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60 Cards in this Set
- Front
- Back
Crouzon's disease happens because of what
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premature closing of cranial sutures
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premature closing of cranial sutures leads to what in crouzon's disease
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brachycephaly (short head) and trigonocephaly (triangle-shaped head)
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what are char of crouzon's disease other than brachycephaly and trigonocephaly
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ocular proptosis, "cloverleaf" skull, hypertelorism (wide spaced eyes), hypoplastic maxilla ( smaller) with short upper lip, malocclusion, and poor vision and hearing deficits
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what disease has syndactyly of hands and feet
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apert syndrome
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what are char shown in or around mouth for apert syndrome
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hypoplastic maxilla
mandibular prognathism possible cleft soft palate ginival thickening with possible delayed eruption shovel shaped incisors in 1/3 pts |
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what are char outside of mouth for apert syndrome
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"cloverleaf" skull
mentally deficient acrobrachycephaly ocular proptosis hypertelorism down slanting of lateral palpebral fissures syndactyly can have mental retardation |
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what are facial char that are unique to treacher collins syndrome
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"bird-like" appearance
zygomatic atrophy coloboma downward slanting of lower eyelid ear defects |
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what are oral char that are unique to treacher collins syndrome
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max area are indented in
mandibular micrognathia cleft palate in 1/3 cases |
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what is tx for treacher collins syndrome
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cosmetic surgery
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what is osteogenesis imperfecta
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inherited disorder of collagen maturation that happens in bone with thin cortex, fine trabeculation and diffuse osetoporosis
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what disease makes bones fracture easily with inability of the matrix to fully mineralize which leads to exaggerated callous formation
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osteogenesisi imperfecta
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possible blue sclera and possible hearing loss
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osteogenesis imperfecta
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what oral char does osteogenesis imperfecta have
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dentinogenesis imperfecta (25%) and possible malocclusion due to maxillary hypoplasia
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what general char (non oral)does cleiodocranial dysplasia have?
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clavicles absent or hypoplastic, skukk deformities, brachycephaly (short head), hypertelorism, frontal adn occipital bossing (prominance)
short statue |
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what oral char does cleidocranial dysplasia have
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supernumerary teeth
over retained primary teeth delayed eruption of permanent teeth |
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what has cleft palate, mandibular micrognathia, and glossoptosis (airway obstruction due to posterior displacement of tongue)
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pierre robin syndrome
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what do ppl with marfan syndrome look like
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tall, slender, large hands and fingers, long narrow skull and bones
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what problem does people with marfan syndrome have
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abnormally soluble collagen, cardiovascular defects, possible early death
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what does ppl with marfan syndrome have problems orally
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high arched palate, bifid uvula, malocclusion
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what are oralfacial findings for down syndrome
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small max and mandible
weird shaped/smaller teeth macroglossia with protruding tongue peg shaped lateral incisors in some malocclusion increased susceptiblility to infections with an increased susceptibility to periodontal disease |
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what are facial features for down syndrome
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flat face, hypertelorism, and peicanthal folds
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what is massive osteolysis
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progressive destruction of bone where bone is replaced by vascular tissue than filled in by fibrous tissue
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what happens to teeth and bone for massive osteolysis
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teeth mobile and bone fracture
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who has had hereditary benign intraepithelial dyskeratosis
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children and mixture of white, black, and american indians
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what is affected mostly by hereditary benign intraepithelial dyskeratosis
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eye lesions consists of foamy gelatinous plaques affecting the conjunctiva adj to cornea, which can cause blindness
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oral lesions from hereditary benign intraepithelial dyskeratosis look like what
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white sponge nevus or leukoedema
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what diseaes has accumulation of keratinaceous material in nail beds and hyperkeratosis of hands and feet (soles) and excess keratin in hair follicles
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pachyonychia congenita
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what disease has person sweating alot
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pachyonychia congenita
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pachyonychia congenita has what kind of oral lesion
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thickened white plaques on lateral adn dorsal tongue (look like white sponge nevus)
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what are you at risk when you have gardner's syndrome
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colorectal polyps that may transform into adenocarcinoma and thyroid carcinoma
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what do you have non orally when you have gardner's syndrome
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colorectal polyps, osteomas, epidermoid cyst, desmoid tumors
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what are oral char in ppl with gardner's syndrome
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supernumerary teeth and impacted teeth.
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what are facial/oral char of peutz-jeghers syndrome
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melanotic spots on buccal mucosa, gingivae, hard palate, and around mouth, nose, and eyes
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small intestinal polyps resulting in intrassusception happens to whom
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pts with peutz-jeghers syndrome
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people with peutz-jeghers syndrome will be at risk to get what
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adenocarcinoma
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what disease has Small telangiectasias and petechia apparent at birth or puberty and increase with age
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multiple hereditary telangiectasia
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where does the small telangiectasias and petechia in the mouth
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lips, gingivae, palate, tongue
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due to chronic bleeding from telangiectasia in multiple hereditary telangiectasia
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epistaxis (iron-def anemia)
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what is prescribed for dental procedures in pts with pulmonary arteriovenous malformations in multiple hereditary telangiectasia
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prophylactic
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what is sturge-weber angiomatosis
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vascular proliferation of tissues of brain and face known as port wine stain q
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what does sturge-weber angiomatosis present
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unilateral distribution of trigeminal distribution and meningeal angiomatosis that is assoc with convulsions and mental retardation
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basal cell nevus syndrome presents what?
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multiple basal cell carcinomas of skin, odontogenic keratocysts (multi), rid anomalies, mild hypertelorism, palmar/plantar pits, intracranial calcifications
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person with hereditary ectodermal dysplasia presents what generally
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heat intolerance, sparse hair/eyebrows, hyperpigmentation around eye
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what oral char is shown in person with hereditary ectodermal dysplasia
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xerostomia and reduced number of teeth with abnormal shapes
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what does a person with von recklinghausen's disease have
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sessile and pedunculated neurofibromas
cafe-au-lait spots and some have sarcomatous metaplasia |
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what does a person have orally when they have von recklinghausen's disease of skin
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oral lesions, enlarged fungiform papillae, mandibular central lesions seen in oral cases causing pain and paresthesia
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what disease is increased risk for thyroid disease (goiter/theyroid adenoma), breast cancer, and GI polyps
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cowden syndrome
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what disease shows multi papules of gingiva, tongue, and buccal mucosae
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cowden syndrome
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what diseaes has esp MLG on breast and thyroid
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cowden syndrome
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which disease shows abnormal collagen and hypermobility of joints? called rubber man
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ehlers-danlos syndrome
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what is marked elasticity of skin "rubber man"
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ehlers-danlos syndrome
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Papyracous scarring is from what
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ehlers-danlos syndrome
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ehlers-danlos syndrome shows what in dental abnormalities
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malformed or short roots, hypoplastic enamel...ect
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what disease shows hyperkeratosis of palms of hands and soles of feet which appears in first few years of life
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papillion Lefevre syndrome
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what disease causes severe destructive periodontal disease where teeth are loose
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papillon-lefevre syndrome
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primary teeth are lost by what age in papillon-lefevre syndrome
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4
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most permanent teeth are lost in papillon-lefevre by what age
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14
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what bone is mostly destroyed in papillon lefevre syndrome
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alveolar bone
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what disease shows hyperkeratosis of palm of hands and soles of feet in first years of life
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papillon lefevre syndrome
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what is the tx for papillon lefevre syndrome
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retinoids and antibiotics
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