Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Oral & maxillofacial pathology - bone pathology 1

6,211 views

Published on

Oral & maxillofacial pathology - bone pathology 1

Published in: Health & Medicine
  • Be the first to comment

Oral & maxillofacial pathology - bone pathology 1

  1. 1. Oral & Maxillofacial Pathology IIOral & Maxillofacial Pathology II DENS 3702DENS 3702 Thursdays, 10:00 – 11:50 am Room DB 132 Course Director: Dr. J. E. BouquotCourse Director: Dr. J. E. Bouquot Room 3.094b; 713Room 3.094b; 713--500500--44204420 Jerry.Bouquot@uth.tmc.eduJerry.Bouquot@uth.tmc.edu Topic: Diseases of JawbonesTopic: Diseases of Jawbones
  2. 2. This presentation is intended for students of Dr. Jerry Bouquot. Designated owners of the photographic images in this lecture retain the copyrights for those images. You are welcome to use this presentation for your learning and studying, and you may share this with your fellow dental students, but permission is not given for the publication of these photos in electronic or other formats. This presentation created byThis presentation created by Dr. J. E. BouquotDr. J. E. Bouquot Dr. J. E. Bouquot, Professor & Chair, Department of Diagnostic Sciences, University of Texas Dental Branch at Houston Jerry.Bouquot@uth.tmc.edu 713-500-4420
  3. 3. Bone DysplasiasBone Dysplasias Acrocephalosyndactyly Binder’s syndrome Cherubism Chondrodysplasia Cleidocranial dysplasia Craniodiaphysial dysplasia Crouzon syndrome (craniofacial dysostosis) Ehlers-Danlos syndrome Enchondromatosis Fibrous dysplasia of bone Frontonasal dysplasia Gardner’s syndrome Seckel syndrome Segmental odontomaxillary dysplasia (hemimaxillofacial dysplasia) Treacher Collin syndrome (mandibulofacial dysostosis) Scurvy Cemento-osseous dysplasia Goldenhar’s syndrome Hadju Cheney syndrome Hemifacial hyperplasia Hemifacial microsomia Hyperparathyroidism Hypophosphatasia Infantile cortical hyperostosis Lipodystrophy Marfan’s syndrome Maxillofacial dysostosis Oculo-dento-osseous dysplasia Osteogenesis imperfecta Osteopetrosis Paget’s disease of bone Pfeiffer syndrome Pierre Robin syndrome Progeria Progressive hemifacial atrophy Pyknodysostosis
  4. 4. Bone DysplasiasBone Dysplasias Name Abnormalities Cleidocranial dysplasia Missing clavicles; excess teeth Craniometaphyseal dysplasia Excess mandibular bone; wide long bones
  5. 5. CleidocranialCleidocranial DysplasiaDysplasia
  6. 6. Cleidocranial Dysplasia Cleidocranial Dysostosis Missing clavicles Supernumerary teeth Defective CBFA1 -- Chromosome 21 -- Guides osteoblastic differentiation -- Guides appropriate bone formation AD inheritance -- 40% spontaneous mutations GALP: -- None -- Childhood and teenage years -- Clavicles, jaws -- Rare
  7. 7. Cleidocranial Dysplasia Clinical Features Missing or partially missing clavicles Drooping shoulders -- Maybe can touch shoulders together Frontal and parietal bossing Delayed closure of skull sutures -- Wormian bones Failure of permanent teeth to erupt Supernumerary teeth
  8. 8. Cleidocranial Dysplasia Histopathology, Treatment Micro: Bone looks normal Permanent teeth lack secondary cementum Treat: Surgical exposure and orthodontic repositioning of teeth Extraction of teeth with denture construction
  9. 9. CraniometaphysealCraniometaphyseal DysplasiaDysplasia
