SPC

Question 1

Describe features of Apert Syndrome.

Answer 1

supernumerary teeth, cleft palate, delayed/ectopic eruption, shovel shaped incisors, hypoplastic midface, syndactyly, craniosynostosis, hypertelorism, class III with anterior openbite, crowded dentition

Question 2

Describe features of cleidocranial dysplasia.

Answer 2

supernumerary teeth, delayed development/eruption, midface hypoplasia, enamel hypoplasia, missing clavicle, craniosynostosis

Question 3

Describe features of Gardner Syndrome

Answer 3

supernumerary teeth, osteomas of the jaw, delayed eruption, colonic polyps

Question 4

Describe features of Crouzon syndrome.

Answer 4

supernumerary teeth, midface hypoplasia, inverted V shaped palate, craniosynostosis, exopthalamos

Question 5

Describe features of Sturge-Weber syndrome

Answer 5

port-wine stains which follow the trigeminal nerve, supernumerary teeth, overgrowth of maxilla, ipsilateral gyriform calcifications of cerebral cortex, 100% have seizures, hemiplegia, ocular defects, bleeding and gingival hyperplasia, alveolar bone loss, pyogenic granulomas

Question 6

Describe features of orofaciodigital syndrome.

Answer 6

supernumerary teeth and/or hypodontia; multiple or hyperplastic frenula, cleft tongue

Question 7

Describe features of Hallerman-Strieff syndrome.

Answer 7

supernumerary teeth, mandibular hypoplasia, high palatal vault, delayed primary exfoliation, malar hypoplasia

Question 8

Describe features of ectodermal dysplasia.

Answer 8

hypodontia, conical crowns, deficient alveolar ridge

Question 9

Describe features of achondroplasia

Answer 9

hypodontia, short stature, frontal bossing, midface hypoplasia

Question 10

Describe features of chondroectodermal dysplasia (aka Ellis van Creveld).

Answer 10

hypodontia, conical crowns, enamel hypoplasia, short stature, lack of maxillary sulcus, premature teeth

Question 11

Describe features of Incontinentia pigmenti

Answer 11

hypodontia, conical crowns, delayed eruption, premature teeth, cleft lip/palate, blistering of the skin, hyperpigmentation

Question 12

Describe features of Rieger syndrome.

Answer 12

Hypodontia, midface hypoplasia, delayed eruption, short stature, eye malformation

Question 13

Describe features of Seckel syndrome

Answer 13

hypodontia, microcephaly, midface hypoplasia, dwarfism, large eyes

Question 14

Describe features of Williams Syndrome.

Answer 14

Hypodontia, prominent lips, microdontia, enamel hypoplasia, elflike facial appearance, happy demeanor

Question 15

Describe the features of Klinefelter syndrome.

Answer 15

taurodontism, small cranial dimension, bimaxillary prognathism, male with extra X chromosome, gynecomastia

Question 16

Describe the features of tricho-dento-osseous syndrome.

Answer 16

Dolichocephalic with frontal bossing, taurodontism, delayed eruption, kinky or coarse hair at birth, enamel hypoplasia, AI+taurodontism+nail and hair defects

Question 17

Describe the features of Mohr syndrome.

Answer 17

Lobed tongue, upper lip/midline cleft, oligodontia, polydactyly

Question 18

Describe the dental effects of hypoparathyroidism.

Answer 18

permanent teeth affected mostly, short, wedge-shaped roots with delayed apical closure, enamel hypoplasia

Question 19

Describe hypophosphatasia.

Answer 19

lack of serum alkaline phosphatase, increased urinary phosphoethanolamine, autosomal recessive, early exfoliation of primary dentition, deficient amount of cementum, large pulp chambers, permanent teeth are often not affected

Question 20

What is porphyria

Answer 20

group of disorders caused by an overaccumulation of porphyrin which helps hemoglobin, the protein that carries oxygen in the blood.

Question 21

Porphyria results in intrinsic staining of what color?

Answer 21

purplish-brown

Question 22

Neonatal hepatitis results in what color intrinsic stain?

