Developmental lesions of face

Classification


Broadly classified into  Developmental disturbances of tongue  Developmental disturbances of gingiva  Developmental disturbances of lips and palate  Developmental disturbances of oral lymphoid tissue  Developmental disturbances of salivary glands  Developmental disturbances of oral mucosa  Developmental cysts of the oral region

Developmental disturbances of tongue

Developmental disturbances of tongue

classified into

 Microglossia  Macroglossia  Ankyloglossia  Cleft

tongue  Fissured tongue  Median rhomboid glossitis  Geographic tongue  Hairy tongue  Lingual varices  Lingual thyroid nodule

Microglossia
 Small  C/F
 Uncommon

or rudimentary tongue

developmental condition  Aglossia rare  Often associated with one of a group of the overlapping conditions known as Oromandibular limb hypogenesis syndrome
Limb anomalies hypodactylia (absence of digits)  Hypomelia hypoplasia of part or all of a limb  Some patients associated with cleft palate, intra oral bands  Also frequently associated with hypoplasia of mandible


Microglossia

Microglossia
Treatment
 Depends

and prognosis
on the nature and severity of the

condition  Surgery and orthodontics may improve oral function  Speech development is quite good but depends on tongue size

Macroglossia
Enlargement

of the tongue Caused by a wide variety of conditions including both congenital malformations and acquired diseases True macroglossia tongue enlargement Relative macroglossia insufficient space in the oral cavity

Macroglossia
 C/F:
 Most

common in children  Degree of macroglossia Mild to severe  Infants manifest noisy breathing, drooling, difficulty in eating, lispy speech  Pressure of tongue against mandible and teeth produce
Crenated lateral borders of tongue  Open bite  Mandibular prognathism


 Constant

protrusion produces ulceration  If severe, airway obstruction can be produced

macroglossia

Macroglossia
Feature


of Beckwith-Wiedemann syndrome

Visceromegaly  Gigantism  Neonatal hypoglycemia (Prone to Wilms tumor, Adenocarcinoma and Hepatoblastoma)

Macroglossia
 Facial

features include,

 Nevus

of forehead and eyelids  Linear indentations of the ear lobes  Maxillary hypoplasia
 AD

inheritance  Hypothyroid macroglossia enlargement is smooth, diffuse and generalized  Amyloidosis, neurofibromatosis and MEN IIB syndrome produce nodular type of enlargement

Macroglossia
Lymphangiomas

tongue surface shows blebbing with multiple vesicle like blebs Downs syndrome has papillary and fissured surface Hemi-facial hyperplasia shows unilateral enlargement Edentulous patients has lateral spread out of the tongue.

Macroglossia
 H/P:
 Microscopy

depends on the cause  Downs and edentulous patients normal  Amyloidosis shows abnormal proteins  Tumors show abnormal proliferation  Beckwith shows muscular enlargement
 Ttt

and Prognosis:

 Mild

no surgery, speech therapy  Severe Partial glossectomy

Ankyloglossia/Tongue tie
Developmental

anomaly Characterized by short, thick lingual frenum Complete fusion between tongue and floor of the mouth Partial tongue tie short lingual frenum attached to the tip of the tongue Occur in 1.7 to 4.4% of neonates Four times more common in boys than girls

ankyloglossia

Ankyloglossia/Tongue tie
 C/F:
 Speech

difficulties due to restricted tongue movement  High mucogingival attachment cause periodontal problems  Some investigators say ankyloglossia cause open bite due to abnormal swallowing pattern  Ankyloglossia associated with upward and forward displacement of epiglottis resulting in dyspnoea.