  10. 10. Craniometaphyseal DysplasiaCraniometaphyseal Dysplasia Altered metaphyses of long bones Bony overgrowth of jaws, skull, sinuses AD or AR inheritance Large head, mandible Radiopaque jaws, skull Flat nasal bridge Small sinuses; nasal blockage Deafness Blindness Ocular hypertelorism “Erlenmeyer flask” shape of ends of long bones -- ↓ metaphyseal density Look-alike disorders: -- Sclerosteosis (van Buchem disease) -- Craniodiaphyseal dysplasia
  11. 11. Atypical Bone ProductionAtypical Bone Production Name Abnormality Osteogenesis imperfecta Abnormal collagen production Fibrous dysplasia of bone Marrow fibrosis, then excess bone Paget’s disease of bone Excess osteoblastic activity Cemento-osseous dysplasias Marrow fibrosis, excess bone, cementum Cherubism Marrow fibrosis, giant cells Osteopetrosis Lack of osteoclastic activity
  12. 12. OsteogenesisOsteogenesis ImperfectaImperfecta
  13. 13. Osteogenesis ImperfectaOsteogenesis Imperfecta Brittle Bone DiseaseBrittle Bone Disease Mutation in genes for type I collagen AD inheritance, some AR GALP: -- None -- Infant and young child -- Bone, teeth (opalescent dentin) -- 1/8,000 births
  14. 14. OsteogenesisOsteogenesis ImperfectaImperfecta Bowing of legs, arms Angulation of long bones Deformity of long bones Pathologic fracture Hypermobile joints -- “Double-jointed” Capillary fragility -- Pathologic bleeds Osteopenia
  15. 15. OsteogenesisOsteogenesis ImperfectaImperfecta HistopathologyHistopathology Immature, irregular trabeculae Fibrous background Diminished amount of marrow Teeth have small pulps Shell teeth have large pulps
  16. 16. Osteogenesis ImperfectaOsteogenesis Imperfecta Clinical FeaturesClinical Features –– Head & NeckHead & Neck Hearing deficits Wormian skull Blue sclera Maxillary hypoplasia Rarely: mixed radiolucent/opaque areas of jaws Blue/gray “translucent” teeth -- Opalescent dentin -- Like dentinogenesis imperfecta -- Maybe shell teeth -- Fracture of crown
  17. 17. Osteogenesis ImperfectaOsteogenesis Imperfecta Pathophysiology, TreatmentPathophysiology, Treatment Risk of broken bones Anemia (less marrow) Tooth fractures Treat: Cautious behavior to prevent fracture C-section for birth Shorten dental crowns Overdenture
  18. 18. Osteogenesis ImperfectaOsteogenesis Imperfecta Types ITypes I -- IIII Type I: -- Most common -- Mildest form -- Blue sclera throughout life -- AD inheritance Type II: -- Most severe -- 90% are stillborn or die shortly after birth -- Both AD & AR inheritance is seen
  19. 19. Osteogenesis ImperfectaOsteogenesis Imperfecta Types IIITypes III -- IVIV Type III: -- Second most severe form -- Usually noticed after 6 months -- Sclera often normal -- Most die before adulthood (usually from cardiopulmonary problems from kyphoscoliosis) -- Both AD & AR inheritance Type IV: -- Fractures in 50% at birth -- Blue sclera usually fades with time -- AD inheritance
  20. 20. OsteopetrosisOsteopetrosis
  21. 21. OsteopetrosisOsteopetrosis AlbersAlbers--SchSchöönberg Syndromenberg Syndrome Inherited failure of osteoclastic function -- AD or AR Number of osteoclasts is normal or high -- But no bone resorption -- Gradual thickening of trabeculae/cortex -- Gradual sclerosis of bone GALP: -- None -- Infancy (except adult onset type) -- None -- Prevalence rate: 1/100,000 persons
  22. 22. OsteopetrosisOsteopetrosis HistopathologyHistopathology Thick lamellar bone Forms concentric rings as it fills marrow spaces Thick cortex & trabeculae Small marrow spaces, fibrosed Osteoclasts & osteoblasts Inflammation if also has osteomyelitis
