Oral Pathology and Syndrome Pt 2 Flashcards
Achondroplasia: genetics? prevalence? physical appearance?
80% sporadic mutations, AD, syndromic
- 1/20k live births
- Short limbed dwarfism: endochondral bone formation
- enlarged head, depressed nasal bridge
- short, stubby, trident hands
- lordotic lumbar spine
- prominent buttocks
- protuberent abdomen
What are 7 major causes of dwarfism?
- Genetic
- Constitutional delay (hypopituitarism)
- Chronic disease (nutritional deficiency, mucopolycaccharidosis)
- Chromosomal/syndromic (chondroectrodermal dysplasia, turner syndrome, hallerman streiff, achondroplasia)
- Endocrine (hypothyroidism)
- Psychosocial
- Intrauterine
Hypopituitarism: how do they look? why? dental fx? What causes it?
WELL PROPORTIONED body, due to a lack of growth hormone, so they are normal but small.
- Causes: tumors (pituitary, parasellar, suprasellar, or radiation, pitutiary apoplexy) infiltrative disease, etc
- Fine, silky hair, wrinkled atrophic skin
- hypogonadism
- delayed eruption/exfoliation
- malocclusion common due to small dental arches
- Pan hypopituitarism may lead to other systemic problems
Mucopolysaccharidoses
- are a group of metabolic disorders caused by the absence or malfunctioning of lysosomal enzymes needed to break down glycosaminoglycan (which help build bone, cartilage, tendons, corneas, skin and connective tissue)
- these children also become dwarves
- Over time, these glycosaminoglycans collect in the cells, blood and connective tissues. The result is permanent, progressive cellular damage which affects appearance, physical abilities, organ and system functioning, and, in most cases, mental development.
Chrondroectodermal Dysplasia : results in? concurrent problems?
Dwarfism (ie achondroplasia + hidrotic ectodermal dysplasia classic findings of both)
- polydactaly (extradigits, ‘spade shaped hands), exctodermal dysplasia (hidrotic) brittle nails and missing teeth
- Multiple frenae
- Cardiac defects seen in 50%
Hidrotic ectodermal dysplasia is characterized by?
- Hidrotic ectodermal dysplasia is characterized by partial or total alopecia, dystrophy of the nails, hyperpigmentation of the skin (especially over the joints), and clubbing of the fingers.
Oculo-mandibulo-dyscephaly (Hallerman-Streiff syndrome): characterized by what facial characteristics? what would you see on their ceph? Dental? Body?
Dwarfism
- Dyscephaly (malformed head)
- hypotrichosis (abnormal hair patterns, loss)
- microphthalmia
- cataracts
- beaked nose
- severe micrognathia- gonial angle of the mandible is almost straight, very obstuse
- short stature
- may have supernumerary teeth/natal teeth
Name 4 syndromes characterized by a senile like appearance?
- Progeria
- Werner Syndrome
- Cockayne syndrome
- Rothmund-Thomson Syndrome
Hypothyroidism
Short stature if untreated
- Large posterior fontanel
- macroglossia
- hypothermia
- Lethargy
- hypotonia
- bradycardia
- delayed growth and skeletal maturation
Turner’s Syndrome
- chromosomes? prevalence? IQ? Other important findings? dental findings?
45X -1/8000 Females only -near normal IQ -sterile -Coarction of aorta, most common cardiac defect -webbed neck -enamel hypoplasia
Osteogenesis imperfecta: what are the types? what is the severity of each type? Which are assoc w/dentinogenesis imperfecta?
Type 1: mildest form, assoc w/blue sclera (due to collagen defects)
Type 2: Perinatally lethal, severe fragility of connective tissues; multiple in utero fractures
Type 3: Progressive deforming, severe fragility, usually assoc w/in utero fractures
Type 4: similar to type 1 but more severe,
Type 1 and 4 are assoc w/dentinogenesis imperfecta
Congenital indifference to pain: inheritance? what happens?
