Diseases of Jaws and Maxillary Sinus Assoc. Professor Jan Laco, MD, PhD

Diseases of jaws and maxillary sinus 2. Temporomandibular joint 3. Maxillary sinus

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1a. Genetic diseases Osteogenesis imperfecta Achondroplasia Cleidocranial dysplasia Cherubism

1a. Genetic diseases Osteogenesis imperfecta “brittle bone disease“ AD inheritance defect of biosynthesis of collagen I thin bones without cortex -  fragile  multiple fractures  dwarfism + blue sclera + deafness

1a. Genetic diseases Achondroplasia most common genetic skeletal disease 75 % cases sporadic mutation FGFR3 gene mutation short-limbed dwarf with normal trunk and head failure of cartilage proliferation in epiphyses protrusive mandible  malocclusion

1a. Genetic diseases Cleidocranial dysplasia defective formation of clavicles retrusion of maxilla delayed eruption of permanent teeth

1a. Genetic diseases Cherubism AD inheritance, mutation of SH3BP2 gene (chr. 4p16.3)  boys painless symmetrical swelling of angle of mandibles ± maxillae ± eyes turned upwards begins at 2-4 years rapid growth until puberty  regression RTG: symmetrical multilocular radiolucencies Mi: fibrous tissue + hemorrhage + multinucleated giant cells + woven bone treatment: none x conservative (recurrence)

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1b. Metabolic diseases Hyperparathyroidism Rickets primary (hyperplasia / adenoma of PTG) secondary (hyperplasia of PTGs in renal failure) RTG: multilocular cyst-like areas Mi: similar to giant cell granuloma and cherubism Rickets vitamin D deficiency during bone development infancy wide fontanels + bossing of frontal and parietal eminences Gigantism and acromegaly  mandibular condyle  prognathism + macroglossia

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1c. Diseases of unknown origin Paget´s disease of bone Fibro-osseous lesions

1c. Diseases of unknown origin Paget´s disease of bone (osteitis deformans) past middle age common RTG finding x clinically silent ? ethiology (paramyxovirus) ? axial skeleton skull – vault + maxilla jaws: symmetric enlargement of alveolar processes + fusion of teeth with hypercementosis to sclerotic bone  difficult extraction

1c. Diseases of unknown origin Paget´s disease of bone (osteitis deformans) resorption (osteoclasts)  irregular new bone formation (osteoblats) Mi: irregular mosaic bone trabeculae +  osteoblasts and osteoclasts + vascularity result: thick spongy bone + marrow fibrosis complication: osteosarcoma (rare) treatment: calcitonin and/or bisphosphonates

1c. Diseases of unknown origin Fibro-osseous lesions Fibrous dysplasia monostotic polyostotic McCune-Albright´s syndrome Ossifying fibroma (controversial) conventional – 30-35 y, mandible juvenile trabecular – 10 y, maxilla juvenile psammomatoid – 20 y, paranasal sinuses (Cemento) Osseous dysplasia periapical (cemental) osseous dysplasia focal (cemental) osseous dysplasia florid (cemental) osseous dysplasia familial gigantiform cementoma

1c. Diseases of unknown origin Monostotic fibrous dysplasia painless, poorly circumscribed bone swelling in childhood or adolescence (10-30Y) F : M ... 1:1  maxilla  malocclusion RTG: replacement of trabecular bone by ground-glass (orange-peel) texture + NO clear demarcation mutation of GNAS1 gene encoding α-subunit of G-protein self-limiting, usually no treatment required rare complication - osteosarcoma

1c. Diseases of unknown origin Polyostotic fibrous dysplasia several bones, 50% pts. develop lesions of jaws F : M … 3:1 McCune-Albright´s syndrome poly FD + skin pigmentations + sexual precocity Mi: fibrous tissue + woven bone trabeculae without osteoblast linings

1c. Diseases of unknown origin Cemento-osseous dysplasia hamartomatous lesions in vicinity of roots of vital teeth ? of periodontal ligament origin ? same process x differ clinically in extent and RTG incidental findings (dif. dg. ossifying fibroma) periapical cemento-osseous dysplasia F : M … 10 : 1 asymptomatic lesion anterior mandible (lower I + few adjacent teeth) RTG: radiolucent (~ periapical granuloma)  radiopaque

