: painless, unilateral, slowly growing swelling, proptosis, maxillary sinus obliteration, nasal obstruction, and dental manifestations

Radiographic Features of Facial Fibrous Dysplasia

3 patterns depending on the amount of formed lesional bone: Orange peel pattern, Ground-glass appearance, Ill-defined radiolucent pattern with few faint trabeculae of bone, and Smoke screen pattern

Microscopic Features of Facial Fibrous Dysplasia

Osteoid stage: Resorption of the original bone and replacement by dysplastic fibrous tissue, Trabeculae of osteoid/woven bone are laid down equidistant from each other and are Chinese letter shaped or U,C and W shaped
Osseous stage: Calcification of osteoid occurs & formation of woven bone is complete without osteoblastic rimming, Resorption of the new trabeculae occurs and the whole cycle is repeated

Complications of Facial Fibrous Dysplasia

Malocclusion, Rarely osteosarcoma, Deformity of the orbit, Obliteration of the maxillary sinus

Treatment of Facial Fibrous Dysplasia

Plastic surgery after 21 years of age

Cherubism

Developmental jaw swelling, its growth stops with cessation of the skeletal growth
Etiopathogenesis: Hereditary, AD, Cherubism gene (SH3BP2) which enhances osteoclastogenesis

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Description

This quiz covers the classification and types of bone diseases, including developmental, metabolic, inflammatory, and more. Test your knowledge of oral and maxillofacial pathology with this comprehensive quiz.