  23. 23. OsteopetrosisOsteopetrosis Clinical FeaturesClinical Features Slow opacification Anemia -- Spaces fill with bone Reduced immune function -- Fewer hematopoietic cells in small marrow spaces Decreased blood flow to bone (ischemia) -- Maybe painful Pathologic fractures Often osteomyelitis, fails to heal -- Primarily a jaw problem Crimping of nerves in foramina: -- Hearing loss -- Vision loss -- Facial palsy Delayed/stopped tooth eruption
  24. 24. OsteopetrosisOsteopetrosis Infantile osteopetrosis -- Malignant osteopetrosis -- Severe (usually die in first decade) -- Most common form -- AR inheritance Adult osteopetrosis -- Benign osteopetrosis -- Not too severe -- Usually no marrow deficit -- Bone pain is common -- AD inheritance Treat: -- Antibiotics to counter fewer hematopoietic cells -- Treat anemias if possible -- Try to prevent dental infection -- Hyperbaric therapy
  25. 25. Fibrous DysplasiaFibrous Dysplasia of Boneof Bone
  26. 26. Fibrous Dysplasia of BoneFibrous Dysplasia of Bone Developmental anomaly Postzygotic mutation of GNAS 1 gene (guanine nucleotide-binding protein, alpha-stimulating activity polypeptide 1) -- Explains regional nature of disease GAL: --None -- 7-20 years of age -- Posterior maxilla -- 85% of cases -- Jaws: among most common sites
  27. 27. Fibrous Dysplasia of BoneFibrous Dysplasia of Bone Clinical FeaturesClinical Features Painless bony enlargement, diffuse -- Cortical expansion and thinning Ground-glass radiopacity -- Or irregular opacities -- Or mixed lucent/opaque Poorly demarcated Lamina dura: -- May be hard to see PDL may be thin Teeth become separated -- Remain viable Growth is slow
  28. 28. Fibrous Dysplasia of BoneFibrous Dysplasia of Bone
  29. 29. Fibrous DysplasiaFibrous Dysplasia Clinical VariantsClinical Variants Monostotic fibrous dysplasia -- One bone involved -- 85% of cases -- Jaws: among most common sites Polyostotic fibrous dysplasia -- Multiple bones involved -- Jaffe-Lichtenstein syndrome -- McCune-Albright syndrome
  30. 30. Fibrous DysplasiaFibrous Dysplasia Clinical VariantsClinical Variants McCune-Albright syndrome: -- Relatively uncommon -- May involve most of skeleton -- Café au lait spots -- Sexual precocity -- Hyperthyroidism -- Pituitary adenoma -- Hockey stick deformity of hip Jaffe-Lichtenstein syndrome -- Only café au lait spots -- FD of bone
  31. 31. Fibrous Dysplasia of BoneFibrous Dysplasia of Bone Clinical VariantsClinical Variants Craniofacial fibrous dysplasia: -- Adjacent maxillary bones only Segmental odontomaxillary dysplasia: -- Single maxillary quadrant -- Radiopaque vertical stripes Becomes more ossified with time Treat: surgical recontouring -- Usually “burns out” -- May require multiple recontouring procedures
  32. 32. Fibrous DysplasiaFibrous Dysplasia HistopathologyHistopathology Irregular immature bony trabeculae Fibrous stroma Few osteoblasts Almost no osteoclasts No capsule Becomes more ossified with time
  33. 33. Fibrous Dysplasia ofFibrous Dysplasia of BoneBone Usually “burns out” with adulthood Treat: surgical recontouring May require multiple procedures
  34. 34. CherubismCherubism
  35. 35. CherubismCherubism Familial Fibrous DysplasiaFamilial Fibrous Dysplasia Developmental anomaly AD inheritance -- Defective gene on chromosome 16 GALP: -- None -- 1-20 years old -- Posterior jaws -- Maybe: ribs and humerus -- Rare
  36. 36. CherubismCherubism HistopathologyHistopathology Loose, immature fibrous stroma Scattered multinucleated giant cells Eosinophilic cuffing -- Around small vessels Sparse, immature bone Like giant cell granuloma Old lesions: -- Densely fibrous -- Fewer giant cells -- More mature bone -- Maybe becomes normal?