1c. Diseases of unknown origin focal cemento-osseous dysplasia similar to periapical OD x molar teeth florid cemento-osseous dysplasia non-expansile, 2 or more jaw areas middle-aged Black females familial gigantiform cementoma expansile, 2 or more jaw areas, autosomal dominant Mi: cellular fibrous tissue + trabeculae of woven / lamelar bone + cementum-like material  calcification

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1d. Inflammations acute osteomyelitis chronic osteomyelitis alveolar osteitis

1d. Inflammations Acute osteomyelitis sources predispositions periapical infection, open fractures predispositions fractures, radiation damage, Paget disease, leukemia, DM oral anaerobes (Bacteroides, Porphyromonas), mixed infection adult males – mandible, rarely neonates - maxilla clinically severe deep-seating pain, swelling, teeth tenderness, red swollen gingiva, anaesthesia of lower lip, regional lymphadenopathy, fever + leucocytosis RTG: ill-defined radiolucent areas (10th day)+ dense sequestra

1d. Inflammations Acute osteomyelitis suppurative inflammation in marrow spaces  compression of blood vessels (thrombosis)  bone necrosis  sequestrum  subperiostal area  subperiostal abscess  perforation  fistulas to skin and oral mucosa complete restoration possible granulation tissue  fibrous tissue  fibrous bone  compact bone  remodelation treatment: ATB + debridement complications: fracture  chronic osteomyelitis

1d. Inflammations Chronic osteomyelitis Chronic focal sclerosing OM inadequate treatment of acute osteomyelitis de novo by low-grade infection complication of irradiation Mi: scant fibrous tissue with chronic inflammation + fragments of dense bones tissue (mosaic, necrotic) Chronic focal sclerosing OM uncommon, young people with carious lower M1 RTG: radiopaque area below tooth root Diffuse sclerosing OM old Black people, superinfection of florid OD

1d. Inflammations Chronic osteomyelitis with productive periostitis (Garré´s OM) uncommon response to chronic periapical low-grade infection young people, carious lower M1  reactive new bone formation

1d. Inflammations Alveolar osteitis (dry socket) most common painful complication of tooth extraction sources excessive extraction trauma, low blood supply, local anaesthesia, oral contraceptives, after radiotherapy after extraction of M3 (less vascularized bone),  females few days after extraction, mucosa around red and tender destruction of clot by fibrinolytic enzymes – infection and food in contact with bone  bone necrosis  sequestra treatment: irrigation of mild hot antiseptics

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1e. Cysts Odontogenic Non-odontogenic Pseudocysts solitary bone “cyst“ aneurysmal bone “cyst“

1e. Pseudocysts Solitary bone “cyst“ (simple, traumatic) ? etiology unknown F : M … 3 : 2 < 25 years painless swelling of mandible frequently no content RTG: cavity expands between teeth roots Mi: NO epithelial lining + wall: fibrous tissue may regress spontaneously treatment: surgical opening and closure (healing by fibrous tissue follows)

1e. Pseudocysts Aneurysmal bone “cyst“ exceedingly rare in jaws ? etiology unknown between 10 – 20 years painless swelling of mandible RTG: multilocular radiolucency Mi: many blood-filled spaces without lining + wall: fibrous tissue + giant cells treatment: curettage recurrence

1. Diseases of jaws 1a. Genetic diseases 1b. Metabolic diseases 1c. Diseases of unknown origin 1d. Inflammations 1e. Cysts 1f. Tumors

1f. Tumors Odontogenic Non-odontogenic

Non-odontogenic tumors Primary: benign x malignant bone cartilage other Secondary: metastases

Non-odontogenic tumors Benign primary tumors osteoma osteochondroma central giant cell granuloma ossifying fibroma - rare hemangioma - rare

Non-odontogenic tumors Osteoma x Exostosis torus palatinus: posterior midline of hard palate torus mandibularis: lingual aspect of mandible opposite to mental foramen, bilateral hard swelling Mi: compact or cancellous bone Gardner´s syndrome - AD inheritance multiple osteomas + polyposis coli  malignancy

Non-odontogenic tumors Osteochondroma “cartilage-capped osteoma“ hard bony protuberance coronoid or condylar process joint dysfunction

