developmental anamolies of soft tissues of oral cavity

7/27/2019 developmental anamolies of soft tissues of oral cavity

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DEVELOPMENTALANOMALIES OF SOFT

TISSUES OF ORAL

CAVITY


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OVERVIEW

I. OROFACIAL CLEFTS:

- Cleft Lip & Cleft palate

II. ANOMALIES OF TONGUE:

- Microglossia & Macroglossia

- Ankyloglossia

- Hairy tongue

- Fissured tongue

- Lingual thyroid

III. ANOMALIES OF ORAL MUCOSA:

- Fordyce granules

- Leukoedema


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OROFACIAL CLEFTS


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Development of face and oral cavity

involves the development of variousfacial processes that fuse with each

other.

Any disturbance in growth of theseprocesses can result in formation of

orofacial clefts.

Thus it is essential that facialdevelopment be reviewed in brief.


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Central face begins to developby 4thweek, when olfactory

placodes appear on both sideof frontonasal process.

Gradually both placodesdevelop to form medial and

lateral nasal processes.

Upper lip is formed by 6thweekby fusion of two medial nasal

processes in midline and themaxillary process of the 1stbranchial arch.


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Primary palate alsoforms by fusion of

medial nasal processes

to form the premaxillawhich includes the four

incisor teeth.


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Secondary palate is formedfrom the maxillary processes

of the 1stbranchial arch.

By the 6thweek bilateral

projections emerge from

medial aspect of maxillary

processes to form palatalshelves.


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Initially these shelves are

vertically oriented on eitherside of developing tongue.

As mandible grows, tongue

drops down allowing palatal

shelves to rotate horizontallyand fuse in midline by 8thweek.

Palatal shelves also fuse withthe primary palate anteriorlyand nasal septum superiorly.


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Defective fusionof medial nasal

process with themaxillary process

leads to cleft lip

(CL).


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Similarly, failureof fusion of

palatal shelvesleads to cleft

palate (CP).


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Frequently, CL & CP occur together.

Approximately 45% cases are CL + CP,

while 30% are isolated CL and 25%isolated CP.


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Both, isolated CL and CL associated

with CP (CL+ CP) are thought to beetiologically related conditions andare considered as a group.

Isolated CP appears to represent a

separate entity.


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Other rare facial clefts like lateral facial cleftoccurs as a result of failure of fusion of

maxillary and mandibular processes leadingto a cleft from corner of mouth toward ear.

Oblique facial cleft extends from upper lip

towards the eye and is almost alwaysassociated with CP. Caused by failure offusion between maxillary process with lateralnasal process.

Midline cleft of upper lip is extremely rare andrepresents failure of fusion of medial nasalprocesses in the midline.


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ETIOLOGY Cause is still being debated.

Important to distinguish between isolatedclefts and clefts associated withdevelopmental syndromes.

More than 250 syndromes are associated withorofacial clefts and account for 3% - 8% oforofacial clefts, e.g. Pierre Robin syndrome,

Treacher-Collins syndrome etc. Environmental factors like alcohol

consumption, viruses etc also may combinewith developmental factors.


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CLINICAL FEATURES

RACIAL PREVALENCE: - Clefting is one of the most common

congenital defects in humans.

Prevalence varies between races, with whiteshaving prevalence of 1 in 1000 births, while itis 1.5 times higher in Asians and leastamongst blacks (0.4 in 1000 births).

Isolated CP is less common than CL + / - CP.


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SEX PREDILECTION: -

CL + / - CP is more common in males. More severe the defect, greater is the male

predilection.

Male to female ratio for isolated CL is 1.5 :1.

M:F ratio for CL+CP is 2 : 1.

In contrast, isolated CP is commoner infemales with the M:F ratio being 1 : 2.


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SIGNS & SYMPTOMS: -

About 80% cases of CL are unilateral,with 70% of unilateral cases occurring

on left side.

A complete CL extends till the nose

A CP shows considerable variation in

severity, with the defect involving

both hard and soft palate or softpalate alone.


