SAADIA ASHRAF

INTRODUCTION HYPERPARATHYROIDISM OSTEOPOROSIS OSTEOMALACIA LEUKEMIA LANGERHANS CELL DISEASE PAGETS DISEASE MULTIPLE MYELOMA RARITIES REFERENCE

CONTENTS

• Maintenance of normal bone density or mass requires normal function and the interaction of a number of intricate and delicate systems:osseous endocrine hematopoetic ,renal gastrointestinal,nutritional and neurocirculatory.

• Radiographs reveal a considerable variation in the density of normal jaw bones.

• All the rarefying diseases included here represent a disruption of bone homeostasis that may result from an imbalance among the factors noted or a direct influence of a disese process on the bone itself.

INTRODUCTION

HYPERPARATHYROIDISMHyperparathyroidism is an endocrine abnormality in which there is an excess of circulating parathyroid hormone(PTH).

TYPES

• Primary hyperparathyroidism

Results from hyperplasia or benign tumour of one of the four parathyroid glands , resulting in production of excess PTH.

• Secondary hyperparathyroidism

This results from compensatory increase in the output of PTH in response to hypocalcaemia.

Tertiary Hyperparathyroidism

Occasionally,the parathyroid tumour after long standing secondary hyperparathyroidism may develop a condition called tertiary hyperparathyroidism.

Ectopic hyperparathyroidism

Caused due to excessive parathyroid hormone synthesis in a patient with malignant disease.

CLINICAL FEATURES

More common in females

Occurs mainly in 30-60 years of age

Classis triad and signs of hyperparathyroidism are described as having “stones , bones, and abdominal groans”

Stones refers to the development of renal calculi (kidney stones,nephrolithiasis) because of the elevated serum calcium levels

Metastatic calcifications involving blood vessel walls,subcutaneoussoft tissues, sclera, dura and the regions around the jaws

Bones refers to variety of osseous changes that may occur in conjunction with hyperparathyroidism.

Bone deformities such as bending of long bones,occasional pathologic fractures,collapse of vertebrae and pigeon chest

Loosening and drifting of teeth leading to malocclusion with diastemaformation.

Abdominal groans refers to the tendencyfor the development of duodenal ulcers.

Palatal enlargement associated with secondary hyperparathyroidism.

RADIOGRAPHIC FEATURES

Radigraphically observable changes are seen in 1 out of 5 patients

Earliest changes are subtle erosions of the bone from the periosteal surfaces of the phalanges from hand

Overall demineralization of the skeleton resulting in overall grayness and lack of normal contrast on the radiograph

Generalized osteoporosis

In prominent hyperparathyroidism ,the entire calvarium has a granular appearance of the bone ,caused by loss of central trabeculaeand thinning of cortical tables

Osteitis fibrosa cystica or osteitis fibrosa generalisata are localized regions of bone loss produced by osteoclastic activity,resulting in loss of all apparent bone structure. These appear cyst like on radiograph

Brown tumor occur late in the disease .

A change in normal trabecular pattern gives ground glass appearance of numerous ,small , randomly orientedtrabeculae.

Periapical radiographs reveal loss of laminadura.

Brown tumour

DIFFERENTIAL DIAGNOSIS

UNILOCULAR Post extraction socket Primordial bone cyst

MULTILOCULAR Pagets disease Ameloblastoma Central giant cell granuloma Cherubism Aneurysmal bone cyst Osteomalacia Fibrous dysplasia Multiple myeloma

MANAGEMENT

• After successful surgical removal of the causative factor,almost all radiographic changes revert to normal,except for brown tumourswhich heals with bone that is radigraphically more sclerotic than normal

OSTEOPOROSISOsteoporosis is defined as “ a disease characterized by low bone massand micro architectural deterioration of bone tissue leading toenhanced bone fragility and an increase in fracture risk.

There are 2 types:1. primary• Associated with aging process

2. Secondary• Results from nutritional deficiencies,hormonal

imbalance,inactivity or corticosteroid or heparin therapy.

CLINICAL FEATURES• Post menopausal women are at risk

• Bones are fragile and succeptible to fractures

• Congenital form is inherited as an autosomal recessive disorder which isfatal in early life due to massive haemorrhage, anemia and rampantbone infections occurring due to progressive loss of bone marrow andtheir cellular products

• They may notice a gradual loss of height due to shortening of trunk,characterized by attacks of severe pain, aggrevated by movements

• Percussion over the affected vertebrae is painful

• Bone becomes succeptible to osteomyelitis

• Fracture of the jaws is common during tooth extraction with common occurrence of osteomyelitis

• Teeth may show delayed eruption, early loss of teeth, arrested root formation, enamel hypoplasia and are more prone to caries due to poor calcification

• Patients have anemia due to replacement of hemolytic marrow by bone.(RBC count below 1,000,000 per cumm)

RADIOGRAPHIC FEATURES• Results in overall reduction in bone density,which may be observed in

the jaws by using the unaltered density of the teeth in comparison.