  37. 37. CherubismCherubism Clinical FeaturesClinical Features Usually bilateral; maybe 4 quadrants Greatly expansile radiolucencies -- Usually multilocular Usually stabilizes and slowly regress Face appears greatly enlarged “Eyes toward heaven” Painless Tooth buds often pushed out of position Wide alveolus
  38. 38. CherubismCherubism Prognosis; TreatmentPrognosis; Treatment Begins prior to 5 years of age Enlarges until puberty, then “burns out” Face is normal by 25 - 40 years of age Can push tooth buds great distances May develop central giant cell granuloma Maybe: pathologic fracture Treat: none, unless pathologic fracture -- Surgical curettage can be performed -- Irradiation works, but risk of future sarcomas
  39. 39. Paget DiseasePaget Disease of Boneof Bone
  40. 40. Paget Disease of BonePaget Disease of Bone Osteitis DeformansOsteitis Deformans Enhanced bone resorption & deposition -- Possibly from a slow virus -- Paramyxovirus detected in osteoclasts GALP: -- Men -- Late middle age and older -- Maxilla and skull -- 1/100 persons over 45 years of age (but most disease is subclinical)
  41. 41. Paget Disease of BonePaget Disease of Bone HistopathologyHistopathology Chinese character bone -- Mosaic; jig-saw bone Immature trabeculae Abundant osteoblastic activity Lesser osteoclastic activity Fibrous background stroma Many reversal/cement lines in the bone
  42. 42. Paget Disease of BonePaget Disease of Bone Clinical FeaturesClinical Features Irregular radiolucency/radiopacity Bowed legs Maybe: hypercementosis Maybe: bone pain Early stage is radiolucent -- Osteoporosis circumscripta Become more radiopaque over time -- Cotton wool appearance -- Cloud-in-the-sky appearance May get anemia and bleeding -- No hematopoietic tissue/platelets -- Immune deficiency Deafness and visual loss -- Pinched nerves in foramina
  43. 43. Paget Disease of BonePaget Disease of Bone Clinical FeaturesClinical Features Thickened cortex Enlarged bone -- Denture no longer fits -- Hat no longer fits Elevated alkaline phosphatase -- 25%+ above normal Normal calcium and phosphorus Urinary hydroxyproline Osteoarthritis
  44. 44. Paget Disease of BonePaget Disease of Bone Clinical FeaturesClinical Features Lincoln’s beard (black beard) -- Technetium bone scan (scintigraphy) -- Hot (black) mandible Small sinuses Bone pain may mimic toothache
  45. 45. Paget Disease of BonePaget Disease of Bone Prognosis; TreatmentPrognosis; Treatment Seldom causes death Parathyroid hormone antagonists -- e.g. calcitonin, bisphosphonates -- To reduce bone turnover Cytotoxic antibiotics, e.g. plicamycin -- Inhibit osteoclastic activity (used only in severe cases) Aspirin for pain Antibiotics for osteomyelitis (sclerotic, late phase) New dentures or bridgework may be required as maxilla expands May do skull base surgery to relieve nerves, vessels Caution! 1 - 13% risk of osteosarcoma or giant cell tumor -- The former seldom in jaws -- The latter often in jaws
  46. 46. CementoCemento--OsseousOsseous DysplasiaDysplasia Developmental anomalies, sometimes familial Subtypes: Periapical cemento-osseous dysplasia Localized cemento-osseous dysplasia Florid cemento-osseous dysplasia Gigantiform cementomas
  47. 47. CementoCemento--Osseous DysplasiaOsseous Dysplasia Histopathology = Same for All SubtypesHistopathology = Same for All Subtypes Immature bone and globular cementum Fibrous stroma, maybe immature Fibrous capsule, often Relatively avascular No inflammatory cells More calcification with time
