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    Developmental Disorder of The Bone

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    Arslan Siddiqui
    The document discusses three developmental disorders of bone: Osteogenesis imperfecta (brittle bone syndrome), Osteopetrosis (marble bone disease), and Cleidocranial dysplasia. Osteogenesis imperfecta is caused by defects in collagen that result in fragile bones that fracture easily. Osteopetrosis results from defects in bone remodeling that lead to excessive, weak bone formation and fracturing. Cleidocranial dysplasia is an autosomal dominant condition characterized by abnormalities of the skull, jaws, clavicles, and teeth including delayed tooth eruption.

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    Developmental Disorder of The Bone

    Uploaded by

    Arslan Siddiqui
    206 views14 pages
    The document discusses three developmental disorders of bone: Osteogenesis imperfecta (brittle bone syndrome), Osteopetrosis (marble bone disease), and Cleidocranial dysplasia. Osteogenesis imperfecta is caused by defects in collagen that result in fragile bones that fracture easily. Osteopetrosis results from defects in bone remodeling that lead to excessive, weak bone formation and fracturing. Cleidocranial dysplasia is an autosomal dominant condition characterized by abnormalities of the skull, jaws, clavicles, and teeth including delayed tooth eruption.

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    Developmental Disorder of the Bone

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    The document discusses three developmental disorders of bone: Osteogenesis imperfecta (brittle bone syndrome), Osteopetrosis (marble bone disease), and Cleidocranial dysplasia. Osteogenesis imperfecta is caused by defects in collagen that result in fragile bones that fracture easily. Osteopetrosis results from defects in bone remodeling that lead to excessive, weak bone formation and fracturing. Cleidocranial dysplasia is an autosomal dominant condition characterized by abnormalities of the skull, jaws, clavicles, and teeth including delayed tooth eruption.

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    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    206 views14 pages

    Developmental Disorder of The Bone

    Uploaded by

    Arslan Siddiqui
    The document discusses three developmental disorders of bone: Osteogenesis imperfecta (brittle bone syndrome), Osteopetrosis (marble bone disease), and Cleidocranial dysplasia. Osteogenesis imperfecta is caused by defects in collagen that result in fragile bones that fracture easily. Osteopetrosis results from defects in bone remodeling that lead to excessive, weak bone formation and fracturing. Cleidocranial dysplasia is an autosomal dominant condition characterized by abnormalities of the skull, jaws, clavicles, and teeth including delayed tooth eruption.

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    At a glance
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    The key takeaways are that the document discusses three developmental disorders of bone: Osteogenesis Imperfecta (Brittle bone Syndrome), Osteopetrosis (Albers-Schoenberg or Marble Bone Disease), and Cleidocranial Dysostosis (Cleidocranial Dysplasia).

    Some characteristics of Osteogenesis Imperfecta include bones being fragile and brittle, fractures being common but usually healing without trouble, thin and bulging skull, and severely affected jaws.

    Some characteristics of Osteopetrosis include excessive bone density, obliteration of marrow cavities, development of secondary anemia due to defective osteoclastic activity, and fractures being common due to weak bone formation.

    Developmental Disorders

    of the Bone

    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.

    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

    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

    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

    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

    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

    Osteopetrosis (Albers Schoenberg


    or Marble Bone Disease)

    There are two types:

    a) Malignant and prograsive inherited as


    autosomal recssive and occur early in
    life with severe bone fragility

    Death usually occurs before buberty

    Osteopetrosis (Albers Schoenberg


    or Marble Bone Disease)

    b) A benign autosomal dominant type

    Bone charges are less severe


    There may be repeated fracture following
    minor trauma

    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

    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

    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

    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

    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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