Linea Alba

Developmental Defects and Variations

Dr. John Koutlas
Division of Oral Pathology

Buccal mucosa, less common tongue

Pressure, friction, sucking trauma
Restricted to dentulous areas
More prominent on posterior mucosa
HistDx: Hyperkeratosis

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Pits (Fistulas)
Commissural lip pits
12-20% of adults; 0.2-0.7% in children
Males > Females
Unilateral or bilateral
Accentuated with age? or not developmental?
Failure of processes to fuse
Blind fistulas; sometimes saliva
Infection can occur
Associated with hearing loss, preauricular pits, rib
anomalies
Combination with paramedian (one case reported)

Pits (Fistulas)
Paramedian lip pits
Blind ends
Presence of salivary glands
van der Woude syndrome (AD)
Interferon regulatory factor 6 gene mutations (role in fusion of lip
and palate); chr 1 long arm
Pits and cleft lip and/or cleft palate
Mental retardation; dental malformations (hypodontia)

Popliteal pterigium syndrome

Same gene
Popliteal webs
Cleft lip and/or cleft palate
Syngnathia (webs connecting upper & lower jaw)

Double Lip
Redundant fold
Congenital (persistence of the pars glabrosa and
pars villosa) and acquired (trauma, habits)
Ascher (Laffer-Ascher) syndrome
Double lip
Blepharochalasis (Fuchs 1896)
Edema of the upper eyelid; vision interference
Hyperplasia of lacrimal glands with prolapse of orbital fat

Nontoxic thyroid enlargement (50%)

DiffDx: angioedema, tumor

Fordyce Granules

Sebaceous glands
Ectopic or normal anatomic variation
Buccal mucosa, lips, tonsillar pillar
Adults > Children (puberty stimulates development)
Glands with ducts; no hair
Hyperplasia, cystic transformation, tumors (extremely
rare)

Duct

Sebaceous glands

Sebaceous gland

Interesting note
Recent evidence indicates an increase in
size and number of sebaceous glands
following activation of the hedgehog
pathway, a crucial signaling pathway for
animal development that is aberrantly
activated in several types of cancer.
Unrecognized activation of the sebaceous
glands system occurs in HNPCC

Leukoedema
Diffuse grayish-white appearance of mucosa
Blacks > Whites (racial pigmentation may make this
variation more prominent)
Variation, not a disease
More prominent in smokers, poor oral health
Buccal mucosa extending to the lips
Vagina, larynx
Hyperplastic epithelium; intracellular edema
No treatment
DiffDx: Lichen planus, other leukoplakic lesions,
dentifrice stomatitis (chemical burn)

Microglossia
Oromandibular-limb hypogenesis syndromes
Hypodactyly
Hypomelia
Underdeveloped organs
Some potential etiologic factors
Lithium during pregnancy
Chorionic villi sampling procedures

Macroglossia
Congenital and hereditary
Vascular malformations
Hemihyperplasia
Cretinism
Beckwith-Wiedemann syndrome
Omphalocele, visceromegaly, gigantism, hypoglycemia,
Wilms tumor, adrenal or liver tumors (hepatoblastoma)

Down syndrome
NF 1
MEN 2B

Macroglossia
Acquired
Edentulous patients
Amyloidosis
Myxedema
Acromegaly
Angiedema
Tumors

Ankyloglossia
NEVER FORGET THAT THE TONGUE IS SHORT
AT BIRTH
Far more common in boys than girls
Complete ankyloglossia extremely rare
Relation to open bite ?
Relation to periodontal disease
Dyspnea (epiglottis comes forward)
Breast-feeding
Speech articulation
Mechanical tasks such as licking the lips and kissing

Lingual Thyroid
Birth of the thyroid & foramen cecum
Small remnants of thyroid can be found in the tongue
~75% of pts with infantile hypothyroidism have
lingual thyroid
33% have hypothyroidism
Females >> males
Nodule of varying size
Dysphonia, dysphagia
Large nodules can cause sleep apnea

May be the only functioning thyroid tissue

Goiter, Graves disease have been described

Rare examples of thyroid carcinoma (MALES)

Lingual thyroid in a cat

Fissured Tongue
Grooves and fissures
More prevalent in whites, blacks; less in Mexican
Americas
Entire tongue or part
Usually asymptomatic
Prevalence increases with age
Strong association with geographic tongue and
Melkersson-Rosenthal syndrome
Tongue brushing

Melkersson-Rosenthal syndrome
1. Fissured tongue
2. Cheilitis granulomatosa
3. Facial paralysis

