Cyst arising w/in intraoral lymphoid tissue

-Lymphoid tissue normally found in oral cavity in Waldeyer's ring, accessory oral tonsils or lymphoid aggregates in floor of mouth, ventral tongue and soft palate

- Oral epithelium often invaginates into tonsillar tissue forming crypts which can close over and fill w/ keratin resulting in OLC

young adults

M=F

50% occur in floor of mouth, ventral/posterior tongue, anywhere else oral lymphoid tissue is found

less than 1 cm white or yellow submucosal mass, firm or soft

- May contain/express creamy or cheesy keratin; overlying mucosa may be smooth or ulcerated

- Usually asymptomatic unless secondarily inflamed

Treatment and prognosis of oral lymphoepithelial cyst

Simple surgical excision

Excellent

asymptomatic unilateral overgrowth of one or more parts of body: entire body, one or both limbs, head

- May be isolated finding or associated with syndromess

- Almost all isolated cases are sporadic, not inherited

Can be detected at birth or not evident until later childhood

F>M 2:1

Right side of body>left side

changes may involve all tissues on affected side

- Skin often thickened and may show increased pigmentation, hypertrichosis, telangiectasias, nevus falmmeus ("port wine stain")

Increased prevalence of abdominal tumors - 5.9%

- Wilms' tumor, adrenal cortical carcinoma, hepatoblastoma

Macroglossia w/ prominent papillae

Enlargement of soft tissues, bone, mandibular canal, sinuses, crowns & roots of teeth on affeted side, premature development & eruption of teeth, root resorption, malocclusion w/ open bite

- Evaluation to rule out additional syndromes & abdominal tumors

cosmetic surgery

Ortho treatment

Good

Orbits shallow --> ocular proptosis

Visual impairment or total blindness & hearing deficit

some may have headaches (from intracranial pressure)

Maxilla underdeveloped (max. crowding)

1/65,000 births

Autosomal dominant, sporadic new mutations - paternal origin assoc. w/ paternal age

Tower skull

Occiput is flattened

Tall appearance to forehead

Middle third of face is significantly retrued & hypoplastic --> relative mandibular prognathism

Narrow nasopharynx --> mouth breathers

Middle ear infectionsc ommon + conductive hearing loss

Mental retardation common

3/4s of all pts exhibit cleft of soft palate or bifid uvula

Maxillary hypoplasia --> v-shaped arch and crowding

Class III malocclusion usually occurs

Swelling along lateral hard palate

gingival thickening --> delayed eruption of teeth

shovel-shaped incisors 1/3 of pts

cosmetic surgies

craniectomy

Ortho

- Autosomal dominant, variable expressivity

- 1/25,000 to 50,000 live births

- 60% of cases new mutations, increased incidence w/ increased paternal age

- 75% have coloboma (notch) on outer part of lower eyelid

50% no eyelashes medial to coloboma

- Sideburns show tongue-shaped extension toward cheek

- Ears may show deformity or misplaced pinna, extra ear tags; ossicle defects or absence of external auditory canal can cause conductive hearing loss

- Mandible underdeveloped --> retruded chin

- Hypoplasia of condylar & coronoid processes w/ prominent antegonial notching

- Mouth downward w/ 15% incidence of lateral facial cleft & cleft palate in 1/3 cases

- Parotid glands may be hypoplastic or absent

- Hypoplasia of naso- and oropharyngeal structures

Mild cases - not much treatment

Others require surgery - cosmetic, functional deficits

Ortho

Good prognosis

- Wide range of appearance, extent and severity

- Most common in bicuspids

- Similar enamel defects reported in permanent successors associated with caries or trauma to overlying deciduous teeth

Defects of one or more 1st permanent molars

Altered enamel may be white, yellow, or brown w/ sharp demarcation b/t defective & surrounding normal enamel

Enamel soft and porous

Often sensitive to cold, warm, or mechanical trauma