Ankyloglossia/Tongue tie
A.

superior (Glossopalatine ankylosis)

 Rare

- congenital adherence of tongue to the palate  Usually combined with other congenital anomalies in the maxillofacial region and extremities (A. superior syndrome)  Causes suckling and respiratory dysfunction

(JOMFS,95 53:588-589)

Ankyloglossia superior

Ankyloglossia/Tongue tie
Ttt

and prognosis:

 Most

cases do not exhibit any clinical problem and ttt is not required  In children, mostly self corrective  If functional and periodontal problems develop, frenectomy to allow free tongue movement  A.superior requires surgical separation

Cleft tongue / Bifid tongue

cleft:

a rare condition  Caused due to lack of merging of lateral lingual swellings

cleft:

more common  Manifested as a deep groove in the midline on the dorsal tongue  Results due to incomplete merging and failure of groove obliteration by the underlying mesechymal proliferation

Cleft tongue

Cleft tongue / Bifid tongue

of Oro-facial-digital syndrome in association with

Rarely

associated with epignathus teratoma, cleft palate, median glossal salivary mass
(Mills et al, JOMFS,2004 379-383)

Bifid tongue with median glossal salivary mass

Cleft tongue / Bifid tongue

and Prognosis:

No clinical significance except collection of food debris and microorganisms at the base of the cleft, which may cause irritation  If marked surgery

Fissured tongue
Relatively common anomaly  Presents as numerous grooves or fissures  Etiology is uncertain  Different etiology is suggested

 Aging

and vitamin deficiency (Halperin et al)  Hereditary AD  Children with extra oral congenital anomalies  Children with a history of allergy (Bessa et al JOPOM2004:1722)

Fissured tongue
Based

on clinical appearance, classified as

and

Fissured tongue
 C/F:
 Prevalence

2-5% of population  Groove depth is 2-6mm  Seen in children and adults  Prevalence increase with age  Mild to severe
Mild shallow fissures only on the dorsum of tongue  Severe numerous fissures covering the entire dorsum and dividing the tongue papillae into multiple separate islands


Fissured tongue

Fissured tongue
Some

patients have fissures extending dorsolaterally Some have large central fissures with radiating fissures Usually asymptomatic except mild soreness or burning sensation Strong correlation between Fissured tongue and Geographic tongue

Fissured tongue

Fissured tongue
H/P:
 Hyperplasia

of rete ridges  Loss of keratin on the surface of filiform papillae  Papillae vary in size and separated by deep grooves  PMN migrate into the epithelium forming microabscesses in the upper epithelial layers.  Mixed inflammatory cell infiltrate in the lamina propria

Fissured tongue
Ttt

& Prognosis:

 Usually

no specific ttt  Patient encouraged to brush the tongue to remove the entrapped food debris in the grooves which may act as a source of infection

Median Rhomboid Glossitis

known as Central Papillary Atrophy of tongue Described classically as congenital anomaly occurring due to failure of tuberculum impar to retract or withdraw before fusion of the lateral half of the tongue Hence the structure devoid of papillae is interposed between them

Median Rhomboid Glossitis

possibility of candidal infection is suggested,

there is no report in children  More common in diabetics  Hyphae demonstrated in some histologic sections  Lesion resolves on antifungals
Prevalence

is 2-3%. Three times more frequent in mentally retarded

Median Rhomboid Glossitis

diamond or rhomboid shaped  Reddish patch or plaque like  Located on the dorsal surface of tongue immediately anterior to the circumvallate papillae  De-papillated no filiform papillae  Obvious clinically, often asymptomatic  Smooth or lobulated

Median rhomboid glossitis

Median Rhomboid Glossitis

MRG occurs with oral candidiasis in other sites erythematous candidiasis termed as Chronic multifocal candidiasis Carcinomatous change has been reported When a lesion in the location of MRG accompanied with induration, soreness or pain, a neoplastic origin should be suspected. Biopsy is then mandatory.
(Drosky et al,JOMFS, 1993: 51-798-800)

Median Rhomboid Glossitis

of papillae with varying degrees of hyperkeratosis  Proliferation of spinous layer with elongation of rete ridges  Lymphocytic infiltrate within the connective tissue  Numerous blood vessels and lymphatics are seen  Degeneration and hyalinization within the underlying muscle  Fungal hyphae in the Parakeratin or very superficial spinous layer or both.  Best seen by PAS stain


