Developmental Bone Disorders

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Developmental Bone Disorders
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Traumatic (Simple) Bone Cyst

• Idiopathic condition seen in 1st and 2nd decade
• Questionable relation to trauma
• Male predilection posterior mandible
• Well-circumscribed radiolucency with scalloping
  between roots

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Traumatic Bone Cyst

• At surgical exploration, an empty cavity is found
  within the bone
• Difficult to obtain lesional tissue
• Fragments of bone lined by chronically inflamed
  granulation tissue

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Traumatic Bone Cyst

• Empirically, the recommendation has been to enter
  the lesion, establish the diagnosis, then induce
  bleeding
• Supposedly the hemorrhage organizes and the
  lesion heals

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Stafne Cyst

• Lingual mandibular salivary gland depression
• Asymptomatic, discovered on routine panoramic
  radiograph
• Adult males well-demarcated radiolucency below
  the mandibular canal, posterior mandible
• CT scan helps confirm the diagnosis

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Osteoporotic Bone Marrow Defect

• Asymptomatic ill-defined radiolucency in body of
  mandible at old extraction site
• Middle-aged female
• May resemble metastatic disease biopsy is
  sometimes necessary
• Fatty and hematopoietic marrow seen
  microscopically

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Idiopathic Osteosclerosis

• Asymptomatic lesion discovered on routine
  radiographs
• Very radiopaque, no expansion
• Premolar - molar region most common
• Margins may be sharp or blend with adjacent bone
• Dense viable bone microscopically

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Cherubism

• Autosomal dominant condition?
• Detected in childhood
• Painless, bilateral expansion of jaws, especially
  the mandible
• Results in chubby cheeks, suggestive of cherubs
  depicted in Renaissance etchings

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Cherubism

• Radiographically, presents as bilateral
  multilocular radiolucencies of posterior mandible
• Less frequently, maxillary involvement
• Often significant displacement of teeth

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Cherubism

• Edematous, cellular fibrous connective tissue
• Relatively sparse, benign-appearing
  multinucleated giant cells
• Sometimes see perivascular hyalinization

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Cherubism

• Optimal treatment has not been determined
• Surgical intervention has been known to
  accelerate the growth of some lesions
• Many cases seem to involute during puberty

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Osteogenesis Imperfecta

• Several rare disorders of bone characterized by
  defective collagen, which results in abnormal
  bone mineralization
• Bones are very fragile, but the degree of
  fragility varies with the type of OI
• Some are autosomal dominant, others are recessive

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Osteogenesis Imperfecta

• In severe forms, death may result from passage
  through the birth canal
• Blue sclera and dentinogenesis imperfecta may be
  seen as components of OI
• Minimize factors that cause fractures
• Prognosis depends on type of OI and expression of
  the gene

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Osteopetrosis

• Rare inherited bone disease caused by lack of
  osteoclastic activity
• Marrow spaces are filled in by dense bone,
  resulting in loss of hematopoietic precursor
  cells, leading to pancytopenia
• Blindness, fractures and osteomyelitis are common
  in the autosomal recessive form

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Osteopetrosis

• Radiographs show diffuse density of the skeleton
• Thickening of bones of the skull seen on CT
  imaging

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Osteopetrosis

• Treatment consists of transfusions and
  antibiotics when necessary
• Bone marrow transplant has had limited success
• Prognosis is poor for AR form, with many patients
  dying before 20 years of age

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Cleidocranial Dysplasia

• Uncommon autosomal dominant condition
• Affects skull, jaws and clavicles primarily
• Prominent forehead, hypoplastic midface
• Primary dentition is retained because permanent
  teeth do not erupt
• Numerous impacted permanent and supernumerary
  teeth

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Cleidocranial Dysplasia

• Treatment today consists of combined surgical and
  orthodontic care to correct skeletal relations,
  remove supernumerary teeth and bring permanent
  teeth into proper relation
• Prognosis is good - life span of these patients
  is essentially normal

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Osteitis Deformans

• Also known as Pagets disease of bone
• abnormal resorption and deposition, resulting in
  distortion and weakening of bone
• Unknown etiology
• Older patients rare lt40 years of age
• 21 male predilection

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Osteitis Deformans

• Symptoms vary, but bone pain may be present
• Most cases are polyostotic
• Affected bones become thickened and weak
• With involvement of femurs, simian stance
  develops due to bowing of legs

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Osteitis Deformans

• Jaws are involved in 10-15 of affected patients
• Affects maxilla more than mandible
• Cotton-wool appearance radiographically
• Often extensive hypercementosis of teeth

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Osteitis Deformans

• Markedly elevated serum alkaline phosphatase
• Irregular trabeculae with resting and reversal
  lines - mosaic pattern
• Rimmed by osteoclasts and osteoblasts
• Marrow is replaced by vascular fibrous connective
  tissue

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Osteitis Deformans

• Chronic and progressive, but usually not
  life-threatening
• No good therapy
• Patients should be monitored for the development
  of giant cell tumor of bone as well as malignant
  bone tumors, especially osteosarcoma

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Fibrous Dysplasia

• Developmental, tumor-like lesion
• Recent work suggests a post-zygotic mutation of a
  tumor suppressor gene
• Usually presents in the first or second decade
• No sex predilection

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Fibrous Dysplasia

• 80-85 are monostotic (affecting one bone)
• Painless swelling, slow growth
• Jaws are among the most commonly affected bones
• Maxilla is affected more often than mandible

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Fibrous Dysplasia

• Craniofacial fibrous dysplasia represents a
  more severe presentation
• Maxillary lesions may involve the adjacent facial
  bones, including the sphenoid, zygoma and occiput
• Results in marked facial deformity