Non-odontogenic tumors Central giant cell granuloma (lesion) benign hyperplastic lesion, developmental ???, reparative ??? < 20 years F : M … 2 : 1 anterior mandible, painless swelling RTG: rounded cyst-like area Mi: vascular fibrous tissue + giant cells + bleeding + hemosiderin treatment: curettage recurrence after incomplete removal

Non-odontogenic tumors diff. dg. of giant cell lesions in jaws giant cell granuloma hyperparathyroidism cherubism giant cell tumor of bone (osteoclastoma) aneurysmal bone cyst

Non-odontogenic tumors Ossifying fibroma uncommon mandibular premolar or molar area F : M … 2 : 1, 20-40Y painless swelling, ± capsule RTG: radiolucent + opaque with well-defined margins Mi: variable cellular fibrous tissue + woven / lamellar bone + cementum-like material treatment: enucleation variants: juvenile trabecular – children, rapid growth, maxilla juvenile psammomatoid – walls of sinonasal cavities

Non-odontogenic tumors Osteosarcoma  malignant  common primary non-odontogenic neoplasm irradiation, Paget´s disease 30 - 40 years, males body of mandible irregular firm swelling developing in months  painfull RTG: irregular bone destruction Mi: osteoid formation + atypical osteoblasts + cartilage + fibrous tissue

Non-odontogenic tumors Osteosarcoma rapid local growth + early metastases (lungs) treatment: radical surgery 50% recurrence within 1st year 5-year survival: 40-70 % ( 5 cm)

Non-odontogenic tumors Chondrosarcoma 45 years anterior maxilla (60%) painful irregular swelling RTG: radiolucency Mi: cartilage tissue with pleomorphic, binucleated chondrocytes + mitoses locally aggressive + metastases (lungs) treatment: radical surgery (radioresistant)

Non-odontogenic tumors Rare tumors Ewing´s sarcoma Multiple myeloma Langerhans cell histiocytosis

Non-odontogenic tumors Secondary tumors – hematogenous metastases breast lung prostate thyroid kidney

Jaw necrosis bisphosphonate osteonecrosis osteoradionecrosis complication of radiotherapy insufficient previous dental care

Bisphosphonate osteonecrosis bisphosphonates (↓ bone resorption), denosumab jaw necrosis + mucosal defect – ulceration previous injury in oral cavity – tooth extraction, … nonhealing painful ulcer on alveolar mucosa + necrosis bone at bottom (sequestrum) + inflammation 2/3 cases … mandible diff. dg.: tumors, chronic osteomyelitis treatment: ATB + sequestrotomy

Diseases of jaws and maxillary sinus 2. Temporomandibular joint 3. Maxillary sinus

2. Temporomandibular joint Trismus - inability to open mouth fully joint inflammation injuries tetanus Ankylosis - permanent limitation of movement fibrosis (irradiation) congenital trauma acute pyogenic arthritis condylar tumors oral submucosal fibrosis, systemic sclerosis

2. Temporomandibular joint Osteoarthritis metabolic defect of art. cartilage - defective old people pain, minimal symptoms

2. Temporomandibular joint Rheumatoid arthritis - multisystemic disease autoantibodies (rheumatoid factors) females, 25 - 45 years symmetrical involvement of smaller joints crepitation, little pain inflammation of synovia - granulation tissue (pannus) Osteochondroma Condylar hyperplasia --------------------------------------------------------- cranial giant cell arteritis pain on mastication (jaw claudication)

Diseases of jaws and maxillary sinus 2. Temporomandibular joint 3. Maxillary sinus

3. Maxillary sinus Inflammation - sinusitis acute x chronic x hypersensitive rhinogenic x odontogenic periapical inflammation of P or M long oro-antral communication pain + percussion sensitivity

3. Maxillary sinus Inflammation Mi: respiratory epithelium  squamous metaplasia thickened basal membrane infiltrate - acute - neutrophils - chronic - lymphocytes + plasma cells - hypersensitive - eosinophils

3. Maxillary sinus Oro-antral communication after extraction of P or M nose-blowing test root or tooth in sinus prolaps of antral mucosa

3. Maxillary sinus Antral cysts from mucous glands asymptomatic disappear spontaneously odontogenic cysts radicular, residual

3. Maxillary sinus Tumors squamous cell carcinoma males > 50 years advanced stage – local spread nose obstruction, nose bleeding paresthesia of infraorbital nerve visual disturbances poor prognosis