Fibroosseous Lesions Of Jaws Flashcards
Gene undergoing mutation for FIBROUS
DYSPLASIA
GNAS 1
McCune Albright Syndrome consists of
(a) Cafe au Lait spots (Melanocytes)
(b) Fibrous dysplasia (monostotic or polystotic) (osteoclasts)
(c) Precocious puberty (Endocrine cells)
Jaffe’s Syndrome consists of
Cafe au Lait spots (Melanocytes)
Fibrous dysplasia (polystotic) (osteoclasts)
most common bone affected because of monostotic
fibrous dysplasia
- Ribs
- Jaw bone
Radiographic appearance for fibrous dysplasia:
• Ground glass appearance
• orange peel / peau d orange
• frosted glass appearance
ground glass
appearance is a characteristic feature for
Hyperparathyroidism
< fibrous dysplasia
IOPA of fibrous Dysplasia shows
Finger print appearance
Finger print appearance is seen in
IOPA of fibrous Dysplasia
Histopathology of fibrous dysplasia shows
→ Woven - immature bone seen here
→ cuneiform /chinese letter pattern
→ Alphabet soup pattern
Woven - immature bone seen in
Histopathology of fibrous dysplasia shows
cuneiform /chinese letter pattern
Histopathology of fibrous dysplasia shows
Alphabet soup pattern seen in
-Histopathology of fibrous dysplasia shows
What is contraindicated in fibrous dysplasia and why
Radiotherapy is contraindicated as osteosarcoma can develop
Mazabraud’s Syndrome
fibrous Dysplasia with intramuscular myxomas
fibrous Dysplasia with intramuscular myxomas seen with
Mazabraud’s Syndrome
Most serious complication of polystotic fibrous dysplasia is
.
Osteosarcoma.
Gene mutation in Cherubism
SH3 BP2 gene
SH3 BP2 gene mutation seen in
Cherubism
Eye to Heaven appearance seen with
Cherubism
Radiographic appearance of
Cherobism.
floating tooth apperance
floating tooth apperance
-Radiographic appearance of
Cherobism
Which is the Radiographic grading of
Cherubim.
Arnott Radiographic grading of
Cherubim.
→ Syndromes associated with
Cherubism
• Ramon Syndrome
• Rutherford Syndrome
• Noonan syndrome
The following syndromes are associated with?
• Ramon Syndrome
• Rutherford Syndrome
• Noonan syndrome associated with
Cherubism
Histopathology of cherubism shows
→ Giant cells which are TRAP positive (Tastarate resistant acid phosphatase)
→ Perivascular eosinophilic cutting
→ Giant cells which are TRAP positive (Tastarate resistant acid phosphatase)
→ Perivascular eosinophilic cutting
Seen with
Histopathology of cherubism
Focal Cemento osseous dysplasia site :
Periapical mandibular molar
Periapical cemento osseous dysplasia site :
Mandibular anterior region
Florid Cemento osseous dysplasia site :
Multi quadrant varient of cemento osseous dysplasia
Grenz zone seen in
Ossifying Fibroma
The pathology is separated from surrounding healthy bone by this zone of connective tissue.
What connective tissue zone is present in Ossifying Fibroma
Grenz zone
Types of Juvenile Ossifying Fibroma
- Trabecular type
- Psammamatoid type
Parry Romberg syndrome aka
progressive hemifacial atrophy
progressive hemifacial atrophy aka
Parry Romberg syndrome
Crouzon Syndrome aka
Craniofacial dysostosis Craniosynostosis
Gene responsible for Crouzon Syndrome
Gene: FGFR 2
FGFR 3
frog faces seen in
Crouzon Syndrome
Radiographic feature f
Crouson Syndrome
• Beaten metal appesance
• Copper beaten appesance
• Beaten metal appesance
• Copper beaten appesance seen in
Radiographic feature of
Crouson Syndrome
Tower skull seen in
Apert Syndrome
Mitten hand and sock feet appearance seen in
Apert Syndrome
Treacher Collins
Syndrome aka
Mandibulofacial dysostosis
And
First arch syndrome
Treacher Collins
Syndrome gene
gene: Treachilin a TCOF
Coloboma with normal vision seen in
Treacher Collins
Syndrome
Fish face apperance seen in
Treacher Collins
Syndrome
Triad of Pierre Robin Syndrome
Mandibular micrognathia
• Glossoptosis (OSA) (obstructive sleep apnea)
• Cleft palate
Mandibular micrognathia
• Glossoptosis (OSA) (obstructive sleep apnea)
• Cleft palate
Seen in
Triad of Pierre Robin Syndrome
Brush field spot seen in Iris in
Down Syndrome
Tongue features of
Down Syndrome
• fissured tongue
• Macroglossia.
Paget Disease aka
Osteitis Deformans
Osteitis Deformans aka
Paget Disease
Paget Disease gene
gene: sequestosome I
gene. SQS TMI as
p 62
gene: sequestosome I
gene. SQS TMI as
p 62
Seen in
Paget Disease
Or
Osteitis Deformans
Lion like face known as Leontasis Ossea seen in
Paget Disease
Or
Osteitis Deformans
Cotton wool upperance is radiographic feature of
Paget Disease
Or
Osteitis Deformans
Gorham’s
Disease aka
Phantom bone Syndrome aka Massive Osteolysis
Phantom bone Syndrome aka Massive Osteolysis aka
Gorham’s
Disease