• Thinning of the cortical boundaries of the inferior mandibular cortex

• Reduction in the volume of cancellous bone

• Lamina dura appear thinner than normal

• Anatomical shadows such as the nasal fossa and maxillary sinus are less distinct

• Wedge shaped appearance of the effected vertebrae

Wedge shaped fracture of vertebrae

Panoramic view showing generalized granular appearance of the trabeculae involving the entire maxilla and mandible.

DIFFERENTIAL DIAGNOSIS

Infantile cortical hyperostosis

MANAGEMENT

• Estrogen , calcium and vitaminD supplement after menopause

• Weight-bearing exercise programs are also effective

OSTEOMALACIA• “Rickets” in children and “osteomalacia” in adults embrace a

group of disorders characterized by an accumulation of osteoid in place of mineralized bone.

• They are caused by deficiency of calcium.

• It may be a result of 1 or a combination of the following:• Vitamin D Deficiency• Calcium malabsorption• Liver and renal disorders• Prolonged anticonvulsive drug therapy• Hypophosphatemic rickets

CLINICAL FEATURES

RICKETS• In the 1st 6 months of life,tetany and convulsions are the

clinical problems resulting due to hypocalcaemia

• This may be followed with swelling of the wrist and ankles

• Craniotabes .a softening of the posterior of the parietal bones may be the initial sign of the disease

• Short stature and deformity of the extremities are common

• Development of dentition is delayed ,

OSTEOMALACIA

• Patient will have bone pain and muscle weakness

• Pelvic deformities are common in females

• They have a peculiar waddling or penguin gait ,tetany and green stick(incomplete) bone fractures.

• Dental abnormalities are rarely seen ,there may be incidence of periodontitis.

RADIOGRAPHIC FEATURESGeneral radiographic features

• Widening and fraying of the epiphyses of the longbones

• Bowing of the weight bearing bones

• Bone cortex may be thin

• Pseudofractures occurs most commonly in the ribs,pelvis and weight bearing bones.

Radiographic features of the jaws

• The jaws cortical structures , such as the inferior mandibular border , follicular walls of the developing teeth and lamina dura are thinned or missing

• Within the cancellous portion of the jaw,the trabeculae become reduced in density ,number and thickness

Radiographic features associated with teeth

• Teeth may be poorly formed,with thin enamel caps and large pulp chamber and root canals.

• Periapical and periodontal abscess occur frequently

Thinning or decreased mineralization of the enamel seen in bitewing view. Large pulp chamber

DIFFERENTIAL DIAGNOSIS

• Hyperparathyriodism

• Rheumatoid arthritis

• Muscle strain

MANAGEMENT

Dietary enrichment of vitamin D and calcium

LEUKEMIA

Definition

Leukemia is a malignant tumour of hematopoietic stem cells.These malignant cells displace normal bone marrow constituents and spill out into the peripheral blood.

CLASSIFICATIONACUTEAcute lymphoblastic leukemia

L1 : acute lymphoblastic (primarily paediatric)L2 : acute lymphoblastic (primarily adults)L3 : Burkitt’s

Acute nonlymphoblastic or myeloid leukemiaM1 : myeloblastic (without maturation)M2 : myeloblastic (with maturation)M3 : promyelocyticM4 : myelomonocyticM5 : monocytic , promonocytes or undifferentiated blastM6 : erythroleukemiaM7 : megakaryocytic

CHRONIC

Chronic lymphatic leukemia involving lymphocytes seriesChronic myeloid leukemia involving granulocyte series

CLINICAL FEATURES ACUTE LEUKEMIA

• More common in young and adult males

• Onset is usually abrupt,with pyrexia and enlargement of spleen

• Patient may complaint weakness ,fever ,headache ,vomiting ,generalized swelling of lymph nodes ,petechae or hemorrhage in the skin and mucous membrane

• Recurrent infection of the lungs,urinary tract,skin mouth rectum and upper respiratory tract.

• Localized tumours consisting of leukemic cells called chloroma,whosesurface turns green when exposed to light are seen.

• Oral cavity shows pallor,ulceration with necrosis petechiae and bleeding

• Gingiva appears boggy,hypertrophic with cyanotic discoloration

• Paresthesia of the lower lip and chin with toothache due to leukemic cell infiltration of the dental pulp.