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Fibrous Dysplasia

• Classic radiographic description ground glass
  pattern
• Poorly defined, blending margins
• Early stages radiolucent or mottled
• With maxillary involvement, obliteration of
  maxillary sinus is common

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Fibrous Dysplasia

• Irregularly shaped trabeculae of immature (woven)
  bone
• Abnormal bone fuses to adjacent normal bone no
  capsule
• Moderately cellular intertrabecular connective
  tissue

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Fibrous Dysplasia

• Two presentations of polyostotic fibrous
  dysplasia
• Jaffe type two or more bones affected, in
  conjunction with café-au-lait spots that have
  jagged borders (like the coast of Maine)

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Fibrous Dysplasia

• The second form of polyostotic fibrous dysplasia
  is the McCune-Albright type
• These patients have two or more bones affected by
  fibrous dysplasia, in addition to café-au-lait
  pigmentation and endocrine disturbances manifest
  as precocious puberty

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Fibrous Dysplasia - Tx

• Small lesions may not need treatment, or may be
  removed by en bloc resection
• Significant cosmetic or functional deformity may
  require an attempt at surgical reduction
• Sometimes the disease stabilizes with skeletal
  maturation

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Fibrous Dysplasia

• 25-50 of surgically treated lesions show
  regrowth, particularly in younger patients
• Malignant transformation to a mesenchymal
  malignancy is rare and usually is reported in
  lesions that have received radiation therapy

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Hyperparathyroidism

• Inappropriate secretion of parathormone
• Primary - due to parathyroid hyperplasia,
  parathyroid adenoma, parathyroid carcinoma
• Secondary - due to renal failure, which is
  responsible for poor calcium retention and
  altered vitamin D metabolism

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Hyperparathyroidism

• Radiographically, loss of lamina dura and
  ground-glass trabecular pattern
• Unilocular or multilocular radiolucencies may
  develop - brown tumor
• Enlargement of jaws may develop in long-standing
  renal failure - renal osteodystrophy

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Hyperparathyroidism

• Histopathologically, brown tumors show vascular
  granulation tissue with extravasated erythrocytes
  and numerous benign multinucleated giant cells
• Microscopically identical to central giant cell
  granuloma

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Renal Osteodystrophy

• Unusual hyperplastic response of the bone in
  patients with poorly controlled secondary
  hyperparathyroidism
• Seen as prominent jaw enlargement in some cases

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Hyperparathyroidism

• Treatment remove the source of the hormone
  secretion if primary
• If secondary, better control of serum calcium.
  Parathyroidectomy may be necessary. Renal
  transplant is another alternative.
• Prognosis Fair

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Cemento-Osseous Dysplasias

• Benign, possibly reactive, process that may
  originate from the periodontal ligament
  fibroblast
• Most commonly seen in African-American females,
  but can affect either sex and any ethnic group

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Cemento-Osseous Dysplasias

• Occurs in a spectrum of severity
• Periapical cemental dysplasia (mild)
• Focal cemento-osseous dysplasia (moderate)
• Florid cemento-osseous dysplasia (severe)

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Periapical Cemental Dysplasia

• Usually detected on routine radiographic
  examination
• Mandibular anterior region, middle-aged
  African-American women
• Initially, radiolucencies at apices of teeth,
  with gradual central opacity developing

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Periapical Cemental Dysplasia

• Diagnosis based on clinical and radiographic
  features
• Treatment none necessary
• Prognosis excellent

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Focal Cemento-Osseous Dysplasia

• Probably confused with a true neoplasm, the
  central cemento-ossifying fibroma, in the past
• FCOD is much more common than CCOF, but they are
  seen in a similar demographic group younger
  adult women
• Also seen more commonly in African-American women

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Focal Cemento-Osseous Dysplasia

• Usually detected on routine radiographic
  examination
• Body of mandible female predilection
• Most common in the 20-40 year age range
• Unilocular radiolucency, with or without
  radiopaque central component
• Swelling or discomfort is unusual

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Focal Cemento-Osseous Dysplasia

• At surgery, the lesion is usually poorly defined
  from the surrounding bone, and multiple small,
  gritty fragments are obtained
• Connective tissue with embedded mineralized
  tissue that resembles either woven bone or
  cellular cementum
• Ginger root shape of the trabeculae

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Focal Cemento-Osseous Dysplasia

• Treatment may be unnecessary, however biopsy is
  often warranted in order to rule out other
  disease processes
• Prognosis is generally regarded as good, although
  a lesion that initially appears as a focal
  process may in fact represent the first sign of
  florid cemento-osseous dysplasia

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Florid Cemento-Osseous Dysplasia

• Most severe expression of the cemento-osseous
  dysplasias
• Middle-aged or older African-American women
• Affects multiple quadrants of the jaws
• Generally asymptomatic, unless overlying mucosa
  ulcerates, resulting in sequestration

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Florid Cemento-Osseous Dysplasia

• Radiolucencies with multiple cotton-wool
  radiopacities in at least two quadrants of the
  jaws
• Lesions become more radiodense with time
• May be associated with traumatic bone cysts

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Florid Cemento-Osseous Dysplasia

• Generally a biopsy is not necessary because of
  the typical clinical presentation
• Submission of the sequestrating fragments shows
  densely mineralized tissue with necrotic debris
  and inflammation

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Florid Cemento-Osseous Dysplasia

• For the asymptomatic patient, careful follow-up
  is recommended, with attempts to maintain the
  dentition
• Difficulty arises when secondary infection
  results in sequestration, requiring debridement
  and antibiotics
• Malignant transformation?