Ttt:
 No

specific ttt  Some lesions regress with/without antifungal ttt

Benign migratory glossitis

as Geographic tongue Common benign condition Incidence is 1-3% More common in females

Benign migratory glossitis

etiologies suggested

 Hypersensitivity

Marks et al  Hormonal OCP, Waltimo et al  Heredity Multifactorial mode of transmission associated with environmental factors
Relationship between HLA complex and GT (DR5 , DRW6 raised)  Increased incidence of GT & FT in patients with psoriasis  Psoriasis also shows increase in HLA antigen  GT & Psoriasis share same HLA CW6T


(Redman, Fenarali, Pindborg et al)

Benign migratory glossitis

seen in anterior two thirds of tongue  Appear as multiple, demarcated zones of erythema on the tip and lateral borders of the tongue  Erythema is due to atrophy of filiform papillae  Atrophic areas typically surrounded by slightly elevated, yellowish white, serpentine or scalloped borders  Lesion persists for short time in one area  New lesion develops in other sites of the tongue

Geographic tongue

Benign migratory glossitis

lesions similar to GT may occur in other sites of oral cavity called Ectopic GT Associated with GT Usually asymptomatic Occasionally patient may experience burning sensation

Benign migratory glossitis

planus  Cicatricial pemphigoid  Pernicious anemia  Leukoplakia

Benign migratory glossitis

blood examination and vit B12 assay to rule out pernicious anemia and IDA  Saliva for candida culture  Immunofluorescence to rule out pemphigoid

Benign migratory glossitis

of filiform papillae  Margin of the lesion shows hyperparakeratosis, spongiosis, acanthosis, elongation of rete ridges  Center of the lesion shows desquamated Para keratin  Migration of PMN and lymphocytes into the epithelium producing degeneration of epithelial cells and micro abscesses.  As this features are reminiscent of psoriasis, it is called psoriasiform mucositis.  Inflammatory cells in underlying CT chiefly neutrophils, lymphocytes and plasma cells

Benign migratory glossitis

& Prognosis:

 Generally

no ttt  Reassurance of patient is sufficient  Patients with intolerable burning sensation, topical corticosteroids may be given  Zinc supplementation may be effective

Hairy tongue
Marked

accumulation of keratin on the filiform papillae resulting in hair like appearance Represents increased keratin production or decrease in normal keratin desquamation Incidence is 0.5% in adults

Hairy tongue
 Etiology:
 Uncertain  Affected

people are mostly heavy smokers  Other possible factors include,

Hairy tongue
 C/F:
 Mostly

affects midline just anterior to CVP, sparing the lateral and anterior borders  Elongated papillae brown, black or yellow depending on the pigment producing bacteria, staining from tobacco and food  Sometimes most dorsum affected matted appearance  Asymptomatic occasionally pt complains of gagging sensation and bad taste

Hairy tongue

Hairy tongue
 H/P:
 Marked

elongation and hyperparakeratosis of the filiform

papillae  Numerous bacteria seen growing on the epithelial surface

& Prognosis:

 Benign

condition hence no serious sequelae  Major concerns are esthetic appearance and bad breath  Predisposing factors should be eliminated  Excellent oral hygiene should be maintained  Desquamation promoted by periodic scraping or brushing

H/P of hairy tongue

Lingual varices
 Abnormally

dilated or tortuous veins  Common in old adults and rare in children  Etiology:
 Age

related degeneration loss of CT zone supporting the blood vessels  Not associated with systemic hypertension or cardiac diseases  Sometimes, patients with varicose veins of legs are likely to have varicosities of the tongue

Lingual varices
 C/F:
 Sublingual

varices is most common  Multiple, bluish-purple, elevated, papular blebs  Ventral and lateral borders of the tongue  Usually asymptomatic except when secondary thrombosis occurs  Less frequently solitary varices occur in other parts of the mouth like lips and buccal mucosa  Usually noticed after becoming thrombosed.  Thrombosed varix present as firm, non-tender, bluishpurple nodule

Lingual varices

Lingual varices
 H/P:
 Reveals

a dilated vein  Walls show little smooth muscle and poorly developed elastic tissue  If secondary thrombosis occurs, lumen show Lines of Zahn  Organization and recanalization  Older thrombi exhibit dystrophic calcification Phlebolith
 Ttt

& Prog:

 No

ttt required for asymptomatic sublingual varices  Solitary varices can be removed surgically to confirm the diagnosis and for esthetic reasons.