• There may be associated normochromic anemia ,thrombocytopenia and decrease in the normal functioning of the neutrophils.

CHRONIC LEUKEMIA

• Usually occurs in the older age group

• No presenting signs or complaints

• Patient may complaint of acute left upper abdominal pain ,weakness , fatigue and dyspnea on exertion ,petechiae , ecchymosis and hemorrhage on the skin and mucous membrane

• Oral findings are-hypertrophy of the gingiva, with ulceration , necrosis and dark gangrenous degeneration. Swollen tongue.,loosening of the teeth,regional lymphadenopathy

• Shows presence of normocytic and normochromic anemia.

Extensive hemorrhagic enlargement of the maxillary and mandibular gingiva.

The ulcerated soft tissue nodule of the hard palate represents leukemc cells that have proliferated in this area

RADIOGRAPHIC FEATURES

• The lesions are seen as patchy areas of radiolucency which may later coalesce to form generalized areas of ill-defined radiolucency of the bone

• The lesions are usually bilaterally present

• Foci of leukemic cells may be present as a mass (chloroma) which may behave like a localized malignant tumor. These are rare in the jaws

• Developing teeth in their crypts and teeth undergoing eruption may be displaced in the occlusal direction or into the oral cavity before root development resulting in the premature loss of teeth

• If the lesion involves periodontal structures, the crestal bone may also be lost

Periapical radiographs of left mandible illustrating multi focal areas of bone destruction and widening of portions of periodontal ligament space.

Occlusal displacement of developing mandibular second molar out of its follicle.

DIFFERENTIAL DIAGNOSIS

• Metabolic disorders : these cause generalized rarefaction of the bone.May be differentiated on the basis of the blood picture

• Lymphoma, neuroblastoma : the clinical presentation is different and it may be differentiated on the basis of the blood picture

• Rarefying osteitis : careful clinical and radiological examination will show no apparent cause for the same in case of leukemia

MANAGEMENT

Leukemia is primarily treated with a combination of chemotherapywith or without allogenic or autologous bone marrowtransplantation

LANGERHAN’S CELL DISEASE

Definition

Disorders included in this category are abnormalities that result from the abnormal proliferation of langerhan’s cells or their precursers.

These are classified into 3 distinct clinical forms :

1. Eosinophilic granuloma (solitary)2. Hand-Schuller-Christian disease (chronic disseminated)3. Letterer-Siwe disease (acute disseminated)

The acute disseminated form ; the most aggressive and lethal can produce a generalized rarefactions of the bones

CLINICAL FEATURES• Often occurs in infants below 3 years of age

• Initial onset is manifested as skin rash involving the trunk,scalp and extremities.

• The rash may be erythematous,purpuric and ecchymotic and sometimes it may be ulcerative.

• Soft tissue and bony granulomatous lesions are disseminated through out the body.

• Oral cavity is affected with multiple ulcers and enlargement of gingival tissues.

• Diffuse destruction of maxilla and mandible leading to loosening and premature loss of teeth.

RADIOGRAPHIC FEATURES

• Rarified appearance of jaw bones closely mimic leukemic changes.

• Bone destruction in Langerhans cell disease commences at alveolar crest .

• Extrusion of teeth , thinning of the cortices, and lamina dura and crypt and tooth involvement may be present in Langerhans cell disease.

• New bone formation in the form of proliferative periostitis is commonly seen in juvenile cases.

Marked bone loss resulting in “floating-in-air “ appearance

Severe bone loss that resemble advanced periodontitis.

DIFFERENTIAL DIAGNOSIS

Periodontal disease Traumatic bone cyst Cherubism leukemia Multiple myloma

MANAGEMENTSurgical curettage and limited radiation therapy.disseminated disease is treated with chemotherapy.

PAGETS DISEASEDEFINITIONThis is a condition of resorption and apposition of osseous tissue in one or more than one bone simultaneously

CLINICAL FEATURES

• Disease of later middle and old age, more in males

• It affects the maxilla more than the mandible and the disease is bilateral

• The affected bone is usually enlarged and commonly deformed resulting in bowing of the legs ,curvature of the spine and enlargement of skull.

• When affected the jaws also enlarge,with increase in alveolar width. Mouth remain open as lips are too small to cover the jaws.

• Movement and migration of the affected teeth,malocclusionand in edentulous cases poor fit of the denture

• Bone pain,more often in the weight bearing bones

• Extraction sites heal slowly.

• Associated complications are osteogenic sarcoma ,osteomylelitis ,giant cell tumour,carcinoma of overlying mucosa and maxillary antrum,pathologicalfractures and facial paralysis.