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The minimal

manifestation

of CP is a BIFID /CLEFT UVULA.


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Cleft patients suffer from many direct as

well as well as indirect problems. The most obvious problem is clinical

appearance leading to psychosocial

problems. Feeding and speech difficulties are

common especially with CP.

CP also causes collapsing of maxillary arch,missing of teeth, supernumerary teeth ,leading to malocclusion.


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TREATMENT & PROGNOSIS

Treatment is multidisciplinary, involvingPediatrician, Otolaryngologist, Oral &maxillofacial surgeon, Pedodontist, Plastic

surgeon, speech therapist and geneticcounselor.

Surgical repair involves multiple primary

and secondary procedures throughoutchildhood, depending on severity of defectand philosophy of the team.


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DEVELOPMENTAL

ANOMALIES OFTONGUE


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OVERVIEW OF TONGUE DEVELOPMENT

The medial most parts of

the mandibular archesproliferate to form twoLINGUAL SWELLINGS.

They are partially

separated from each other

by another swelling that

appears in the midline,known as TUBERCULUM

IMPAR.


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Immediately behindthe tuberculum impar,the epitheliumproliferates to form

down growth fromwhich the thyroidgland develops. Thesite of this down

growth is subsequentlymarked by thedepression, called asFORAMEN CAECUM


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Tongue is divided into twoparts:

1) Anterior 2/3rdof thetongue.

2) Posterior 1/3rdof thetongue.

Anterior 2/3rdof tongue isformed by fusion of:-

a) the tuberculum impar

b) the two lingual swellings

Anterior 2/3rdof tonguederived from themandibular arch.


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Posterior one third of thetongue is derived from thecranial part of hypobranchialeminence (copula)

In this situation, the secondarch mesoderm gets buried

below the surface. The thirdarch mesoderm grows overit to fuse with themesoderm of the first arch.

Posterior most part oftongue is derived from 4THarch.


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MICROGLOSSIA

Uncommondevelopmental

anomaly ofunknown cause ,

characterized by

an abnormallysmall tongue.


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In extremely rare cases, tonguemay be entirely missing(Aglossia).

Mild degrees of microglossia may

be difficult to detect.


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Usually associated with syndromes likeoromandibular limb hypogenesis

syndrome.

This syndrome is characterized byhypodactylia (absence of digits) and

hypomelia (hypoplasia of part or all of alimb).

Microglossia is also associated frequentlywith hypoplasia of mandible and lowerincisors may be missing.


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TREATMENT & PROGNOSIS: -

Depends on nature and severity ofcondition.

Surgery and orthodontics may

improve oral function.

Speech development is quite good

but depends on tongue size.


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MACROGLOSSIA

Characterized by enlarged tongue.

Can be caused by both congenital

malformations and acquired diseases.

Most frequent causes are vascularmalformations and muscularhypertrophy.


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1. CONGENITAL & HEREDITARY: -

Vascular malformations

- lymphangioma

- hemangioma

Hemihyperplasia

Cretinism

Down syndrome

Neurofibromatosis

Multiple endocrine neoplasia, type 2B

Beckwith-Weidmann syndrome


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2. ACQUIRED: -

Edentulous patients Amyloidosis

Myxedema

Acromegaly

Angioedema

Carcinoma and other neoplasia


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CLINICAL FEATURES ; -

Common in children.

Can be mild to severe in degree.

Manifested in children as noisy breathing,drooling, difficulty in eating and lisping

speech.

Tongue pressure against mandible cancause scalloped border of tongue, open

bite and mandibular prognathism.


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Patients with

hypothyroidism orsyndromes presentwith diffuse, smooth,

generalized

enlargement.

Patients with

amyloidosis, neoplasia

and neurofibromatosisshow nodular

enlargement.


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Tongue inlymphangiomapatients appears as

a pebbly surfacewith multiple vesiclelike blebs.


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Down syndromepatients showpapillary fissured

surface. In hemifacial

hyperplasia,

enlargement isunilateral.


