Manifested in the Jaws

Diseases of Bone

Sarah Ahmed

Pagets disease

AKA Osteitis Deformans is a skeletal disorder that involves

abnormal bone resorption and regrowth of poor quality bone
which results in deformity

Monostotic or polyostotic

Unclear etiology

MOA:
Wave of osteoclastic activity causing irregular resorption cavities
After a period of time osteoblastic activity increases, forming
woven bone

I.
II.

Pagets disease

Later middle and old age 40yrs

M:F = 2:1

Clinical Features

Pagets disease

Clinical Features

Facial or Jaw pain is uncommon

Ill defined neurologic pain as a result of bone impingement on

foramina and nerve canals

Severely elevated levels of serum alkaline phosphatase during the

osteoblastic phase

High levels of hydroxyproline in the urine

Pagets disease

Radiographic Features

Location: pelvus, femur, skull, vertebrae (infrequent in jaws)

Mx:Mn = 2:1

Bilateral but occasionally affects only one maxilla or the

involvement may be significantly greater on one side.

Pagets disease

Saggital

Coronal

Radiographic features

Pagets disease involving all the cranial

bones and the maxilla and mandible
I. Increase bone density between inner
and outer cortex of the skull
II. Enlargement of mandibular ramus

Pagets disease

Internal structure

Depends on stage of disease:

I.

Early radiolucent resorptive stage

II.

Granular or ground glass appearing stage

III.

Denser more radiopaque appositional late stage

Pagets disease

Internal structure

Pattern of trabeculae

I.

Long trabeculae aligned in linear pattern

More common in the mandible

II.

Short randomly oriented granular pattern

D/D: FD

III.

Rounded radiopaque patches of abnormal

bone cotton wool appearance
D/D: FOD

Pagets disease

Surrounding structures

Skull may increase to 3 or 4 times its normal thickness

When jaws are enlarged the outer cortex maybe thinned but
remains intact

The disease may involve the sinus floor and the boundaries will be
less apparent but the air space is not diminished to a great extent

Lamina dura is less evident and altered

Exuberant irregular hypercementosis develops on a few or most

of the teeth in the involved jaw

The teeth may become spaced or displaced in the enlarging jaw

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Pagets disease

Surrounding structures

PAs show exuberant irregular hypercementosis of roots

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Pagets disease

& Fibrous dysplasia

Similarities:
I.
Granular pattern
II. Cause jaw expansion

Differences:
I.
Pagets disease occur in older group
II. Almost always bilateral
III. Fibrous dysplasia has tendency to encroach antral air space
.

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Pagets disease

& Florid Osseous Dysplasia

Similarities:
Cotton wool appearance

Differences:
FOD Lesions centered above the inferior alveolar nerve canal
FOD lesions have radiolucent capsules
Changes seen in FOD do not affect all of the jaw
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Pagets disease

I.
II.

.
I.

& Metabolic bone diseases

Similarities:
Bone pattern
Bilateral

Differences:
Metabolic diseases do not enlarge bones

How to differentiate Pagets disease?

II.
Specific bone pattern changes
III. Late age of onset
IV. Enlargement of involved bone
V.
Extreme elevation of serum alkaline phosphatase

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Pagets disease

Management

I.

Medications (Calcitonin, Sodium etidronate, Bisphosophonates)

relief pain, reduces serum alkaline phosphatase, osteoclastic
activities.

II.

Surgery to correct deformities and fractures of long bones.

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Pagets disease

Complications

I.

Extraction sites heal slowly.

II.

The incidence of jaw osteomyelitis is higher than for non

affected individuals.

III.

Osteogenic sarcoma develops in about 10% of patients with

polyostotic disease.

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LCH

Histiocytosis X, idiopathic histiocytosis, Langerhans cell disease

Abnormal proliferation of Langerhans cells of histiocytes that are
normally found on the skin which results in a spectrum of clinical
diseases.

Old classification:
Eosinophilic granuloma (solitary)
Hand-Schuller-Christian disease (chronic disseminated)
Letterer-Siwe disease (acute disseminated)

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LCH

New Classification
Non-malignant disorders (ex, unifocal or multifocal eosinophilic granuloma)
Malignant disorders (ex, Letterer Siwe disease and histiocytic lymphoma)

Head and neck lesions are common at initial presentation.

Approximately 10% of all patients with LCH have oral lesions.

Often the oral changes are the first clinical signs of the disease.
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LCH

Eosinophilic Granuloma

Appears in the skeleton (ribs, pelvis, long bones, skull and jaws)

Rare in soft tissue

Older children and young adults

Quick formation and may cause a dull, steady pain

Single focus or multi focal, aggressive disease

Disseminated form may involve:

Multiple bone lesions
Diabetes insipidus
Exophathalmos

I.
II.
III.

Hand Schuller Christian disease

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LCH Eosinophilic Granuloma

Bony swelling

Soft tissue mass

Gingivitis and bleeding gingiva

Pain and ulceration

Loosening or sloughing of the teeth

The lost sockets of the teeth do not heal normally

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LCH

Letterer-Siwe disease

Malignant form of LCH

Occurs in infants <3 yrs

Soft tissue and bony granulomatous reactions disseminate

throughout the body

Lesions of bone are rare

Death usually occurs within several weeks of onset

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LCH

Letterer-Siwe disease

The condition is marked by:

Intermittent fever
Hepatosplenomegaly
III. Anemia
IV. Lymphadenopathy
V. Hemorrhage
VI. Failure to thrive
I.
II.

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LCH

Radiographic features

Divided into alveolar type (multiple), and intraosseous type

(solitary)

Mn > Mx

Post > Ant

Mandibular ramus is a common site of intraosseous lesions.

Solitary lesions of the jaw may be accompanied by lesion in other

bone.

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LCH Radiographic Features

EG lesions varies from moderately to well defined but without

cortication.

The periphery sometimes appears punched out.

The margins may be smooth or somewhat irregular.

The alveolar lesions commonly start in the midroot region of the teeth.
The bone destruction progresses in a circular shape.

After it includes a portion of the superior border of the alveolar

process it may give the impression that a section of the alveolar
process has been scooped out.

The shape of intraosseous lesion may be irregular, oval or round.

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LCH

Radiographic Features

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LCH

Radiographic features

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LCH

Radiographic Features

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LCH

Internal structure & surrounding structures

The internal structure is usually totally radiolucent.

Effects on surrounding structures:

LCH destroys bone

I.
II.

Teeth appear to be standing in space

III.

The lesion does not displace teeth

IV.

Minor root resorption

V.

Periosteal new bone formation

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LCH

Surrounding structures

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LCH

Surrounding structures

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LCH

Differential diagnosis

D/D of alveolar type lesion:

1. Periodontal disease

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LCH

Differential Diagnosis

D/D of alveolar type lesion:

2. SCC

May not be possible by radiograph alone

Borders of LCH are better defined

Multiple lesions in a younger age group usually in the first 3 decades

are more likely to be LCH than SCC, which typically appears as a
single lesion in middle or old age

Superficial resemblance to simple bone cyst but the alveolar crest is

maintained in simple bone cyst and a partial cortex may be present
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LCH

Differential Diagnosis

D/D of solitary intraosseous type lesion:

1. Metastatic malignant neoplasia.

2. Malignant tumors from adjacent soft tissue.

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LCH

Management

Surgical Curettage

Limited radiation therapy

Chemotherapy

The earlier EG of the mandible is diagnosed and controlled, the

fewer teeth are lost due to bone destruction

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