• Elevated levels of serum alkaline phosphatase.

RADIOGRAPHIC FEATURES

It has 3 radiographic stages.

An early radiolucent resorptive stage• In the mandible,the inferior cortex appears osteoporotic and has a

laminated structure

• Trabeculae may be altered in shape and decreased in number ,they may be long and align themselves in a horizontal linear pattern.

• Root resorption may be seen

• In the skull the early lytic lesion may be seen as discrete radiolucent area termed as osteoporosis circumscripta.

A granular or ground glass second stage:

• The trabeculae become short,with random orientation and have a granular pattern

• There are rounded patches of abnormal bone of greater density ,within the radiolucent bone.

A denser more radiopaque appositional stage:

• The trabeculae may be more organized into rounded radiopaque patches of abnormal bone ,creating a cotton wool appearance.

• Bone is enlarged and nearly 4 times its normal thickness on the lateral radiographs.

Periapical film showing “cotton wool” appearance of the bone

Lateral skull film shows marked enlargement of the cranium.

• Irregular enlargement of the alveolar process,which become prominent and bulge.

• Pagets disease in the maxilla may encroach the maxillary sinus

• Hypercementosis is seen on the involved teeth and the lamina dura and periodontal ligament space is obliterated around the hypercementosedand normal roots resulting in ankylosis.

• Development of osteogenic sarcoma may produce frank dissolution or destruction of the bone

DIFFERENTIAL DIAGNOSIS

Early stage ( radiolucent appearance):

• Giant cell lesions of hyperparathyroidism• Osteoporosis• Osteomalacia• Multiple myeloma

Second stage(mixed radiolucent radiopaque appearance)

• Osteogenic sarcoma• Cementifying and ossifying fibroma• Fibrous dysplasia• Osteoblastic metastatic carcinoma

• Chondroma and chondrosarcoma• Metabolic disease

Advanced stage(purely radiopaque appearance)

• Florid osseous dysplasia• Tori• Osteoma

MANAGEMENT

• managed medically by using either calcitonin or sodium etidronate.

• Surgery may be required to correct the deformities of the bone

• Complications of the disease treated with radiotherapy

MULTIPLE MYELOMA

DEFINITIONIt is a malignant neoplasm of plasma cells of the bone marrow with widespread involvement of the skeletal system including the skull and jaws.

CLINICAL FEATURES

• Common in men between the age of 35 and 70 years.

• Mandible is more commonly involved than the maxilla

• Patient will complain of-fatigue,weight loss,fever,bone pain(lower back pain) and anemia. Hypercalcemia is also seen.

• Intraoral swelling is usually tender and elicite egg shell cracking,tends to be ulcerated and bluish red.

• Intraoral hemorrhage and susceptibility to infection may also be present.

• Oral amyloidosis is a complication of this disease.

RADIOGRAPHIC FEATURES

• Lesions are bilateral,well-defined but not corticated radiolucencies,with an oval or a cystic shape –punched out appearance. It may appear ragged and even infiltrative.

• The inferior alveolar canal looses its cortical boundary. The mandibular lesions cause thinning of lower border of mandible or endosteal scalloping.

Multiple circular radiolucent lesions in the skull

Panoramic image depicting multiple areas of well-defined bone destruction lacking any cortical boundary.

Cropped panoramic image shows a solitary lesion in the condylar neck region and a pathologic fracture

DIFFERENTIAL DIAGNOSIS

Metastatic carcinoma Simple bone cyst Hyperparathyroidism

MANAGEMENT

• Chemotherapy with or without autologous or allogenic bonemarrow transplantation.

• Radiation therapy may be used.

RARITIES

• Acromegaly• Agranulocytosis• Burkitts lymphoma• Central cavernous hemangioma• Congenital heart disease• Cyclic neutropenia• Diabetes• Down syndrome• Erythroblastosis foetalis• Gauchers disease• Hypoparathyroidism• Hypophosphatemia• Hypovitaminosis C

These diseases may produce generalized rarefactions of the skeleton . Either these diseases occur rarely or they seldom produce rarefactions of the jaws or bones

Differential diagnosis of oral and maxillofacial lesions-5th edition -NORMAN K WOOD & PAUL W GOAZ

Oral radiology-principles and interpretation STUART C WHITE & MICHAEL J PHAROAH

Burket’s oral medicine – MICHAEL GLICK

Essentials of oral and maxillofacial radiology -FRENY R KARJODKAR

Oral and maxillofacial pathology-Neville , damm, allen , bouquot

REFERENCE