01 Developmental defects Flashcards
orofacial clefts
secondary to lack of improper merging and fusion.
face, lips, & palate develop during weeks 4-12 of gestation.
treatment usually involves surgery
various clefts
cleft lip cleft palate lateral facial cleft oblique facial cleft median cleft of upper lip median maxillary anterior alveolar cleft
causes of orofacial clefts
genetic environmental: alcohol cigarettes folic acid deficiency corticosteroids antivconvulsants
cleft lip &/or palate
failure of medial nasal process & maxillary process to fuse. 45% both 30% CP only 25% CL only 3-8% associated with syndromes
cleft lip
1:700-1000 births
more common in males, 80% unilateral.
often involves alveolus affecting incisors & cuspid
cleft palate
0.4:1000 births
failure of palatal shelves to fuse, more common in females. can be severe or mild (bifid uvula)
bifid uvula
1:80 births
higher frequency in asians & native american (1:10), less common in blacks (1:250).
uvula can also be fused or enlarged
median maxillary anterior alveolar cleft
between #8-9, may cause diastema, may require surgical correction
Pierre Robin syndrome
CP + mandibular micrognathia + glossoptosis + airway obstruction.
feeding, speech, occlusion problems.
requires surgery
commissural lip pits
12-20% occurrence
blind fistulas 1-4mm deep at commissures
more in males, no treatment
double lip
redundant fold on maxillary mucosal side visible usually only when smiling.
congenital & isolated
also with Ascher syndrome
Ascher syndrome
double lip + nontoxic thyroid enlargement + blepharochalasis (eyelid inflammation)
plastic surgery correction
fordyce granules
ectopic sebaceous glands in oral mucosa.
yellow-white papules most commonly in buccal & vermilion area. less common in retromolar & tonsilar area.
hyperplastic sebaceous glands (sebum).
no treatment
leukoedema
unknown cause, common in blacks (70-90%).
diffuse, grayish-white milky opalescent buccal tissue appearance.
does not rub off, bilaterally symmetrical, disappears upon stretching
histologically: intracellular edema of spinous layer
No treatment necessary
developmental tongue defects
aglossia micro (hypo) glossia macroglossia ankyloglossia (tongue-tied) lingual thyroid fissured tongue (scrotal tongue) black hairy tongue lingual varicosities
microglossia
uncommon
range from totally missing (aglossia) to slightly small
often associated with syndrome and/or malocclusion
macroglossia
uncommon
may cause drooling, difficulty eating, speech trouble, open bite, crenated lateral border of tongue.
congenital & hereditary: lymphangioma, hemangioma, cretinism, Down syndrome
aquired: edentulous mandible, myxedema, acromegaly
ankyloglossia
short thick lingual frenum, limits protrusion.
1.7-4.4% of births, more common in males.
speech impediments
frenectomy
lingual thyroid
embryonic primitive thyroid fails migration.
more common in females.
may cause dysphasia, dysphonia & dyspnea.
diagnose with radioactive thyroid scan, don’t remove
fissured tongue (scrotal tongue)
common (2-5% population), usually asymptomatic
deep grooves on dorsal tongue, mild burning or soreness.
unknown cause, associated with geographic tongue.
brush tongue
black hairy tongue
elongated filiform papillae, accumulates keratin & debris.
halitosis, bad taste, gagging.
associated with smoking, antibiotics, poor hygiene, post radiation, excess mouthwash/antacids.
txt: tongue brushing or scraping
lingual varicosities
dilated & tortuous veins.
most commonly sublingual, then lips & buccal mucosa.
common in elderly
no treatment
exostoses
bony protuberances torus palatinus torus mandibularis buccal exostoses palatal exostoses subpontine exostoses
buccal exostoses
bony hard nodule along facial of max. & mand.
asymptomatic, male=female
surgical removal for pre-prosthetic surgery or recurrent trauma
torus palatinus
very common (20-35% population).
female 2:1 male.
surgical removal for pre-prosthetic surgery or recurrent trauma.
dense cortical bone along palate midline
torus mandibularis
very common (7-10% population).
slightly more male.
sublingual area.