  48. 48. Periapical CementoPeriapical Cemento-- Osseous DysplasiaOsseous Dysplasia
  49. 49. Periapical CementoPeriapical Cemento--OsseousOsseous DysplasiaDysplasia Periapical Cemental DysplasiaPeriapical Cemental Dysplasia Developmental anomaly Unknown etiology (inherited?) GAL: -- 14:1 female;male ratio -- 70% in blacks -- 30-50 years old -- Anterior mandible -- Apices of teeth May be part of florid cemento-osseous dysplasia
  50. 50. Periapical CementoPeriapical Cemento--Osseous DysplasiaOsseous Dysplasia Periapical Cemental DysplasiaPeriapical Cemental Dysplasia Radiolucencies at apices Maybe: central opacity Viable teeth Center = 1-2 mm below apex Irregular capsule at periphery Intact PDL Asymptomatic, usually -- May be tender, aching Nonexpansile
  51. 51. Periapical CementoPeriapical Cemento--Osseous DysplasiaOsseous Dysplasia Periapical Cemental DysplasiaPeriapical Cemental Dysplasia More radiopaque over time (years) -- Calcified from center outward Treat: none required If symptomatic: curettage Can extract the tooth -- Lesion is not attached Prevent infections (lesion is avascular)
  52. 52. Focal CementoFocal Cemento-- Osseous DysplasiaOsseous Dysplasia
  53. 53. Focal CementoFocal Cemento--OsseousOsseous DysplasiaDysplasia Developmental anomaly? Etiology: unknown GAL: 90% females Mean age 38 years Whites >> blacks Posterior mandible -- Single site involved
  54. 54. Focal CementoFocal Cemento--OsseousOsseous DysplasiaDysplasia Asymptomatic, usually May be tender, aching Nonexpansile Radiolucent/opaque areas < 1.5 cm. diameter Moderately well demarcated More sclerotic over time
  55. 55. Focal Osseous DysplasiaFocal Osseous Dysplasia With Traumatic Bone CystWith Traumatic Bone Cyst ©Photo: ESTOP, Dr. John Fantasia, Long Island Jewish Hospital, New York, New York
  56. 56. Focal CementoFocal Cemento--OsseousOsseous DysplasiaDysplasia Prognosis; TreatmentPrognosis; Treatment Limited growth Will not resorb with alveolar ridge -- Under denture Treat: none required If symptomatic: curettage Can extract adjacent teeth -- Lesion is not attached Prevent infections -- Lesion is avascular
  57. 57. Florid CementoFlorid Cemento-- Osseous DysplasiaOsseous Dysplasia
  58. 58. Florid CementoFlorid Cemento--OsseousOsseous DysplasiaDysplasia Developmental anomaly? Some familial cases GAL: Females Middle-aged Blacks Usually mandible Multiple quadrants involved
  59. 59. Florid CementoFlorid Cemento--OsseousOsseous DysplasiaDysplasia Radiolucent/radiopaque -- Irregular “capsule” around it Multiple quadrants Maybe: expanded cortex Maybe: tenderness/pain Larger than focal/periapical types Usually some periapical lesions Maybe: surface exposure -- Rather avascular Maybe: -- Traumatic bone cyst(s)
  60. 60. Florid CementoFlorid Cemento-- Osseous DysplasiaOsseous Dysplasia Prognosis; TreatmentPrognosis; Treatment Treat: none required If symptomatic: curettage Can extract the tooth -- Lesion is not attached Prevent infections -- Lesion is avascular
  61. 61. Familial GigantiformFamilial Gigantiform CementomasCementomas
  62. 62. Familial GigantiformFamilial Gigantiform CementomaCementoma Developmental anomaly? AD inheritance -- Variable expression GAL: -- None -- First and second decades -- Posterior mandible -- Whites
  63. 63. Familial GigantiformFamilial Gigantiform CementomaCementoma Radiolucent/radiopaque -- Early: more radiolucent -- Late: more radiopaque Well demarcated Irregular “capsule” Expansile Asymptomatic, usually May be very large Maybe all 4 quadrants -- Facial deformity Impaction of teeth