AR
- Frequent scarring of face w/mutilation of lips, tongue, arms, legs
- mild MR
Lesch Nyhan Syndrome: genetics? concurrent issues? what is it’s cellular basis?
X linked recessive
- MR
- Spastic CP
- choreaoathetosis
- bizarre self mutilating behavior
- absence of hyppoxanthine-guanine-phosphoribosyltransferase
Gingivitis in children: prevalence? increases w/? What about kids is different?
40-90% of children, it is the most common periodontal infection in children/adolescents
- Generally increases with age, eruption, puberty
- Rounded gingival margins of children accentuate inflammatory changes; tissues may become fibrotic
- Does not occur to the same degree as in adults w/comparable plaque
Periodontitis: prevalence? clinically see?
Prevalence of destructive disease in children:
- Age 5-11 years 1-9%
- Age 12-15 years: 1-46%
Clinical attachment loss precedes radiographic bone loss: disease threshold CEJ-ABC >2mm in primary dentition; loss of lamina dura
Aggressive Periodontitis in the permanent dentition: primary and secondary findings? Prevalence? Ethnicities?
Common characteristics of local/generalized forms:
- Primary findings: Rapid bone loss, familial aggregation, genetic predisposition
- Secondary findings: Phagocyte abnormalities, hyper-responsive macrophage phenotype, Reports of disease being self-limiting
- Prevalence in adolescents <1%; higher in african americans and hispanics
Aggressive periodontitis in the primary dentition: Radiographic? clinical? Prevalence? Cellular level what is occuring? tx?
- Bone loss around some of the primary teeth, esp molars
-Inflammation is not prominent - Children are healthy, no evidence of systemic disease
- Prevalence is <1%, higher in African Ams
- Leukocyte chemotactic defect, defect in cementum
Tx: S/RP, or EXT of primary teeth; antibiotics Amoxicillin for 7-10days
Localized aggressive periodontitis in the permanent dentition: radiographic? rate of progression vs adult? clinical? ages? Prevalence by ethnicity? What usually happens before this? Tx?
- Interproximal attachment loss on at least 2 permanent teeth and incisors w/attachment loss on no more than 2 additional teeth
- Radiographic: VERTICAl bone loss around molars; HORIZONTAL bone loss around incisors
- Rate of progression is 3-5x adult periodontitis
- Inflammation is not prominent; very little plaque/calculus
- Children are healthy w/no evidence of systemic disease
- Usually detected between 10-15 years
- Prevalence is .3% overall; 10% of Afr Americans, 5.5% latinos
- 50% preceded by bone loss in the primary dentition
- Defect: PMN chemotaxis, phagocytosis, hyper-responsive monocytes, genetic defect
- Micro: +/- actinobacillus actinomycetemcomitans
Tx: sx and non-sx debridement w/ antibiotics (doxycycline/amox/metronidazole for 7-10days)
Generalized Aggressive Periodontitis: Dx criteria? Radiographic findings? Prevalence (gender/ethnicity)? Clinical findings? Cellular findings?
Generalized attch loss including AT LEAST THREE teeth that are not 1st molars.
- Prevalence: overall .15%; higher in males and African Ams
- Usually dx under 30 yo; less common than localized form and is likely a progression of the localized form
- Clinically: marked periodontal inflammation with heavy plaque and marked calculus accumulation (differs from localized forms)
- Cellular: suppressed neutrophil chemotaxis, subgingival bacterial cultures typically non-motile, facultatively anaerobic, gram negative rods
Predisposing Genetic Traits/syndromes for periodontitis? (11)
- Cyclic neutropenia; chronic idiopathic neutropenia
- Diabetes Mellitus
- Down syndrome
- Leukocyte adhesion deficiency
- Ehlers Danlos syndrome
- Chediak-Higashi syndrome
- Papillon-lefevre
- Acatalasia (AR, lack of peroxidase primary problem is predilection for periodontal disease)
- Dentin dysplasia
- Hypophosphatasia (and hypophosphatasia vitamin D resistant rickets)
- Lesch-nyhan syndrome
Describe Chediak-higashi and papillon lefevre Syndromes?
