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

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Title: Developmental%20Disorders%20%20%20%20%20%20%20of%20the%20Bone


1
Developmental Disorders of the Bone
2
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.

3
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

4
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

5
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

6
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

7
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

8
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

9
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

10
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

11
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

12
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

13
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

14
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
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