  64. 64. Familial GigantiformFamilial Gigantiform CementomaCementoma Prognosis; TreatmentPrognosis; Treatment Continues to enlarge until fifth decade Caution: watch for infection! -- It’s rather avascular Treat: when predominantly radiopaque -- Surgical resection -- Recontouring -- May have to repeat
  65. 65. Inflammatory/Ischemic Bone DisordersInflammatory/Ischemic Bone Disorders Name Abnormality Infantile cortical hyperostosis Excess mandibular cortical bone Bone scar Exuberant bone response to infarction Osteitis Inflamed bone Acute osteitis Dry socket, poor bone healing Osteomyelitis, acute/chronic Inflamed bone marrow or cortex Ischemic osteonecrosis Poor blood flow through marrow spaces Condensing osteitis Exuberant bone response to infection
  66. 66. Infantile CorticalInfantile Cortical HyperostosisHyperostosis
  67. 67. Infantile Cortical HyperostosisInfantile Cortical Hyperostosis Caffey Disease; CaffeyCaffey Disease; Caffey--Silverman SyndromeSilverman Syndrome First report: Röske, 1930 AD inheritance 3/1,000 infants Onset 2-4 months (up to 2 years) Mandible>>leg bones>>clavicle>>etc. -- Maybe: multiple bones affected Bilateral swelling of mandible from excess periosteal activity -- Enlarged bone, radiopaque Mild fever Tender mandible, face, extremities Maybe: alveolar soft tissue necrosis No effect on eruption or tooth shape Lasts 4-6 months May have lasting mandibular asymmetry
  68. 68. Bone ScarBone Scar
  69. 69. Idiopathic OsteosclerosisIdiopathic Osteosclerosis Bone Scar; Enostosis; Bone IslandBone Scar; Enostosis; Bone Island Excess bone laid down after bone infarction (or infection?) Etiology: unknown GALP: -- None -- 5-20 years of age -- Mandible (90%), especially first molar/premolar region -- Prevalence: 5%
  70. 70. Idiopathic OsteosclerosisIdiopathic Osteosclerosis Bone Scar; Enostosis; Bone IslandBone Scar; Enostosis; Bone Island Dense lamellar bone Fibrous tissue in small spaces No capsule No inflammation Early lesion: -- Large fibrous spaces -- Immature bone -- Osteoblasts -- Osteoclasts Late: dense bone -- Few fibrous spaces -- Few osteoblasts -- No osteoclasts
  71. 71. Idiopathic OsteosclerosisIdiopathic Osteosclerosis Prognosis; TreatmentPrognosis; Treatment Remains indefinitely once formed -- Rare: diminish in size over time Reaches maximum size in months -- May enlarge until adulthood Does not expand cortex May be through-and-through No treatment required Unless secondarily infected/painful -- Then conservative surgical removal
  72. 72. Idiopathic OsteosclerosisIdiopathic Osteosclerosis
  73. 73. CondensingCondensing OsteitisOsteitis
  74. 74. Focal Chronic SclerosingFocal Chronic Sclerosing OsteomyelitisOsteomyelitis Condensing Osteitis; Ischemic Osteosclerosis?Condensing Osteitis; Ischemic Osteosclerosis? Etiology: exuberant bone “healing” -- After infection, trauma GAL: None Teenagers & young adults Mandible Usually painless -- Sometimes painful or tender Nonexpansile Diffuse radiopacity, no capsule
  75. 75. Focal Chronic SclerosingFocal Chronic Sclerosing OsteomyelitisOsteomyelitis Condensing OsteitisCondensing Osteitis Well demarcated No capsule Diffuse sclerosis Periapical location Nonviable tooth? Usually painless -- Sometimes painful /tender Nonexpansile Micro: -- Mature bone -- Small spaces filled with fibrous tissue
  76. 76. Focal Chronic SclerosingFocal Chronic Sclerosing OsteomyelitisOsteomyelitis Condensing OsteitisCondensing Osteitis Remains indefinitely once formed No treatment need unless painful If painful: surgical curettage

×