Developmental%20Disorders%20%20%20%20%20%20%20of%20the%20Bone

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Developmental Disorders of the Bone
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Osteogenesis Imperfecta (Brittle
bone Syndrome)

• It is hereditary disease of bone
• Osteoporosis and Osteopetrosis
• In osteporosis
• Bones are fragile and brittle
• It is often fatal if it develops during
  intraurterine life.
• It may also develop during child hood or early
  adult life.

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Osteogenesis Imperfecta (Brittle
bone Syndrome)

• In osteoporosis the long bones are thin with
  narrow poorly formed cortics
• Fractures are common but usually heal without any
  trouble
• The skull is thin and tends to bulge over the ear
• The jaws are severely affected

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Osteogenesis Imperfecta (Brittle
bone Syndrome)

• They consists of small delicate trabeculae
• Cortices of the bone composed of immature woven
  bone Osteoblasts fail to form bone in adequate
  amount
• Other abnormalities are joint hypermobility
• Lax ligaments thin translucent skin, blue sclerae

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Osteogenesis Imperfecta (Brittle
bone Syndrome)

• The basic defect appears to lie in collagenous
  matrix of the bone
• It is suggested that the disturbance of normal
  cross-linking between collagen molecules as
  collagen matures
• It is inherited as an autosomal dominont
• It is often associated with dentinogenesis
  imperfecta

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• It is characterized by excessive density of bones
• Obliteration of marrow cavities
• Development of secondary anemia
• Defect in Osteoclastic activity
• Failure in remodeling of developing bone

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• There is an excessive bone formation which is
  mechanically weak so the fractures are common
• Delayed eruption of teeth
• Osteomyelitis is common complication of tooth
  extraction

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• There are two types
• Malignant and prograsive inherited as autosomal
  recssive and occur early in life with severe bone
  fragility
• Death usually occurs before buberty

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• A benign autosomal dominant type
• Bone charges are less severe
• There may be repeated fracture following minor
  trauma

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• RADIOLOGY shows increase density of bone in all
  skeleton with no distinction between cartical and
  madullary bone
• Base of the skull shows marked radiopacity where
  as the vault is generally less dense

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Osteopetrosis (Albers Schoenberg or Marble Bone
Disease)

• RADIOLOGY
• Mandible is more frequently affect than maxilla
• Density of the bone reaches to the roots of the
  teeth so roots are invisible on X-Ray

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Cleidocranial Dysostosis (Cleidocranial
Dysplasia)

• The disease is transmitted as an autosomal
  dominant trait
• It is characterized by abnormalities of many
  bones
• The disease is particularly of the skull, Jaws
  Clavicle and dental abnormalities

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Cleidocranial Dysostosis (Cleidocranial
Dysplasia)

• In the skull the fontanelles and sutures tends to
  remain open
• Skull appears flat with prominent frontal paratal
  and occipital bones
• Partial or complete absence of clavicles allows
  the shoulders to be brought forwards until they
  meet in midline

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Cleidocranial Dysostosis (Cleidocranial
Dysplasia)

• The maxilla may be under developed with high
  narrow arched palate
• Deciduous dentition tends to retain with delayed
  or non eruption of permanent dentition
• Multiple impactions and supernumerary teeth are
  common specially in a mandibular premolar and
  incisor region
• The roots of teeth are thinner than normal