Bone Disorders Notes And Short Essays

Oral Medicine Bone Disorders Important Notes

1. Fibrous dysplasia:

• Classification:
  • Monostotic – Only one bone is involved
  • Polyostotic – More than one bone is involved
    • Jaffe’s type – Polyostotic along with cafe-au-lait-skin pigmentation
    • Albright syndrome – is characterized by polyostotic fibrous dysplasia, cafe-au-lait skin pigmentation, and endocrine disturbances

2. Cleidocranial dysplasia:

• It is characterized by abnormalities of the skull, shoulder girdle, jaws, and teeth
• Skull – delayed closure of sutures and wormian bones
• Shoulder – the partial or complete absence of clavicles
• Teeth – prolonged retention of deciduous and delayed eruption of permanent
• Numerous supernumerary teeth are found in the mandibular premolar and incisor areas.

3. Paget’s disease:

• It is characterized by excessive and abnormal remodeling of bone
• Affects the adult skeleton
• Patients suffer from deafness, blindness, and facial paralysis
• There is a progressive enlargement of the skull and maxilla because of which the patient has to change the hats and dentures frequently

4. Cherubism:

• Manifests by the age of 3-4 years
• Painless symmetric swelling of the mandible or maxilla occurs
• Results in chubby face appearance
• The deciduous teeth shed prematurely and numerous teeth are absent
• X-ray shows numerous unerupted teeth floating in cyst-like spaces

Read And Learn More: Oral Medicine Question and Answers

5. Eagle’s syndrome: elongation of styloid process or ossification of stylohyoid ligament leading to

• Dysphagia
• Sore throat
• Otalgia
• Glossodynia

6. Albright syndrome:

• Precocious puberty
• Polyostotic fibrous dysplasia
• Cafe-au- lait pigmentation

7. Marfan’s syndrome:

• Long thin extremities
• Hyperextensibility of joints
• Spidery fingers
• Arachnodactyly
• Bifid uvula
• CVS complications

8. Cafe-au-lait pigmentation:

• It has brownish pigmentation
• Seen in
  • Neurofibromatosis
  • Fibrous dysplasia
  • Peutzjeghers syndrome
  • Hypothyroidism

9. Blue sclera is seen in:

• Osteogenesis imperfect
• Marfan’s syndrome
• Cherubism
• Ehlers Danlos syndrome
• Osteopetrosis
• Fetal rickets

10. Ground glass appearance is seen in:

• Primary hyperparathyroidism
• Monostotic fibrous dysplasia
• Cherubism

11. Cotton wool appearance is seen in:

• Paget’s disease
• Chronic sclerosing diffuse osteomyelitis
• Fibrous dysplasia
• Cemento-osseous dysplasia

12. Hypermobility of joints is seen in:

• Ehler Danlos syndrome
• Marfan’s syndrome
• Osteogenesis imperfecta
• Down’s syndrome

13. Radiographic features in different diseases:

14. Laboratory investigations

Oral Medicine Bone Disorders Short Essays

Question 1. Fibrous Dysplasia.

Answer:

Fibrous Dysplasia Definition:

It is an idiopathic condition, in which an area of normal bone is gradually replaced by abnormal fibrous connec¬tive tissue, which then again undergoes osseous metaplasia, and eventually, the bone is transformed into a dense lamellar bone.

Fibrous Dysplasia Classification:

• Monostotic – Only one bone is involved
• Polyostotic – More than one bone is involved
  • Jaffe’s type: Polyostotic along with cafe-au-lait-skin pigmentation
  • Albright syndrome: Characterized by polyostotic fibrous dysplasia, cafe-au-lait skin pigmentation, and endocrine disturbances

Etiology:

• Developmental – Developmental anomaly occurring during the growth period of life
• Endocrine disturbances – Complex endocrine disturbances
• Liver damage
• Infections
• Trauma
• Abnormal osteoclastic maturation of bone-forming – mesenchyme

Fibrous Dysplasia Clinical Features:

• Age – It commonly occurs in the first and second decade of life
• Sex – It commonly occurs in females

Fibrous Dysplasia Sites:

• Commonly affected sites are
• Skull
• Facial bones: Maxilla more affected than the mandible
• Clavicles
• Pelvic bones
• Long bones – femur, tibia, humerus

Fibrous Dysplasia Presentations:

• Skeletal lesions
  • Unilateral distribution of the lesion
  • Swelling is seen on the affected side
  • Recurrent bone pain is common
  • There may be a cessation of growth but proliferation may occur
  • Spontaneous fracture is a common complication
• Skin lesion
  • Cafe-au-lait pigmentation is seen
  • It consists of irregularly, pigmented, light brown, flat, melanotic spots
• Oral symptoms
  • Slow enlarging, painless, unilateral swelling of the jaw
  • This leads to facial deformity
  • Expansion and distortion of the cortical plate
  • Displacement of teeth
  • Disturbances in tooth eruption
  • Severe malocclusion
    • Maxillary lesions
    • They extend into the maxillary sinus and upto orbital floor leading to exophthalmos proptosis and nasal obstructions
  • Mandibular lesions
  • Protuberances in the premolar-molar area result in increased depth of the jaw
  • Other – Precocious puberty leading to premature vaginal bleeding, breast development, and axillary and pubic hair at the age of 2-3 years.

