Oral Pathology and Syndromes Flashcards
Describe Epstein’s pearls - location? cell type? incidence? look? Feel? Prognosis?
Epstein’s Pearls aka Palatal cyst of the newborn
Location: Median Palatal raphe
Incidence: 75+/-% of Neonates
Cells: Epithelial inclusion cyst
Looks like small white papules that slough off, feels firm, goes away in a few months
Describe Bohn’s nodules - location? derived from? looks like? feels like? Prognosis?
Bohn’s nodules aka palatal cyst of the newborn
Location: junction of the hard and soft palate, the buccal and lingusal surface of the alveolus
- Derived from minor salivary glands (ectopic mucous glands)
- Mimics epstein’s pearls, often multiple
- firm to palpation
- needs no tx, will go away in a few months
What odontogenic cyst occurs in up to 50% of neonates? Describe where it occurs, what it is a remnant of? Prognosis?
Dental lamina cysts (gingival cysts of the newborn)
Occurs on alveolar mucosa
Remnants of dental lamina
- will spontaneously rupture and resolve on their own
- Physicians may think they are natal teeth
This developmental variation is often seen on the lateral border of the tongue and is usually bilateral. What is it and what type of tissue is it?
Hyperplastic foliate papillae
Normal lympoid tissue
**note : May increase in size/tender but is normal variation
Describe an oral lymphoepithelial cyst : tissue/cell type, long term effects, where does it occur
Oral lymphoepithelial cyst:
- entrapped epithelium within lymphoid tissue
- Undergoes cystic degeneration, is a persistent pale/yellowish/white nodule
- occurs in oral floor, soft palate, tonsillar region
Ectopic sebaceous glands in oral mucosa: name? Prevalence? describe the look? common sites?
Fordyce granules 30% of children, 70% of adults Slightly elevated yellowish papules May be discrete or confluent Commonly: buccal mucosa, upper lip
Fissured Tongue: typical age group, genetic pattern? Frequency? Associated with?
Fissured tongue: Rarely seen before age 4 Appears AD/genetic 3-5% freq but higher in certain groups Assoc w/geographic tongue
Leukoedema: prominent in which ethnic group? describe color/texture? uni or bilateral? cell type? characteristic physical property?
Leukoedema:
prominent in Afr Ams
Filmy grayish/white, wrinkled thickening of buccal/labial mucosa
Bilateral
Extensive intracellular edema of epithelium
Diminishes when cheek is stretched
What are the three most common cysts of the new born?
Epstein’s Pearls (palatal cyst of new born)
Bohn’s nodules (palatal cyst of newborn)
Dental lamina (gingival cyst of the new born–50% of neonates)
Median Rhomboid glossitis- theory of why it occurs? Symptoms? Looks like? Infectious agent?
Result of anomalous vascularity vs persistence of tuberculum impar
- Usually asymptomatic but may cause soreness/burning
- Surface flat or slightly raised
- Color varies from pale pink or whitish to bright red
- Candida infection present in 40%
Fissured Tongue: age? Describe? frequency?
- Rarely seen before age 4 yo
- Genetic (AD)
- 3-5% frequency, but higher in mentally retarded population
- Maybe assoc w/Melkersson-Rosenthal syndrome
Leukoedema: Seen most frequently in which people? Looks like? test for? If it fails test, what does it look like?
- Most commonly seen in blacks
- Grayish-white thickening of buccal mucosa
- usually bilateral
- stress mucosa and it disappears, if doesn’t disappear likely white sponge nevus
Idiopathic osteosclerosis: looks like? where is it found?
- Well-defined radiopacity in the tooth-bearing area of jaw
- No surrounding radiolucent space
- not typical of any other condition
- Mandibular premolar/molar area most common
- Maybe related to root apex, but normal PDL
Stafne bone defect: where? looks like? what is it? Observed more in who?
Cyst like radiolucent area near the angle of the mandible
- Indentation of bone containing extension of submandibular gland
- more often seen in males with square jaws
Bifid tongue:
Developmental malformation
- tongue is three structures that merge, if incomplete results in bifid tongue
- may coexist with orofacialdigital syndrome
Bifid Uvula: likely what? what needs to be ruled out?
Minor expression of clef palate, must r/o sub mucous cleft, may require sx:
Lingual thyroid- looks like? what happens as a result?
Redundant thyroid tissue in tongue
- hypothyroidism ~20%
~70% of these people lack normal thyroid tissue in neck
Neonatal alveolar lymphangioma: what group does it typically occur in? Where and when is it usually found? What is the treatment?
Present at birth
- Usually occurs in aftrican american males
- site : alveolar ridge of the mandible> maxilla
- translucent to pink, fluctuant swelling can be single or multiple
- Tx: none, resolves spontaneously
Lymphangioma: what is it? When are they most often found? Where do they most often occur? What do they look like? Physical characteristics? Most common type of lymphangiomas? Treatment?
A congenital hamartoma of lymphatics
- 50 % are seen at birth.
- 50-75% occur in the head and neck. Tongue is the most common oral site.
- Diffuse or discrete lesions, pink, red or purple enlargement with a papillary or vesciular surface.
- Does not blanch with pressure
- Surface often papillary or vesicular
- Complications: airway obstruction, dysphagia, malocclusion, disfigurement
- A variant: is the cystic hygroma
- Tx: surgical excision
What is the most common vascular tumor of infancy? What percent? When do they appear? A sign of them? Treatment?