Hairy Tongue

DO NOT CONFUSE IT WITH HAIRY LEUKOPLAKIA

Marked hyperkeratinization of filiform papillae
Less than 1%
Causes

Smoking, antibiotics, POH, radiation, debilitated pts

Staining (bacteria, coffee, tobacco)
Oxidizing mouthwashes or antacids
Overgrowth of fungal or bacterial organisms
Medications: olanzapine, fluoxetine hydrochloride, thiothixene
hydrochloride, benztropine mesylate, and clonazepam (antipsychotic
meds)

Asymptomatic
Treatment
Brushing
Shaving
DO NOT USE KERATOLYTIC AGENTS (If you do not know how)

Bizarrities
Unilateral hairlike discoloration of the
tongue was described in a patient with
ipsilateral mandibular division trigeminal
neuralgia. This unusual physical sign
coincided with the patient's painful trigger
zone and was attributed to hypertrophy of
keratinized filiform papillae, where guarded
avoidance of mechanical stimulation over
time prevented normal desquamation.

Varicosities

Dilated and tortuous vein

Age-related (60% of older individuals)
Loss of connective tissue tone
No association with hypertension or CPD
Sublingual area
Multiple or solitary (lips, buccal mucosa)
Phleboliths

Caliber-Persistent Artery

Uncommon
Lower and less often upper lip
Bilateral
Pulsating (you feel it with bare fingers)
Age-related
Associated with ulceration; DiffDx: SCCa
No tx necessary
Arterial bleeding during surgery

Lateral Soft Palate Fistulas

Congenital or the result of trauma or surgey

Bilateral or unilateral
Shallow or deep perforations
Can be associated with anomalies such
absence or hypoplasia of tonsils, hearing
loss, preauricular defects

Vazquez et al. J Ultrasound Med 2005

Coronoid Hyperplasia

Rare
5M:F
Unilateral or bilateral (5x)
Restricted mouth opening
Deviation towards the affected site
Jacobs disease (pseudojoints with the zygomatic
arches; association with osteochondroma of the
coronoid process)
CT better than panoramic
Tx is problematic
Fibrosis
Coronoid regrowth

Condylar hyperplasia

Akan & Mehreliyeva

Dentomaxillofac Radiol. 2006 Jan;35(1):55-9

Uncommon; more common than coronoid

Trauma, endocrine or circulatory cause
Part or not of hemifacial hyperplasia
Facial asymmetry, prognathism, open bite
Occasional compensatory maxillary growth
and tilting of the occlusal plane
Condylectomy

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Condylar hypoplasia
Congenital or acquired
Many syndromes
Treacher Collins
Oculoauricularvertebral
Hemifacial microsomia

During development
Trauma, radiation

Degenerative arthritis

Bifid Condyle
Double head (lateral and medial; less often
anterior-posterior)
Unilateral (less often bilateral)
Can be asymptomatic

Exostoses

Bony protuberance
Palatinus and Mandibularis
Buccal exostoses: facial
Palatal exostoses: lingual aspect of tuberosity
Solitary exostoses: trauma
Subpontic osseous hyperplasia
Mass of non-neoplastic bone with minimal marrow
Can be ulcerated and painful

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Torus Palatinus

Midline of palatal vault

Multifactorial, genetic predisposition
Flat, spindle, nodular, lobular
Rarely show on PAs
More women than men
Surgical excision to accommodate prosthesis

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Torus Mandibularis

Lingual aspect of mandible

Multifactorial
Bilateral
Single or multiple nodules
PAs: Periapical opacities that can be confusing
Not as common as torus palatinus
Surgical excision to accommodate prosthesis

Eagle syndrome
Stylohyoid syndrome
Elongation of the styloid process or
mineralization of the stylohyoid ligament
Bilateral and less often unilateral
Most asymptomatic
After tonsillectomy
Clinical syndrome
Vague facial pain (swallowing, turning head,
opening mouth)
Headache, dizziness, otalgia, transient syncope

Eagle syndrome
Treatment choices
No treatment
Cotricosteroid injection
Surgical excision of the elongated process or
mineralized ligament

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Stafne Defect (Static Bone Cyst)

Radiolucency with sclerotic border
Near angle of mandible (below the canal)
Anterior and upper ramus rarely

Usually normal salivary glands

Submandibular, sublingual, parotid

Asymptomatic
Striking male predilection
Developmental but not present from birth
Sialogram

Queiroz et al, 2004 JOMFS

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Geographic Tongue

Migratory glossitis (stomatitis)

1-3% of population; females twice more often
Wandering patients
? Hypersensitivity reaction; ? hormonal role
Usually asymptomatic
? Association with psoriasis (HLA-Cw6)
Patient reassurance; topical steroid; zinc

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