1. Differential diagnosis of Multilocular Radiolucencies – Part 2
Specific learning objective
To know the criteria for defining a multilocular radiolucency.
To enumerate different diseases with multilocular appearance
To know the differentiating radiographic features among them.
DR.S.KARTHIGA KANNAN PROFESSOR ORAL MEDICINE & RADIOLOGY

2. Introduction Multilocular radiolucency Pathogenesis
Multilocular radiolucency classification
Multiple, adjacent, frequently coalescing and overlapping pathologic compartments in bone
True multilocular lesions contains two or more pathologic chambers partially separated by septa of bone
More common in mandible
Multilocular cyst
Odontogenic keratocyst
Simple /Traumatic bone cyst
Aneurysmal bone cyst
Ameloblastoma
Odontogenic myxoma
Central giant cell granuloma
Cherubism
Giant cell lesion of hyperparathyroidism
Central hemangioma

3. Cherubism
Synonyms –
Familial Fibrous Dysplasia
Disseminated Juvenile Fibrous dysplasia,
Familial Multi locular Cystic Disease Of Jaws
Definition – rare autosomal dominant disease with bilateral enlargement of jaws, giving a cherub like appearance.
The cause of cherubism is believed to be traced to a genetic defect resulting from a mutation of the SH3BP2 gene from chromosome 4p16.3.

4. History
Cherubism is a rare genetic disorder that causes prominence in the lower portion in the face.
Cherubism- the name is derived from the temporary chubby-cheeked resemblance to putti.
A putto (Italian: [ˈputto]; plural putti [ˈputti] is a figure in a work of art depicted as a chubby male child, usually nude and sometimes winged.
"Cherubism" was first coined and documented in 1933 by Dr. W. A. Jones

5. Clinical Features Abnormality not seen at birth.
Early childhood – 2 – 6yrs
Lesion grows till 12 – 15 years then regress
Sex predilection – M > F
Site predilection – Mandi > Maxi
Painless, firm, bilateral enlargement of lower face.
Enlargement of sub mandibular lymph node.

6. Clinical Feature Oral Manifestation
Premature exfoliation of primary teeth – delayed eruption of permanent teeth
Displacement of teeth, transposition, rotation – tooth resorption
Teeth appear floating in cystic cavities.
Oral mucosa is intact and of normal color
Serum alkaline phosphatase level is increased,
Eyes raised to heaven appearance - ( swelling of maxilla – stretching of skin of cheek – depress lower eyelid – expose thin line of sclera )
The effects of Cherubism may also interfere with normal jaw motion and speech.
The afflicted person suffer the emotional trauma of disfigurement.

7. Radiographic Feature
Location – bilateral – involve both jaws or mandible – ramus or maxillary tuberosity – epicenter in posterior aspect
Periphery – well defined – corticated.
Internal Structure – fine wispy trabaculae – granular bone – multilocular pattern seen in CGCG.
Surrounding structure – Expansion of cortical boundaries – maxillary sinus involvement – anterior displacement of teeth – tooth bud destruction

8. Differential Diagnosis
Management
1.Central Giant Cell Granuloma (CGCG) - Common in anterior mandible 2. Fibrous dysplasia – unilateral involvement and common in maxilla 3. Multiple Odontogenic KeratoCyst in Nevoid basal cell carcinoma syndrome (NBCCS)
Growth diminishes – as age progresses – conservative surgical management for cosmetic purpose.
This disease is also one of the few that unexpectedly stops and regresses. Normal bone remodeling activity may resume after puberty.