Answer 22

black/gray

Question 23

Erythroblastosis fetalis results in what color intrinsic stain?

Answer 23

blue-green, brown

Question 24

Cystic fibrosis results in what color intrinsic stain?

Answer 24

yellowish-gray to dark brown

Question 25

Describe the features of McCune-Albright syndrome.

Answer 25

Endocrine dysfunction leading to cafe au lait macules, precocious puberty and polyostotic fibrous dysplasia

Question 26

What is polyostotic fibrous dysplasia

Answer 26

orm of fibrous dysplasia affecting more than one bone. Fibrous dysplasia is a disorder where bone is replaced by fibrous tissue, leading to weak bones, uneven growth, and deformity

Question 27

Describe Heck disease.

Answer 27

multifocal epithelial hyperplasia caused by HPV 13 or 31, more common in poverty, malnutrition, poor hygiene or comorbid with HIV, DD of condyloma, may spontaneously resolve, excise large lesions

Question 28

Describe the features of multiple endocrine neoplasia type 2B.

Answer 28

autosomal dominant, marfanoid body type, narrow facies, full lips, mucosal neuromas of the lips, tongue buccal mucosa and gingiva, medullary carcinoma of the thyroid, pheochromocytoma

Question 29

What is a pheochromocytoma?

Answer 29

tumor of adrenal gland, results in overproduction of epinephrine and norepinephrine

Question 30

Describe features of Goldenhar syndrome

Answer 30

unilateral microtia, failure of formation of mandibular ramus and condyle, macrostomia, frequent eye and skeletal involvement, 50% have cardiac abnormalities like VSD or PDA,

Question 31

Describe the features of cretinism.

Answer 31

congenital hypothyroidism (myxedema in adults); intellectual disability; slow growth, short cranial base, underdeveloped mandible, overdeveloped maxilla, tongue enlargement, delayed dentally, infiltration of skin and mucous membranes by glycoaminoglycans; treatment is thyroid replacement therapy

Question 32

Describe the features of Hurler syndrome.

Answer 32

short stature, large head, decreased IQ, enlarged lips, nasal bridge depresses, open mouth and protruding tongue, widely spaced teeth, areas of bone destruction, delayed dentally, enlarged dental follicles, mucopolysaccharoidosis

Question 33

Describe the features of Beckwith-Wiedemann syndrome.

Answer 33

macroglossia, omphalocele or umbilical hernia, cytomegaly of adrenal cortex, postnatal gigantism, mild microcephaly, severe hypoglycemia, neoplasms

Question 34

Describe the features of Kawasaki disease.

Answer 34

mucocutaneous lymph node syndrome, bilateral conjunctivitis, fissured lips, infected pharynx, strawberry tongue, erythema of palms and soles, rash, fever, inflammatory disease

Question 35

Erythema multiforme is caused by what?

Answer 35

immune reaction triggered by drugs, HSV, mycoplasma pneumonia, infections, tattooing, idiopathic

Question 36

Describe the features of erythema multiforme.

Answer 36

target lesions on skin, fever, sore throat, ulcers, erythema, blood crusted lips

Question 37

What is Stevens-Johnson syndrome?

Answer 37

A type of erythema multiforme with ocular and genital involvement

Question 38

What are the defining features of Behcet syndrome?

Answer 38

oral aphthae (extensive and multiple more than 3 times per year), genital ulcerations, ocular lesions, neurologic disease, large and small vessel vasculitis

Question 39

Describe features of fibrous dysplasia.

Answer 39

enlargement is painless and gradual, begins early in life, stabilizes in adulthood, ground glass appearance on X-ray (similar to McCune-Albright syndrome)

Question 40

Multiple OKCs are associated with what syndrome?

Answer 40

Nevoid basal cell carcinoma syndrome

Question 41

Describe the features of Nevoid basal cell carcinoma syndrome.

Answer 41

enlarged occipitofrontal circumference, mild ocular hypertelorism, multiple basal cell carcinomas, multiple OKCs, palmar and plantar pits, calcified falx cerebri, rib anomalies, spina bifida occulta, hyperpneumatization of paranasal sinuses