- Chediak-Higashi syndrome: AR
- arises from a mutation of a lysosomal trafficking regulator protein which leads to a decrease in phagocytosis.
- Resulting in recurrent pyogenic infections, partial albinism and peripheral neuropathy.
- solar sensitivity and photophobia.
- frequent infections and neuropathy are common
- Infections involve mucous membranes, skin, and the respiratory tract.
- Affected children are susceptible microbial infections with Staphylococcus aureus being the most common cause. - Papillon-lefevre: AR and charac by periodontitis and palmoplantar keratoderma. - -
- The severe destruction of periodontium results in loss of most primary teeth by the age of 4 and most permanent teeth by age 14.
- Clinically can see severe inflammation
- Hyperkeratosis of palms and soles of feet appear in first few years of life.
Hypophosphatasia : etiology, phenotypes, severity, dental findings? diagnostic criteria?
Rare genetic metabolic bone disease
- Etiology: low serum alkaline phosphatase which impairs bone mineralization
- Phenotypes range from premature loss of decidious teeth to severe bone abnormalities leading to neonatal death
- The earlier the presentation of symptoms the more severe the disease
- Abnormal cementum, large pulp chambers
- Dx: low serum alkaline phosphatase,
Neoplasms which can result in periodontal disease?
- lymphoma
- leukemia
- langerhan’s cell histiocytosis (histiocytosis x)
- soft and hard tissue neoplasms
Leukocyte adhesion deficiency
AR
- Leukocyte surface glycoprotein defect resulting in poor leukocyte adherence
- Oral manifestation: generalized periodntitis in the primary and young permanent dentition
- Severe generalized periodontitis refractory to tx
- Frequent respiratory, skin, ear, and other soft tissue bacterial infections
Hereditary Hemorrhagic Telangiectasia: inheritance pattern? Clinically? potential complications?related to?
AD
- a congenital cause of erythematous gingiva
- Mucosal and cutaneous telangiectases
- May result in repeated bleeding episodes
- related to Sturge Weber syndrome
What are acquired causes of erythematous gingiva?
Trauma
- Drugs : chlorhexidene, cinnamonaldehye (cinnamon flavor)
- Infectious: candida
- Desquamative gingivitis
- Leukemia
What are some acquired causes of generalized gingival enlargement?
- Acute Myeloid leukemia (result of infiltration of leukemic cells into gingival tissues)
- Aplastic anemia
- Drugs: phenytoin, cyclosporine, calcium channel blockers
- Sodium valproate, tranexamic acid (rare)
- scurvy
What are some congenital causes of GENERALIZED gingival enlargement?
- Gingival fibromatosis
- mucopolysaccharidoses
What are some congenital causes of LOCALIZED gingival enlargement? (7 and describe them)
- Fabry’s disease (genetic fat build up in cells leads to pain in extremities, kidney problems)
- Cowden’s disease (multiple noncancerous tumour like growths aka hamartomas, on skin and mucous membranes, w/an increased risk of cancer development
- Tuberous sclerosis (benign tumors grow everywhere (brain, kidney, hear, etc), DD and seizures)
- Focal dermal hypoplasia
- Sturge-Weber syndrome
- Congenital epulis
Tuberous sclerosis
AD
- Neurocutaneous syndrome
- Seizures (90%)
- MR (60%)
- Angiofribromas (70%) involving face and sometimes oral mucosa
- Enamel defects
Three neurocutaneous syndromes with oral manifestation?
Tuberous sclerosis
Sturge Weber
Neurofirbomatosis (von recklinghousers)
Localized ACQUIRED gingival enlargement what are some causes? The top 3- how can you tell the difference between them?