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Depends on cause and severity ofcondition.

Mild cases may not require any

treatment. Speech therapy is helpful if speech is

affected.

Reduction glossectomy may beneeded in larger enlargement.


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ANKYLOGLOSSIA (TONGUE TIE)

Characterized by a short, thick lingualfrenum, resulting in limitation of

tongue movement.

Male to female ratio is 4 : 1.

Reported to occur in 2-3 persons out

of every 10,000 people.


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CLINICAL FEATURES: -

Ranges in severity from mild to severe,

where the frenum is actually fused to floorof mouth due to absent frenum.

Can result in speech defects.

Usually however, the shortened frenum

results only in minor difficulties as manypeople can compensate for limitation intongue movement.


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Since most cases are mild, treatmentis often unnecessary.

Frenectomy is recommended if thereare functional or periodontal

problems.


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LINGUAL THYROID Thyroid gland begins as an

epithelial proliferationbehind the tuberculum imparin the floor of pharyngeal gutby the 3rd4thweek of IUL.

By 7

th

week, the glanddescends into the neck to itsfinal resting place.

The site where the

embryonic bud invaginates,later becomes the foramencecum at the junction ofanterior 2/3rd& posterior

1/3rd


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Male to female ratio isabout 1 : 4.

Symptoms develop by

puberty , pregnancy or

menopause. In 70% of cases, this

piece of tissue is the

only thyroid tissue in thepatient.


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Lingual thyroids may range from

small, asymptomatic , nodular lesionsto large masses that block airway.

Common symptoms are dyspnea,

dysphagia and dysphonia.

No distinct features are present to

differentiate it from other massesdeveloping on the tongue.


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Hypothyroidism has

been reported in about

33% of patients.

Diagnosis isestablished by thyroid

scan using iodine

isotopes or technetium

99m.


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Biopsy is often avoided as themass may be highly vascular

and may represent thepatients only functioning

thyroid tissue


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No treatment required for small,asymptomatic masses.

In larger lesions, suppressive therapywith supplemental thyroid hormone

can often reduce the size of the mass.


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FISSURED TONGUE

Relatively common condition. Numerous grooves or fissures are

present on dorsal surface of tongue.

Cause uncertain, but heredity plays animportant role.

Aging or local environmental factors

may also contribute to its

development.


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Age incidence:Prevalence &severity increases with age.

Sex predilection:slight male

predilection seen.


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Signs & symptoms:

Multiple grooves / furrows on

dorsal surface ranging from 26 mm in depth.

In severe cases, tongueappears divided into multipleislands by the deep fissures.

Condition usually

asymptomatic.

Strong association with

geographic tongue.


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HISTOPATHOLOGICAL FEATURES: -

Hyperplasia of reteridges and loss ofkeratin hairs from

surface of filliformpapillae.

Papillae vary in size

and are oftenseparated by deepgrooves.


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PMNLs can be noted

migrating into the epithelium,forming micro abscesses.

Mixed inflammatory infiltrate

present in underlying laminapropria.


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Being a benign condition, no specifictreatment is required.

Patient should be encouraged tobrush his tongue as food entrapped in

the deep fissures of tongue may act

as a source of irritation.


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HAIRY TONGUE

Characterized by marked accumulation ofkeratin on filliform papillae, resulting in ahairy appearance of dorsal surface oftongue.

Represents probably an increase in keratinproduction or a decrease in normal keratindesquamation.

Found in approximately 0.5% of adults.


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ETIOLOGY: -

Cause is uncertain, but many of affected

patients are heavy smokers. Other possible associated factors include:

- Antibiotic therapy

- Poor oral hygiene- General debilitation

- Radiation therapy

- Use of oxidizing mouthwash / antacids- Overgrowth of fungal / bacterial

organisms.


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CLINICAL FEATURES:

Commonly affects

midline, just anteriorto circumvallatepapillae.

Elongated papillaeare brown, yellow orblack as a result ofgrowth of pigment

producing bacteriaor tobacco staining.


