Syndrome

Front 1

Crouzon's disease happens because of what

Back 1

premature closing of cranial sutures

Front 2

premature closing of cranial sutures leads to what in crouzon's disease

Back 2

brachycephaly (short head) and trigonocephaly (triangle-shaped head)

Front 3

what are char of crouzon's disease other than brachycephaly and trigonocephaly

Back 3

ocular proptosis, "cloverleaf" skull, hypertelorism (wide spaced eyes), hypoplastic maxilla ( smaller) with short upper lip, malocclusion, and poor vision and hearing deficits

Front 4

what disease has syndactyly of hands and feet

Back 4

apert syndrome

Front 5

what are char shown in or around mouth for apert syndrome

Back 5

hypoplastic maxilla
mandibular prognathism
possible cleft soft palate
ginival thickening with possible delayed eruption
shovel shaped incisors in 1/3 pts

Front 6

what are char outside of mouth for apert syndrome

Back 6

"cloverleaf" skull
mentally deficient
acrobrachycephaly
ocular proptosis
hypertelorism
down slanting of lateral palpebral fissures
syndactyly
can have mental retardation

Front 7

what are facial char that are unique to treacher collins syndrome

Back 7

"bird-like" appearance
zygomatic atrophy
coloboma
downward slanting of lower eyelid
ear defects

Front 8

what are oral char that are unique to treacher collins syndrome

Back 8

max area are indented in
mandibular micrognathia
cleft palate in 1/3 cases

Front 9

what is tx for treacher collins syndrome

Back 9

cosmetic surgery

Front 10

what is osteogenesis imperfecta

Back 10

inherited disorder of collagen maturation that happens in bone with thin cortex, fine trabeculation and diffuse osetoporosis

Front 11

what disease makes bones fracture easily with inability of the matrix to fully mineralize which leads to exaggerated callous formation

Back 11

osteogenesisi imperfecta

Front 12

possible blue sclera and possible hearing loss

Back 12

osteogenesis imperfecta

Front 13

what oral char does osteogenesis imperfecta have

Back 13

dentinogenesis imperfecta (25%) and possible malocclusion due to maxillary hypoplasia

Front 14

what general char (non oral)does cleiodocranial dysplasia have?

Back 14

clavicles absent or hypoplastic, skukk deformities, brachycephaly (short head), hypertelorism, frontal adn occipital bossing (prominance)
short statue

Front 15

what oral char does cleidocranial dysplasia have

Back 15

supernumerary teeth
over retained primary teeth
delayed eruption of permanent teeth

Front 16

what has cleft palate, mandibular micrognathia, and glossoptosis (airway obstruction due to posterior displacement of tongue)

Back 16

pierre robin syndrome

Front 17

what do ppl with marfan syndrome look like

Back 17

tall, slender, large hands and fingers, long narrow skull and bones

Front 18

what problem does people with marfan syndrome have

Back 18

abnormally soluble collagen, cardiovascular defects, possible early death

Front 19

what does ppl with marfan syndrome have problems orally

Back 19

high arched palate, bifid uvula, malocclusion

Front 20

what are oralfacial findings for down syndrome

Back 20

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

Front 21

what are facial features for down syndrome

Back 21

flat face, hypertelorism, and peicanthal folds

Front 22

what is massive osteolysis

Back 22

progressive destruction of bone where bone is replaced by vascular tissue than filled in by fibrous tissue

Front 23

what happens to teeth and bone for massive osteolysis

Back 23

teeth mobile and bone fracture

Front 24

who has had hereditary benign intraepithelial dyskeratosis

Back 24

children and mixture of white, black, and american indians

Front 25

what is affected mostly by hereditary benign intraepithelial dyskeratosis

Back 25

eye lesions consists of foamy gelatinous plaques affecting the conjunctiva adj to cornea, which can cause blindness

Front 26

oral lesions from hereditary benign intraepithelial dyskeratosis look like what

Back 26

white sponge nevus or leukoedema

Front 27

what diseaes has accumulation of keratinaceous material in nail beds and hyperkeratosis of hands and feet (soles) and excess keratin in hair follicles

Back 27

pachyonychia congenita

Front 28

what disease has person sweating alot

Back 28

pachyonychia congenita

Front 29

pachyonychia congenita has what kind of oral lesion

Back 29

thickened white plaques on lateral adn dorsal tongue (look like white sponge nevus)

Front 30

what are you at risk when you have gardner's syndrome

Back 30

colorectal polyps that may transform into adenocarcinoma and thyroid carcinoma

Front 31

what do you have non orally when you have gardner's syndrome

Back 31

colorectal polyps, osteomas, epidermoid cyst, desmoid tumors

Front 32

what are oral char in ppl with gardner's syndrome

Back 32

supernumerary teeth and impacted teeth.