10% of infants have hemangioma
- Usually appear early in infancy, grow rapidly until age 6-8 months; then slowly involute
- Blanch on pressure
- Generally do not involve the adjacent skeletal tissue
- Tx: Watch and wait, they’ll usually go away
What are some common genetic syndromes associated with enlarged or swollen tongues (macroglossia)?
Beckwith Wiedemann, Down, Mucopolysaccharidosis, neurofibromatosis, multiple endocrine neoplasia
Kasabach-Merrit syndrome: what is it? treatment?
An indication for treatment of a hemangioma
- Kasabach–Merritt syndrome (KMS), also known as Hemangioma with thrombocytopenia[1]:597) is a rare disease, usually of infants, in which a vascular tumor leads to decreased platelet counts and sometimes other bleeding problems, which can be life-threatening.
- It is also known as hemangioma thrombocytopenia syndrome
- hemangioma is so big its stealing up all the platelets, needs to be treated.
Pyogenic granuloma: what is it? What does it look/feel like? Where does it typically occur? Tx?
Common reactive lesion
- painless, nodule, red lesion
- typically pedunculated w/ulcerated surface
- typical site: gingiva>lips>tongue>buccal mucosa
- tx: sx excision or remove local irritant
Riga Fede: what is it? where? Tx?
- Chronic trauma from primary incisors
- typically ulcerated lesion on tip of tongue
- Tx: smooth incisal edges, heals 7-14 days
What is the most common tumor of the oral mucosa? look? cell type? Feel? Tx?
Fibroma (irritation fibroma)
- Most common tumor of oral mucosa
- often the result of chronic trauma
- typically painless, firm, sessile or pedunculated. White in color due to keratin.
- Tx: surgical excision, remove source of irritation
Papilloma- virus? look? what to rule out? other possible explanations?
- Human papilloma virus
- Exophytic, well circumscribed
- usually pedunculated, with either finger like projections or cauliflower surface
- need to rule out condyloma acuminatum (as a potential indication for child abuse, need histology exam)
- Tx : surgical excision.
- Can you auto inoculate from warts on their hands? yes
Neurofibroma: prevalence? Where does it occur? looks like? Tx?
- Rare in children
- Tongue and buccal mucosa most commonly affected
- Maybe solitary or multiple (neurofibromatosis)
- Tx: sx excision
Neurofibromatosis: inheritance? oral involvement %? other findings?
AD (50% spontaneous mutations)
- Peripheral form most common ~90%
~70% have oral involvement
- Cafe au lait spots (cms in diameters with regular well defined borders), subcutaneous neurofibromas, lisch nodules
Granular cell tumor: how common? Where is it usually found? describe? tx?
Granular Cell tumor:
- uncommon benign lesion
- Tongue most common site 25%
- Typically solitary, asymptomatic, well-defined, sessile lesion
- Tx: sx excision
Describe Ehlers Danlos Syndrome: class form? Dental sequela?
Collagen defect (pro-collagen) - Classic form: loose joints, fragile, bruisable skin, laxity of skin, "cigarette paper" scars - Dental : oral bleeding, periodontal disease, delayed healing, pulp stones A "positive ghorlin sign" tongue can touch tip of nose
Down syndrome: oral manifestations? Chromosome? prevalence? Other systems effected? Greater risk for?
Chromosomal disorder - trisomy 21
- 1:800 births
- Most common cause of mental disability
- Maternal risk of down syndrome increases with maternal age
- Congenital heart disease 50%; GI anomalies 15%, Increased incidence of ALL (20x higher than non-DS)
What syndromes are assoc w/increased paternal age?
Achondroplasia
Apert syndrome
Marfan Syndrome
Autism (?)
Dental conditions associated w/Down Syndrome?
- Relative macroglossia
- Microdontia
- Oligodontia
- Class II malocclusion
- Open mouth posture
- Increased periodontal disease
- delayed eruption and over retained teeth
- atypical root morphology
- abnormal palate
- enamel hypoplasia
- decreased caries risk (historically when these kids were institutionalized, however becoming more like normal)
What are the medical conditions associated with down syndrome?
- Congenital heart disease (~50%): AV septal defect, ventral septal defect, and Tetralogy of Fallot
- GI anomalies (15%): tracheosesophageal fistula, pyloric stenosis, dudodenal atresia, celiac disea
- Increased incidence of ALL (20x normal)
Hypothyroidism (Cretinism): physical features? treatment?
Results in short stature -mental retardation - delayed eruption -enamel hypoplasia -generalized edema Tx: replacement therapy, if done so in the early stages will result in normal development
Mucopolysaccharidoses : group of syndromes, what is the ‘prototype’ describe it
Hurler’s syndrome
- progressive infiltration of tissues by mucopolysccharides (tissues expand!)
- Coarse facies, large head
- spacing of teeth, relative macroglossia
- Tx : bone marrow transplant
Cystic Hygroma: what is type of growth is it? Where is it found? What complications occurs as a result?
Large diffuse lymphangioma,
-extends from tongue into neck
-May cause dysphagia or respiratory distress (large neck)
~30% have bone involvement and require surgical/orthognathic surgery
Mortality rate 2-4%
