Lecture 9 - Developmental Defects of the Head & Neck

what is the etiology of a cleft lip?

developmental/genetic

what causes cleft lip?

defective fusion of median nasal processes with maxillary processes

what are the clinical features of cleft lip?

more commonly unilateral (L>R); complete extends up into nostril, between lateral incisor and cuspid, with teeth usually missing; incomplete do not involve nose

what causes cleft palate?

defective fusion of palatal shelves

what are the clinical features of cleft palate?

wide range of severity; hard and soft palate or soft palate only; submucous palatal cleft with surface mucosa intact but defect is in underlying musculature, can appear as bluish midline discoloration; bifid uvula is most minimal manifestation; pierre robin sequence

what is the pierre robin sequence?

cleft palate, mandibular micrognathia, glossoptosis

what causes lateral facial cleft?

lack of fusion of maxillary and mandibular processes

what can lateral facial cleft cause?

macrostomia

what causes oblique facial cleft?

failure of fusion of lateral nasal process with maxillary process/amniotic bands

what is oblique facial cleft almost always associated with?

cleft palate

what causes median cleft of upper lip?

failure of fusion of medial nasal processes

are commissural lip pits associated with cleft lip/palate?

no

what are the clinical features of commissural lip pits?

mucosal invaginations at vermillion border at corners of mouth; more common in men; blind fistulas 1-4 mm

what are the clinical features of paramedian lip pits?

bilateral fistulas on either side of vermillion of LL; inherited with CL +/- CP; most common form of syndromic clefting

what are the clinical features of double lip?

RARE; redundant fold of tissue on mucosal side of lip; much more common on UL; may be from trauma or oral habits; ascher syndrome component

what are the components of ascher syndrome?

double lip, blepharochalasis, nontoxic thyroid enlargement

what are the clinical features of varicosities?

sublingual most common; multiple blue-purple elevated blebs; thrombosed -> firm, nontender nodule

what are the clinical features of buccal exostoses?

asymptomatic, bilateral row of bony nodules along F aspect of maxillary and/or mandibular ridge

what are the clinical features of palatal exostoses?

usually bilateral on lingual aspect of maxillary tuberosity, common in males

what causes solitary exostoses?

may occur due to local irritation

what causes reactive subpontine exostoses?

alveolar crestal bone beneath pontic

what is the treatment of exostoses?

removal to accommodate dental prostheses

if an exostoses is not clinically obvious, what should be done?

biopsy

what are the clinical features of torus palatinus?

exostoses on MIDLINE of hard palate; flat, spindle, nodular or lobular usually <2cm; slowly increases in size throughout life; more common in women

what are the clinical features of torus mandibularis?

exostoses on lingual aspect of mandible; common in premolar area, BILATERAL; may appear on periapical films as radiopacity superimposed on roots

what causes a stafne defect?

focal concavity of cortical bone on lingual surface of mandible from submandibular gland

what are the clinical features of a stafne defect?

asymptomatic radiolucency below mandibular canal commonly between molars and angle of mandible, well-circumscribed with sclerotic border, may interrupt continuity of inferior border with palpable notch in area; unilateral, more common in men; usually do not appear until adulthood

what is the treatment for stafne defect?

none

what are the clinical features of a palatal cyst of newborn?

1-3 mm white-yellow papules; most often along midline near junction of soft and hard palate; keratin-filled cysts with stratified squamous lining

what is the treatment for a palatal cyst of newborn?

self-healing

what are the clinical features of nasolabial cysts?

swelling of upper lip lateral to midline causing elevation of ala; pain is rare; 4th/5th decade, more common in women

are the there radiographic changes with a nasolabial cyst?

no

what is the treatment of a nasolabial cyst?

surgical excision

what is the most common non-odontogenic cyst of the oral cavity?

nasopalatine duct cyst

what are the clinical features of a nasopalatine duct cyst?

4th to 6th decades, more common in men; swelling of anterior palate, drainage and pain; may be discovered radiographically; expect to see nerves and blood vessels in walls of cyst

what is a nasopalatine duct cyst that is only found in the soft tissue known as?

cyst of incisive papilla

what are the radiographic features of nasopalatine duct cyst?

well-circumscribed radiolucency near midline of anterior maxilla; round-oval with sclerotic border; >6mm; +/- root divergence

what is the treatment of a nasopalatine duct cyst?

surgical enucleation

what is the most common epidermoid cyst?

follicular cysts

what are the clinical features of pilar cysts?

common moveable scalp cyst, also derived from hair follicle, F>>M

what are the clinical features of epidermoid cysts?

most common in acne prone areas of head, neck, back; more common in young men; present as nodular, fluctuant, subcutaneous lesions

what are epidermoid cysts associated with?

gardner syndrome

what are the clinical features of milia?

tiny keratin-filled cyst, resembling miniature epidermoid cysts

what is the treatment for follicular cysts?

surgical excision

what are the clinical features of dermoid cysts?

most common on midline FOM; children and young adults; doughy/rubbery mass that pits; above geniohyoid causes sublingual swelling, tongue displacement, difficulty eating and speaking; below geniohyoid causes double chin; slow growing, painless

what is the treatment for dermoid cysts?

surgical removal

if no skin appendages are involved, what is a dermoid cyst known as?

epidermoid cyst

what are the clinical features of thyroglossal duct cyst?

anywhere along midline from base of tongue to suprasternal notch; usually develop during first two decades of life; painless, fluctuant, moveable swelling; if attached to hyoid or tongue - move vertically during swallowing

what is the treatment of a thyroglossal duct cyst?

sistrunk procedure

what is a sistrunk procedure?

cyst removal along with midline segment of hyoid bone and muscular tissue

what are the clinical features of a lingual thyroid?

more common in women, symptoms developing during puberty or pregnancy; 70% only involve gland; dysphagia, dysphonia, dyspnea; vascular appearance

how is lingual thyroid diagnosed?

thyroid scan with iodine isotopes

what is the treatment for lingual thyroid?

no treatment if asymptomatic; suppressive therapy with supplemental thyroid hormone

what are the clinical features of a branchial cleft cyst?

cyst from second arch in upper lateral neck anterior or deep to SCM; most in children and young adults; soft, fluctuant mass 1-10 cm; +/- tenderness or pain

what is the treatment for branchial cleft cyst?

surgical excision

what are the clinical features of an oral lymphoepithelial cyst?

young adults; small, submucosal mass <1.5 cm that is white-yellow in color; asymptomatic; FOM, ventral tongue, posterior lateral border of tongue, palatine tonsil and soft palate

what are the common lymphoid tissues that have lymphoepithelial cysts?

waldeyer's ring, accessory lymphoid tissue on FOM, ventral tongue, soft palate

what is the treatment for an oral lymphoepithelial cyst?

surgical excision