Lingual thyroid
 Condition

in which follicles of thyroid tissue are found in the substance of the tongue  90% between F.caecum and epiglottis  Arising from thyroid analage that failed to migrate to its predestined position or from analage remnants that become detached and left behind  Etiology:
 Enlargement

due to functional insufficiency of the chief

thyroid gland  Patients residing in a goitrous area

Lingual thyroid
 C/F:
 Asymptomatic

remnants on posterior dorsal tongue in

about 10%  Symptomatic thyroid - more common in females  Found anywhere between CVP to epiglottis  Symptoms often develop during puberty, pregnancy and menopause  In 70% patients, these ectopic glands is the only functional thyroid gland  Hypo - thy occur in 33% of patients

Lingual thyroid

Lingual thyroid
 Small

nodules to large masses  Most clinical symptoms are,

and pain  Fullness of throat

usually vascular and smooth  3% - thyroid carcinoma

Lingual thyroid
 Diagnosis:

by thyroid scan using I131 and Tc99M  CT and MRI helpful in delineating the lesion  Due to brisk bleeding, biopsy is better avoided
 Best

 Investigations:
 Hormone

level estimation (T3,T4, TSH)  Ultrasonography  I131 uptake study  Scintigraphy

Lingual thyroid
Ttt

& Prog:

 Asymptomatic

follow up without ttt  Symptomatic supplemental thyroid hormones  No response surgery  As malignant transformation more common in men, some advocate prophylactic excision in men older than 30 yrs

Developmental disturbances of gingiva

Developmental disturbances of gingiva

includes

Fibromatosis

gingivae Retrocuspid papilla

Fibromatosis gingivae
Diffuse

fibrous overgrowth of the gingival

tissue Mostly hereditary AD Familial also occur with Hypertrichosis, epilepsy, mental retardation, sensori-neural deafness, hyperthyroidism and growth hormone deficiency

Fibromatosis gingivae
C/F:
 Manifested

as dense, diffuse, smooth or nodular

overgrowth  In older patients, surface has numerous papillary projections  Occur in one or both dental arches but Most common in maxilla  Appear at the time of eruption of permanent incisors  Mostly before the age of 20

Fibromatosis gingivae

Fibromatosis gingivae
C/F:
 Gingiva

not inflamed  Firm, normal color  Prevent normal eruption of teeth  Not painful and no tendency for hemorrhage  Localized cases involve a group of teeth  May remain stable or may spread to other segments

Fibromatosis gingivae
 H/P:
 Dense

hypocellular and hypovascular collagenous tissue  Appear to run in all directions as interlacing bundles  Epithelial thickening with elongated rete ridges  Mild inflammatory infiltrate  Sometimes dystrophic calcifications seen  EM reveals mixture of fibroblasts and myofibroblast like cells

Fibromatosis gingivae
Ttt

and Prognosis:

Gingivectomy

and oral hygiene measures Follow up required because of its tendency to recur Severe cases selective extraction and gingivectomy

Retrocuspid papilla
 Described

by Hirshfeld in 1933  Small elevated nodule located on the lingual mucosa of the mandibular cuspids  C/F
 Soft,

well circumscribed mucosal nodule  Usually bilateral  Located lingual to mandibular cuspid between free gingival margin and mucogingival junction  Most common in children between 8 16 yrs  Show regression with maturity  More common in females than males

Retrocuspid papilla

Retrocuspid papilla
Histopathology
 Mild

hyperorthokeratosis or hyperparakeratosis,with or without acanthosis  Connective tissues sometimes highly vascularised and show large stellate fibroblasts  Occasionally epithelial rests also seen
Tmt

and prognosis
with age no treatment necessary

 Regress