- **pyogenic granuloma (inflammed look, red, will bleed)
- **Peripheral ossifying fibroma (normal mucosa coloration, can cause displacement of teeth)
- **Peripheral giant cell epulis (ulcerated w/trauma, BROWN color)
- Also: heck’s disease, lymphoma, histiocytosis, papilloma, crohn’s disease, neoplasms
Tx for top3: sx excision
Albright’s Syndrome: alternate name? Physical appearance? characterized by? looks like on radiograph?
- Polyostotic fibrous dysplasia
- Abnormal skin pigmentation (“coast of maine” cafe au lait spots)
- Endocrine dysfunction: precocious puberty
- Radiograph: ground glass appearance (due to the underlying histology, on bx fibrous stroma w/bony trabeculations shaped like ‘chinese characters’)
Cafe au lait spots
Albright’s syndrome and neurofibromatosis
Cherubism: genetics? looks like? histology? on radiograph?
AD
- bilateral fullness of the cheeks
- hypertelorism and upslanting of the eyes
- Giant cell granuloma on histology
- Xray: MULTILOCULAR radiolucencies mandible and maxilla
List :
- Unilocular, pericoronal, radiolucent lesions
- Eruption cyst
- Dentigerous cyst
- Unicsystic Ameloblastoma
- Adenomatoid odontogenic tumor
Eruption Cyst: describe, usually involves what? tx? potential problems?
- A follicular cyst involving soft tissue, blueish, painless swelling over an erupting tooth
- Most frequently involves the primary dentition and permanent molars
- Tx: none
- Usually does not interfere w/eruption
Dentigerous Cyst: describe? Prevalence? Most common sites? Pain? Radiograph?
- A cyst resulting from the separation of the follicle from the crown of the tooth
- Most common developmental cyst (20%)
- Most common sites: mandibular molar, maxillary canine
- Painless expansion, tooth fails to erupt
- Tx: enucleate +/- tooth EXT
Unicystic Ameloblastoma: describe? location? pain? Radiograph? recurrence? tx?
Cystic tumor that occurs in children/adolescents
- 80% occur in the molar/ramus of mandible
- Painless, freq assoc w/an unerupted tooth
- May mimic a dentigerous cyst
- 10 to 30% recurrence rate
- Tx: enucleation
Adenomatoid Odontogenic Tumor:
prevalence? Age/gender? site? Pain? Radiograph? Tx?
Odontogenic tumor arising from the enamel organ.
- Represents 3-7% of odontogenic tumors
- 10-19 years old; Females>males
- Site: anterior maxilla> mandible
- Painless expansion; 75% associated w/crown of an unerupted tooth usually the max canine. may see ‘snowflake opacities’
- Tx: enucleation, recurrence is rare
List the multilocular pericornal radiolucent lesions
- OKC : odontogenic keratocyst
- Nevoid basal cell carcinoma syndrome
- Ameloblastic fibroma
Odontogenic Keratocyst : describe? location? Radiograph? Pain? Tx? Recurrence? assoc w/?
- An aggressive odontogenic or cystic tumor
- most occur in the mandibular third molar-ascending ramus area
- 25-40% associated with an unerupted tooth
- May have pain, soft tissue swellin, drainage, bone expansion
- Tx: surgical excision +/- ostectomy
- High recurrence rate 30%
- Assoc w/nevoid basal cell carcinoma
Nevoid basal cell carcinoma syndrome: extraoral appearance: head/face/jaws/body? Assoc w/?
- Multilocular pericoronal radiolucent lesion
- Enlarged occipitofrontral circumference
- Mild ocular hypertelorism
- OKC of the jaws
- Multiple basal cell carcinomas
- Epidermal cysts of the skin
- Palmar/plantar pits
- Calcified falx cerebri
- Rib anomalies
- Spina bifida occulta of cervical or thoracic vertebrae
- Hyperpneumatization of paranasal sinuses
Ameloblastic Fibroma: describe? When does it occur? where? pain? radiograph? Tx? Potential complications?