9. Giant Cell Lesion of Hyperparathyroidism
PTH (Parathormone) has role in maintenance of plasma calcium homeostasis
Bone and kidney act as major target organs for PTH
PTH acts on tubular epithelium of kidney and causes phosphorus diuresis and induces increased Ca reabsorption.
With 1,25(OH2)D from kidney increase intestional absorption of calcium

10. Giant Cell Lesion of Hyperparathyroidism
Types of hyperparathyroidism
Primary hyperparathyroidisim – primary hyperplasia or benign or malignant tumor of parathyroid gland
> calcium and alkaline phosphatase
< levels of serum phosphorous
Secondary hyperparathyroidism – parathyroid glands are stimulated to produce more of PTH hormone.
< calcium due to renal osteodystrophy
Tertiary Hyperparathyroidism – after long standing secondary hyperparathyroidism . Increased PTH levels produce increased bone resorption. and a resultant hypercalcemia (> calcium)
Normal serum
Calcium level
9-11mg/dl

11. Clinical features
"Psychic moans" refers to effects on the central nervous system. Symptoms include lethargy, fatigue, depression, memory loss, psychosis, ataxia, delirium, and coma.
A helpful mnemonic, "stones, bones, abdominal groans, and psychic moans," can be used to recall the typical symptoms of hypercalcemia
"Stones" refers to kidney stones , nephrocalcinosis. Metastatic calcification – ectopic calcification in soft tissues like subcutaneous tissues, walls of blood vessels, articular cartilages and joint capsules.
"Abdominal groans" refers to gastrointestinal symptoms, of  constipation, indigestion, nausea and vomiting and peptic ulcers 

12. "Bones" refers to bone-related complications
"Bones" refers to bone-related complications. The classic bone disease in hyperparathyroidism is osteitis fibrosa cystica.
Osteitis fibrosa generalisata (cystica) – it produces cyst like lesions of the jaws and appears like multiple punched out radiolucencies with thin cortices and hazy indistinct trabeculae.
which results in pain and sometimes pathological fractures. 
Subperiosteal erosion of bones – middle phalanges is a radiographic hallmark.
Loss of lamina dura is one of the characteristic feature
Migration and mobility of teeth is common.

13. Salt and pepper sign of the calvaria refers to multiple tiny hyperlucent areas in the skull vault caused by resorption of trabecular bone in hyperparathyroidism.
There is loss of definition between the inner and outer tables of the skull and a ground-glass appearance as well as spotty deossification.
Brown’s tumor- It is a giant cell lesion ocuuring in the bone, denoted by its color brown due to the presence of hemosiderin pigment .

14. Central hemangioma
Hemangioma is a benign tumor characterized by proliferation of blood vessels. Its occurrence within the mandible and maxilla is called Central hemangioma.
Central hemangiomas of bone arise from vessels within the marrow spaces and may comprise arterial and venous vessels
It is a relatively rare condition, but may pose a lethal risk to the patient.
Sex predilection - Females > males
Site predilection - mandible >maxilla
Age predilection - 10 – 20yrs

15. Clinical features
A firm, nonpainful, slow growing bony swelling sometimes associated with a subjective pulsating sensation or throbbing discomfort.1
Numbness or tenderness may also be described.
Localized gingival bleeding - hemorrhage may be evident around cervices of teeth.
When the lesion involves a dentulous segment of bone, mobility of the adjacent teeth may occur. Some other dental findings include:
(1) derangement of occlusion;
(2) displacement of teeth;
(3) supraeruption;
(4) premature exfoliation of primary teeth;
(5) early eruption of permanent teeth as a result of the pressure of expanding lesion

16. Radiographic findings
Langland - reported that the presence of a parallel or tube-like arrangement of radioopaque striae is an important indicator of hemangioma.
Worth described a pattern whereby the trabeculae are arranged in a manner resembling the spokes of a wheel, radiating from the lesion’s center towards the periphery
Patchy, multicystic osteolytic areas may give the lesion a soap bubble or honeycomb appearance.
The classical sunburst or sunray appearance, which is caused by coarse trabaculae perpendicular to the bony surface
Sun ray
Spokes of wheel

17. Differential Diagnosis Management
Central hemangioma is a great mimicker, as it resembles: (1) osteosarcoma; (2) fibrous dysplasia; (3) central giant cell granuloma; (4) ameloblastoma; (5) multiple myeloma; (6) dentigerous cyst; and (7) odontogenic cyst radiographically.
Treatment methods include:
(1) Noninvasive radiotherapy;
Injection of sclerosing and embolizing agents; and
surgical intervention by curettage and radical resection with immediate osseous reconstruction.

18. THANK YOU
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