bilateral 90% of cases
eagle syndrome (stylohyoid syndrome)
facial pain & pain on swallowing or turning head/wide opening of mouth.
elongation & calcification of stylohyoid ligament.
may need surgical removal
Stafne defect
submandibular gland depression.
can be seen below premolars when associated with sublingual gland.
more common in males.
asymptomatic, no treatment.
developmental cysts
fluid/debris filled cavity lined by epithelium & not inflammatory origin.x palatal cyst nasolabial cyst nasopalatine duct cyst median palatal cyst epidermoid cyst dermoid cyst thyroglossal duct cyst branchial cleft cyst oral lymphoepithelial cyst
palatal cysts of newborns
extremely common (65-85% neonates)
1-2mm white-yellow papule near midling junction of hard/soft palate.
keratin filled, lined by squamous epithelium.
no treatment, spontaneous rupture.
nasolabial cysts (nasoalveolar cyts)
rare
upper lip, under the alae of nose.
usually without pain, but elevates lip and fills vestibule.
female 3:1 male, 4th-5th decade.
surgical removal
cyst lined with pseudostratified columnar epi.
nasopalatine duct cysts
MOST COMMON non-odontogenic cyst of oral cavity (1% population).
from epithelial remnants of nasopalatine duct.
depends on location, can be named cyst of palatine papillae or nasopalatine duct cyst.
slightly more male, 4-6th decade.
asymptomatic or swelling with pain.
suspect if radiolucency >6mm, surgical removal.
median palatal cyst
rare cyst at palate fusion point, midline posterior hard palate.
asymptomatic, but may have pain/swelling.
surgery
epidermoid cyst
usually hair follicle in acne-prone head, neck & back.
young adults, males
dermoid cysts
similar to epidermoid cyst, also in adnexal structures (sebaceous glands, hair follicles, or sweat glands).
oral cysts usually in floor of mouth.
surgery
thyroglossal duct cyst
epithelial remnants of thyroglossal tract.
generally painless, movable swelling on neck midline.
2nd-3rd decade.
can move during swallowing if attached to tongue.
rarely forms carcinoma.
surgery
cervical lymphoepithelial cyst
lateral neck on anterior sternocleidomastoid of young adults.
surgery
oral lymphoepithelial cyst
small, yellow-white mass.
asymptomatic, young adults.
most common in floor of mouth, lateral tongue, tonsil, & soft palate
surgery
Crouzon syndrome (craniofacial dysostosis)
early closure of cranial sutures leads to increased cranial pressure, migraines, bulging eyes, underdeveloped maxilla.
multiple surgeries
Apert syndrome (acrocephalosyndactyly)
early cranial suture closure, tall forehead, bulging eyes, vision impairment, small maxilla, hearing loss, fused digits of hands and feet, mental retardation, mouth breathing, typically class III occlusion. multiple surgeries and ortho
Treacher Collins syndrome (mandibulofacial dysostosis)
narrow face, depressed cheeks, ear defects, underdeveloped mandible.
multiple surgeries and ortho
hypophosphatasia
hereditary, decrease serum alkaline phosphatase effects calcification of bone/cementum.
premature loss of deciduous anterior teeth.
bowed legs and bone fractures.
gingival fibromatosis
firm fibrous gingival overgrowths begun in childhood.
surgery
cherubism
bilateral face swelling, 1-4yrs old.
mandible most affected.
soap bubble appearance of mand. radiographs.
facial deformities for life.
cleidocranial dysplasia
skull enlarged, small face.
underdeveloped/missing clavicles.
many unerupted supernumerary teeth interfere with normal teeth.
gardners syndrome
main feature: osteomas, including jaws.
polyps in colon, 100% malignant after 30yrs old.
multiple odontomas.
treat with prophylactic colonectomy, remove osteomas
nevoid basal cell carcinoma syndrome
increased inter-eye distance, basal cell carcinoma of head/neck skin, odontogenic keratocysts, skeletal abnormalities (bifid ribs).
surgical removal of cysts and carcinomas
hereditary hemorrhagic telangiectasia
small blood vessel defect, dilate and become visible on skin/mucous membranes.
lesions prominent on anterior dorsal tongue.
may interfere with hygiene procedures, treat bleeding lesions.