- a mixed odontogenic tumor (a multilocular pericoronal lesion)
- Occurs early: first 2 decades of life
- 70% occur in the posterior mandible
- Painless expansion: 75% assoc w/ an unerupted tooth
- Radiograph: Uni or multi-locular radiolucency
- Tx: conservative excision but may recur
- Ameloblastic fibrosarcomas may arise from this lesion
Unilocular periapical or central lesions
- Traumatic bone cyst
- Stafne bone defect
- Nasopalatine duct cyst
- Globulomaxillary cyst
- Median palatal cyst
Traumatic bone cyst: cause? where? pain? radiograph? tx?
Cause: trauma-hemorrhage, interstitial fluid build up, local disturbance in bone growth
- mandible most common site
- may cross midline
- usually asymptomatic without expansion
- Radiograph: uni or multilocular radiolucency w/scalloping between roots of vital teeth
- Tx: surgical curettage, does not recur
Stafne bone defect: gender, what is it? where?
- Usually seen in adolescent males when it occurs in children
- cyst-like radiolucency of the posterior mandible below the mandibular canal
- It is an indentation of bone containing the submandibular gland
Nasopalatine duct cyst: what is it made of? looks like? tx?
-Derived from epithelium of the nasoalatine duct, may perforate labial plate, teeth remain vital
- Ovoid/heart shaped radiolucency between maxillary central incisors
Tx: surgical enucleation
Globulomaxillary cyst: what is it? Where? what happens? tx?
- Not a true cyst: globulo-lying to the max cyst
- Located btwn the maxillary lateral and canine
- Usuallly represents a periapical cyst, odontogenic cyst
- may displace teeth
- tx: surgical enucleation
Median palatal cyst: what? where? feel/look? tx?
- A fissural cyst (unilocular and centrally located)
- epithelial entrapment
- may be confused for posteriorly positioned nasopalatine duct cyst
- form or fluctuant swelling of midline of the hard palate
- tx: surgical excision
What lesions start off unilocular when small, and become multilocular when large?
- Central giant cell granuloma
- aneurysmal bone cyst
- Central hemangioma
- Odontogenic myxoma
- Cherubism
Central giant cell granuloma : describe, pain? Where? recurrence? Tx?
-Aggressive and nonaggressive types, painless swelling sometimes causing displacement of teeth
- Mandible most common site (70%)
- May cross midline
- Moderate recurrence rate (15-20%)
Tx: curettage
Name the jaw lesions with giant cell histology that are found in children?
- Hyperparathyroidism
- Giant cell tumor
- Aneurysmal bone cyst
- Cherubism
Aneurysmal bone cyst: describe? pain?
An eccentric ballooning of the mandible that may be assoc w/a concurrent lesion.
- 50% cause pain
Central hemangioma: radiograph? oral findings? Potential complications?
Radiolucency w/vegue margins; may have honeycomb appearance.
- Gingival bleeding, bruit, pulsation
- Teeth are mobile
- Potentially life threatening
Odontogenic Myxoma : radiograph? Aggressive? location? recurrence?
- Faint radiopaque straition (stepladder appearance of residual trabeculae)
- Large lesions may have soap bubble appearance
- May displace or resorb teeth
- Posterior mandible
- Moderate recurrence rate : 25%
Which mixed radiolucent/opaque lesions are centrally located? Which are pericoronally located?
Centrally located mixed lesions:
- Central ossifying fibroma
- Juvenile ossifying fibroma
Pericoronal mixed lesions:
- Calcifying odontogenic cysts
- Adenomatoid odontogenic tumor
- Ameloblastic fibro-odontoma
Calcifying odontogenic cyst: where? pain? radiograph? Tx? Aggressive?
- Affects Max and Mand, 65% in anterior
- Painless swelling, may expand
- Rg: unilocular radiolucency that may contained scattered opacities; 33% assoc w/an unerupted tooth; 25% assoc w/odontoma
- Tx: sx excision
- Aggressive and malignant versions
Ameloblastic fibro-odontoma: age? where? Symptoms? radiograph? Tx?