Fibrous Dysplasia Radiographic Appearance:

• Radio density – Unilocular or multilocular radiolucent lesion
• Borders – Ill-defined
• Lamina dura – Loss of lamina dura
• Teeth – Resorption of roots occurs
• Jaw – Expansion and distortion of cortical plates
• Granular appearance – Surrounding the margins of the radiolucent area, there may be a wider band of increased density, but granular in appearance
• Ground glass appearance – It may demonstrate areas of whorled amorphous partially calcified materials that are well-circumscribed

Fibrous Dysplasia Differential Diagnosis:

• Lesion and their Distinguishing Feature:
  • Traumatic bone cyst – Absence of cortical expansion
  • Aneurysmal bone cyst – Hemorrhagic aspirate
  • Chronic osteomyelitis – Occurs in older age

Fibrous Dysplasia Management:

• Surgical – Removal of lesion surgically
• Osseous contouring – for esthetic purpose

Question 2. Pathogenesis and management of Osteoradionecrosis.

Answer:

Pathophysiology:

• When small particles of high-energy radiation strike tumor cells or normal tissue cells, the cellular water undergoes radiolysis and is converted into free radicals
• These free radicals interact with the DNA of these cells and disrupt them
• This resultant DNA destruction is manifested differently at various levels
• On a cellular level, this is seen as a chromosomal break-age or disintegration
• The cell may then change like
  • The cell may die
  • It may repair its DNA to survive with impaired function
  • It may repair DNA damage and function normally
• The tissues responsible for developing osteoradionecrosis are endothelium, bone, periosteum, and fibrous connective tissue of mucosa, and skin.
• At the tissue level, these radiation effects are seen as endothelial death, hyalinization, and thrombosis of the vessel
• Fibrosis of marrow spaces takes place
• At the organ level, it is seen as a composite tissue that is hypocellular, hypovascular, and hypoxic.
• This irradiated bone is thus unable to replace the nor¬mal cell loss with new cells and wound healing is thus severely compromised
• This creates a wound in which the demand for oxygen is much more than what can be provided
• Therefore there is tissue breakdown and it manifests as a chronic non-healing wound.

Management Of Osteoradionecrosis:

• The patient is made to breathe 100% oxygen via a pilot’s face mask
• The patient is exposed to 24 atmospheres of absolute pressure of chamber compression
• The oxygen exposure is for 90 minutes, once a day for 5 days a week
• Each exposure to hyperbaric oxygen (HBO) is called a dive
• Protocol

Stage 1 – 30 dives
↓
Condition improved
↓
The next 30 dives are given
↓
No improvement
↓
Stage 2 – Sequestrictomy
↓
Mucosal closure
↓
60 dives are given
↓
If the condition doesn’t improve
↓
Stage 3 – 30 dives
↓
Resection
↓
30 dives
↓
After 10 weeks
Additional 60 dives
are given

Oral Medicine Bone Disorders Short Answers

Question 1. Cherubism.

Answer:

Cherubism

It was first described by Jones in 1933

Cherubism Classification:

• Based on the severity and location of the lesion
  • Grade 1 – Affects ramus of mandible
    • This leads to bilateral and symmetrical expansion of bone
  • Grade 2 – Affects ramus and body of mandible maxillary tuberosity
  • Grade 3 – Affects maxilla and mandible entirely

Etiology:

• Autosomal dominant trait
• Hormonal – latent hyperparathyroidism
• Trauma
• Disturbance in bone-forming mesenchyme

Cherubism Clinical Features:

• Age and sex – 2-3 years males are affected
• Site – Angle of mandible bilaterally
• Appearance
  • Bilateral, painless, symmetrical swelling giving a chubby appearance
  • Swelling is firm to hard on palpation
• Associated symptoms
  • Maxillary swelling pressurizes the floor of the orbit
  • This leads to an upward turn of the pupils of the eyes referred to “heavenward look”
  • Difficulty in speech, deglutition, mastication, and respiration
  • Limited jaw movement
  • Expansion and widening of the alveolar ridge
  • Flattening of palatal vault
  • Chronic lymphadenopathy
  • Malocclusion
• Syndrome Associated: Noonan syndrome

Cherubism Radiographic Features:

• Well-defined, well-corticated cyst-like radiolucency of the mandible
• Teeth appear as hanging in the air
• Displacement of the inferior alveolar canal
• Destruction of maxillary autrium
• Expansion of cortical plates
  • Displacement of teeth

Question 2. Cleidocranial Dysplasia.

Answer:

Cleidocranial Dysplasia

It is a hereditary disorder characterized by abnormal growth of the bones in the face, skull, and clavicles with a tendency for the failure of tooth eruption

Cleidocranial Dysplasia Clinical Features:

• Absence or hypoplasia of one/both clavicles
• Hypermobility of shoulder joints
• Elongated frontal and occipital skull plates
• Underdeveloped entire midface
• Delayed closure of fontanelles
• High and narrow arched palate
• Underdeveloped paranasal sinuses
• Photophobia
• Multiple unerupted and impacted teeth

Radiographic Features – Radiograph Reveals:

• Open sutures
• Open fontanelles
• Partial/complete loss of clavicles
• Multiple impacted teeth
• Thin roots of teeth

Cleidocranial Dysplasia Treatment

• No treatment is possible

Question 3. Enumerate diseases with cafe-au-lait.

Answer:

Pigmentation:

• Albright syndrome
• Von Recklinghausen’s Neurofibromatosis
• Bloome’s syndrome
• Fanconi anemia
• Cowden’s syndrome
• Tuberculosis sclerosis
• Watson’s syndrome
• Ataxia telangiectasia

Oral Medicine Bone Disorders Viva Voce

1. Paget’s disease shows the cotton wool appearance
2. The development of osteosarcoma is the most serious complication of Paget’s disease
3. Central giant cell granuloma commonly involves mandible
4. Cherubism gives a floating tooth appearance
5. Cherubism shows eye to heaven look appearance
6. In osteopetrosis, there is endosteal production of bone
7. Mosaic bone is due to partially resorbed and repaired bone
8. Osteogenesis imperfecta is characterized by extreme fragility and porosity of bones