Front 33

what are facial/oral char of peutz-jeghers syndrome

Back 33

melanotic spots on buccal mucosa, gingivae, hard palate, and around mouth, nose, and eyes

Front 34

small intestinal polyps resulting in intrassusception happens to whom

Back 34

pts with peutz-jeghers syndrome

Front 35

people with peutz-jeghers syndrome will be at risk to get what

Back 35

adenocarcinoma

Front 36

what disease has Small telangiectasias and petechia apparent at birth or puberty and increase with age

Back 36

multiple hereditary telangiectasia

Front 37

where does the small telangiectasias and petechia in the mouth

Back 37

lips, gingivae, palate, tongue

Front 38

due to chronic bleeding from telangiectasia in multiple hereditary telangiectasia

Back 38

epistaxis (iron-def anemia)

Front 39

what is prescribed for dental procedures in pts with pulmonary arteriovenous malformations in multiple hereditary telangiectasia

Back 39

prophylactic

Front 40

what is sturge-weber angiomatosis

Back 40

vascular proliferation of tissues of brain and face known as port wine stain q

Front 41

what does sturge-weber angiomatosis present

Back 41

unilateral distribution of trigeminal distribution and meningeal angiomatosis that is assoc with convulsions and mental retardation

Front 42

basal cell nevus syndrome presents what?

Back 42

multiple basal cell carcinomas of skin, odontogenic keratocysts (multi), rid anomalies, mild hypertelorism, palmar/plantar pits, intracranial calcifications

Front 43

person with hereditary ectodermal dysplasia presents what generally

Back 43

heat intolerance, sparse hair/eyebrows, hyperpigmentation around eye

Front 44

what oral char is shown in person with hereditary ectodermal dysplasia

Back 44

xerostomia and reduced number of teeth with abnormal shapes

Front 45

what does a person with von recklinghausen's disease have

Back 45

sessile and pedunculated neurofibromas
cafe-au-lait spots and
some have sarcomatous metaplasia

Front 46

what does a person have orally when they have von recklinghausen's disease of skin

Back 46

oral lesions, enlarged fungiform papillae, mandibular central lesions seen in oral cases causing pain and paresthesia

Front 47

what disease is increased risk for thyroid disease (goiter/theyroid adenoma), breast cancer, and GI polyps

Back 47

cowden syndrome

Front 48

what disease shows multi papules of gingiva, tongue, and buccal mucosae

Back 48

cowden syndrome

Front 49

what diseaes has esp MLG on breast and thyroid

Back 49

cowden syndrome

Front 50

which disease shows abnormal collagen and hypermobility of joints? called rubber man

Back 50

ehlers-danlos syndrome

Front 51

what is marked elasticity of skin "rubber man"

Back 51

ehlers-danlos syndrome

Front 52

Papyracous scarring is from what

Back 52

ehlers-danlos syndrome

Front 53

ehlers-danlos syndrome shows what in dental abnormalities

Back 53

malformed or short roots, hypoplastic enamel...ect

Front 54

what disease shows hyperkeratosis of palms of hands and soles of feet which appears in first few years of life

Back 54

papillion Lefevre syndrome

Front 55

what disease causes severe destructive periodontal disease where teeth are loose

Back 55

papillon-lefevre syndrome

Front 56

primary teeth are lost by what age in papillon-lefevre syndrome

Back 56

4

Front 57

most permanent teeth are lost in papillon-lefevre by what age

Back 57

14

Front 58

what bone is mostly destroyed in papillon lefevre syndrome

Back 58

alveolar bone

Front 59

what disease shows hyperkeratosis of palm of hands and soles of feet in first years of life

Back 59

papillon lefevre syndrome

Front 60

what is the tx for papillon lefevre syndrome

Back 60

retinoids and antibiotics