- Usually seen in children average age 10 years
- posterior mandible is most common
- Usually asymptomatic; assoc w/failure of tooth to erupt
- Radiograph: usually unilocular radiolucency, variable amount of calcifications
- Tx: curettage; does not recur
Central ossifying fibroma: where? radiograph? describe? assoc w/? tx?
Posterior mandible most common - Unilocular radiolucency with variable amount of calcification - surrounded by a sclerotic border - Progresses from a RL to RO - Neoplastic Tx: enucleation
Juvenile Ossifying fibroma: where? aggressive? rg? tx? recur?
Maxilla>
Rapidly growing neoplasm, aggressive, expansile lesion
- circumscribed radiolucency w/calcification
- Tx: curettage to wide resection
- 30-58% recur
A radiopaque pericoronal lesion: age? where? radiograph? tx? assoc w/?
Odontoma
- Common odontogenic neoplasm, really a hamartoma or developmental anomaly
- Age: children, mean age is 14 yo
- Site: Maxilla>
- +/- expansion assoc w/delayed tooth eruption
- Radiograph: compound: tooth like calcifications; complex: amorphous calcifications; radiolucent border; may be cystic
- Tx and Prog: excision does not recur
- Assoc w/calcifying odontogenic cyst and ameloblastic fibrodontoma
Periapical or centrally located radiopaque lesions
- Idiopathic osteosclerosis (bone scar)
- Craniofacial fibrous dysplasia
- Albright’s syndrome
- Cementoblastoma
- Osteoblastoma
Idiopathic osteosclerosis (bone scar): cause? prevalence? age? site? rg? tx?
Unknown cause
- prevalence is high 5%
- Age: arise in late 1st or 2nd decade
- Site: mandible, molar-PM region
- Rg: well defined, oval to irregular opacity; usually uniformly opaque; no radiolucent rim or space
- Tx: periodic evaluation; stabilizes over time
Craniofacial fibrous dysplasia : what is it ? Looks like? teeth? rg? tx?
A developmental disorder affecting children. It is non-neoplastic, resultingin facial asymmetry.
- a painless unilateral enlargement of the maxillary bone; mandible may be involved
- dental abnormalities : missing permanent PM teeth; hypoplastic primary teeth
- RG: diffuse ground glass appearance, poorly defined margins
- Progresses from RL to RO
- Tx: cosmetic recountouring may be necessary; stabilizes over time
Cementoblastoma: what is it? age? site? pain? aggressive? Radiograph? tx?
an uncommon odontogenic neoplasm
- Occurs in children, young adults (75% <30); site is posterior mandible, molar-PM; 50% involve first molar; rarely involve primary teeth
- Pain, bony expansion, displaced teeth
- Radiograph: opacity w/radiolucent rim fused to roots, may resorb roots
- Progresses from RL to RO
- Tx: excision, extract tooth, 22% recur
Osteoblastoma: what isit ? where ? progression? pain? aggressive? radiograph? tx?
A benign bone tumor in the posterior mandible
- progresses from RL to RO
- Pain and swelling are common
-Aggressive: Vital tooth may have root resorption, may have “sun burst appearance” a well defined radiolucent lesion w/patchy to dense calcification
Tx: excision: a small # undergo malignant transformation
Peripheral radiopaque lesions: what are they? describe them? genetic? assoc w/?
- Tori/exostosis:non-neoplastic developmental, rare before age 10
- Osteoma: benign tumor of mature bone, assoc w/Gardern’s syndrome, AD inheritance, epidermoid/dermoid cysts 50%; multiple polyposes of large intestines with high malignant potential also see multiple supernumerary and impacted teeth.
A localized radiopaque inflammatory lesion ? Describe the cause, age, alternate name, where? aggressive?
Focal sclerosing osteomyelitis aka Condensing osteitis.