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Condition is usually asymptomatic,although in severe cases, patients may

complain of gagging sensation.

Because of similarity in names, care must

be taken to differentiate this conditionfrom hairy leukoplakia (HL).

HL occurs on lateral borders of tongue, iscaused by Epstein-Barr virus and isusually associated with HIV infection orother immunosuppressive conditions.


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HISTOPATHOLOGICALFEATURES: -

Characterized bymarked elongation andhyperparakeratosis ofthe filliform papillae.

Usually, numerousbacteria can be seengrowing on the surface

of epithelium.


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Benign condition with no serious

sequelae.

Esthetic concerns and associated bad

breath problems should be taken careof.

Desquamation can be promoted by

periodic scraping with a toothbrush /tongue scraper.


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DEVELOPMENTAL

ANOMALIES OF ORALMUCOSA


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FORDYYCE GRANULES

Considered ectopic collection ofsebaceous glands occurring on the

oral mucosa.

However since 80% of the populationshows their presence, their presence

in oral mucosa must be considered a

normal anatomic variation.

l l


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Present as multipleyellow or yellowish white

papules.

Occur most commonly on

buccal mucosa and

vermilion border of upper

lip. More common in adults

than children.

Mostly asymptomaticlesions. Some patientsmay feel some roughness

of the mucosa.

HISTOLOGICAL FEATURES


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HISTOLOGICAL FEATURES:

Appear similar to normalsebaceous glands exceptfor absence of associatedhair follicles.

Acinar lobules locatedimmediately beneathepithelial surface oftencommunicating with thesurface via a central duct.

Sebaceous cells within aciniare polygonal, with centralnuclei and abundant foamycytoplasm.


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As they are asymptomatic andrepresent a normal anatomic

variation, no treatment is indicated.

Biopsy also is not necessary as clinicalappearance is characteristic.

Occasionally, Fordyce granules may

become hyperplastic or form keratin

filled pseudo cysts.


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LEUKOEDEMA

Common condition of unknowncause.

Occurs more in blacks than whites.

Because it is so common, it can beconsidered a normal anatomic

variation rather than a disease.

Some studies indicate that it may bemore common in heavy smokers.

CLINICAL FEATURES


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Age incidence:Children

Sex predilection:Nil

Racial predilection:More in blacks

Site of occurrence: Primarily bilaterallyon buccal mucosa. May occur on floorof mouth also.


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Presents as diffuse,grayish white,

milky opalescentappearance of oralmucosa.

Lesions do not ruboff.

Surface appearsfolded, resulting in

whitish streaks.

C b di d


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Can be diagnosed

easily clinically

because the whiteappearance

greatly

disappears whenthe cheek is

everted and

stretched.


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HISTOPATHOLOGICAL

FEATURES: -

Epithelium appearsthickened withpronounced intracellular

edema of spinous layer.

These cells appearvacuolated, having

pyknotic nuclei.

Epithelial surface isusually parakeratinizedwith broad and

elongated rete ridges.


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Benign condition, needs notreatment.

Can be distinguished from othercommon white lesions like

leukoplakia, candidiasis by everting

and stretching the mucosa.


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BIBLIOGRAPHY

1. Soames JV, Southam JC. Oral pathology/. 3rded. Oxford 2002.

2. Shafer WG, Hine MK, Levy BM. A text book

of oral pathology. 6thed. W.B. SaundersCompany. Phil, London, Toronto, 2005.

3. Neville BW, Damm DD, Allen CM, Bouquot

JE. Oral and maxillofacial pathology. 2nd

ed.WB Saunders Company. Phil, London,Toronto, 2007.


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ACKNOWLEDGEMENT

All pictures in this presentation are courtesyof authors mentioned in the bibliographyand the following authors also:

1. Sadler , Langmans, Medical Embryology,Ed. 6.

2. James e Anderson, Grants Atlas ofAnatomy. Williams & Wilkins


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developmental anamolies of soft tissues of oral cavity

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