- an inflammatory lesion of the bone assoc w/a carious or nonvital tooth in theMolar/PM region of the mandible,
- age: children/young adults
- nonexpansile
Localized radiolucent periapical inflammatory lesions of the bone:
- Periapical abscess
- Periapical granuloma
- Periapical (radicular) cyst
Describe: peripaical abcess and periapical granulomas
Abscess: tender, painful, hx of caries/pulpal involvement and non-vital tooth. Unilocular with INDISTINCT
-Granuloma: chronic infection, flare ups common, unilocular radiolucency w/DISTINCT margins; non-vital tooth
Periapical (radicular) cyst: due to? pain? aggressive? tx?
- An inflammatory cyst due to nonvital tooth
- Uncommon in primary dentition, usually asymptomatic but may be tender and cause swelling, root resorption is common; may displace teeth
- Periapical or furcal radiolucency, may mimic a dentigerous cyst
- Tx: ext primary tooth, enucleate cyst, endo for permanent tooth
A pericoronal inflammatory radiolucency? Age? site? describe? clinical findings? radiograph? Tx?
Buccal Bifurcation cyst (an inflammatory paradental cyst):
- Type: uncommon inflammatory cyst
- Age: 5-13 years
- Usually found in MANDIBLE 1st Molar region; 1/3 are bilateral
- Large periodontal defect when probing
- Buccal swelling, foul tasting discharge, partial to fully erupted molar, hx of pericoronitis, buccal enamel extensions into furca
- Rg: unilocular radiolucency superimposed over roots of tooth; proliferative periostitis
- Tx: curettage or enucleation of cyst; bone heals w/in 1 yr
Peripheral inflammatory radiolucent lesion? Looks like? due to a hx of? radiograph? tx?
Traumatic Osteoma:
- some represent calcification of hematoma
- hx of facial trauma, esp to chin
- Inferior border of the mandible
- May be assoc w/ jaw fracture, non vital tooth
- Radiopaqe lesion w/irregular, onion skin or sunburst appearance,
- tx : may remodel or remain static
A diffuse radiolucent lesion with infectious etiology : clinical findings, pain? location? radiograph? tx?
Acute suppurative osteomyelitis:
- pain, trismus
- swelling, purulence, sequestrum
- febrile, leukocytosis
- lymphadenopathy
- usually posterior mandible but anterior maxilla in young children
- Radiograph : ill defined radiolucency +/- periosteal new bone formation
- Tx: antibiotics and drainage, may req microbio studies
Mixed radiolucent and radiopaque lesion with infectious etiologies?
- Chronic diffuse sclerosing osteomyelitis
2. Chronic osteomyelitis w/proliferative periostitis (Garre’s osteomyelitis)
Please describe chronic diffuse sclerosing osteomyelitis: who? where? why? what? radiograph? tx?
- Recurrent episodes of pain, swelling, induration, restricted mouth opening, w/ an absence of fever/purulence/sequestration
- Occurs in adolescents as primary chronic osteomyelitis
- Cause is uncertain; lacks an obvious ondontogenic infection
- radiograph: diffuse, indistinct borders w/mottled sclerotic bone pattern in the posterior mandible
- may result in ankylosis
Tx: refractory to tx
Chronic osteomyelitis w/proliferative periostitis: what is it? where? aggressive? radiograph? tx?
Result of a chronic dental infection
- A diffuse, expansile lesion w/indistinct margins on rg and a mottled bone pattern that looks like “onion skin”
- Poserior mandible
- Tx: tx the source of infection and the bone will remodel in 6-12 mo
Benign but aggressive unifocal and radiolucent tumors
- Melanotic neuroectodermal tumor of infancy
- Desmoplastic fibroma of bone
- Localized langerhans cell histiocytosis
- Central sarcomas of bone
- Primary soft tissue malignancies adjacent to bone
Unifocal and mixed RO/RL
Ewing’s Sarcoma
Osteosarcoma
Mesenchymal Chondrosarcoma
Multifocal and radiolucent :
Disseminated langerhan’s cell histiocytosis
Burkitt’s Lymphoma
Leukemia (AML)
Metastatic disease
Melanotic neuroectodermal tumor of infancy: prevalence, what is it? where? labs? radiograph? tx? Prognosis
A benign but aggressive rare tumor of neuroectodermal origin.
- Site: anterior maxilla
- Rapidly expanding, destructive bony lesion; surface may be blue in color
- Lab: increased urinary levels of vanillymandelic acid
- Rg: ill defined radiolucency, displaced toothbuds; floating appearance, sun ray pattern
Tx: wide excision
Prognosis : 20% recur; rare metastasis
Desmoplastic fibroma of bone: what is it? radiograph? aggressive? tx? recurs?
An aggressive tumor of fibroblastic origin
- Rg: ~multilocular, poorly defined margins
- expansile, “floating tooth buds”
- soft tissue extension
- Tx: curettage if localized, resection if diffuse
- High recurrence rate
Localized Langerhans cell histiocytosis (eosinophilic granuloma): what is it? aggressive? radiograph? Tx? Prognosis?
A mono or polyostotic disease of bone
with punched out radiolucencies
- non-expansile, “floating tooth” often soft tissue involvement
-Tx: localized jaw lesions, curettage
- Good prognosis but disease may disseminate
Central sarcoma of bone: where? what happens? radiograph?
Body of the mandible
- paresthesia occurs
- unilocular or multilocular w/poorly defined margins (aggressive); cortical perforation
Primary soft tissue malignancies adjacent to bone: looks like?
Well to poorly defined margins, “cupped out” appearance or “fine ground glass” appearance
Ewing’s Sarcoma: radiograph? where? pain?
Unifocal and mixed RO/RL: occurs in the posterior mandible and ramus
- painful expansion of bone
- febrile/leukocytosis
- “Moth eaten appearance” w/periosteal proliferation
Osteosarcoma: where? signs of it? radiograph
Posterior mandible, early symmetric widening of the PDL
- occasional periosteal proliferation
- Sunburst pattern UNCOMMON
Mesenchymal chondrosarcoma: location ? fain? radiograph
Man or maxilla. Painful. Primarily radiolucent
Disseminated langerhans cell histiocytosis: what is it? what does it affect? who does it affect? where? EOE? IOE? Radiograph? tx? prognosis?
A neoplastic disease of langerhans cells with multiorgan involvement.
- Age: young children; most are appearance
- Tx: curettage, chemo
- Poor prognosis when developed at a young age
Burkitt’s lymphoma: what is it? what is it assoc w/ ? where? pain? 1st sign? radiograph? tx?
An aggressive malignancy of B cells; many cases assoc w/ EBV
- Posterior maxilla/mandible
- painful swelling w/ 1st sign often tooth mobility
- loss of lamina dura
- “moth eaten” or diffuse radiolucency w/ragged ill defined margins
Tx: chemo
Leukemia AML: IOE? radiograph? tx?
Widespread involvement
IOE: occassional gingival enlargement due to leukemic infiltrate, necrotizing ulcerations, loss of lamina dura, and tooth mobility
- Diffuse poorly defined radiolucency
tx : chemo +/- BMT
Metastatic Disease: most commony found? looks like?
Most common soft tissue site: tongue/gingiva
Most common jaw site: posterior mandible
- Soft tissue extension is common
- Paresthesia
Genetic/Hereditary causes of premature primary tooth loss?
Acatalasia (lacking catalase, high perio disease) Chediak-Higashi syndrome Chronic cyclic neutropenia dentin dysplasia Down syndrome Hypophosphatasia Vit D resistant rickets Lesch-Nyhan syndrome -Papillon Lefevre syndrome
Neoplastic, infection, or other possible reasons for premature primary tooth loss?
- Neoplasms (Lymphoma, leukemia, langerhans’ cell histiocytosis, soft/hard tissue neoplasms)
- Infection: abscess, osteomyelitis, periodontitis
- Misc: acrodynia (pink hands due to heavy metal/mercury exposure), regional odontodysplasia, vit c deficiency, Leukocyte adhesion deficiency-1
