DEPARTMENT OF ORAL PATHOLOGY

2. DEVELOPMENTAL
DISTURBANCES OF
TONGUE
DR. SUHANA . H.S
DEPARTMENT OF ORAL PATHOLOGY
AND MICROBIOLOGY

3. AGLOSSIA AND MICROGLOSSIA SYNDROME
• This malformation is very rare, and is almost always
associated to malformations in the extremities, especially
the hands and feet, cleft palate and dental agenesia.
• Aglosia syndrome is, in reality, a microglossia with
extreme glossoptosis.
• What is commonly observed is a rudimentary, small
tongue.
• As a consequence of the lack of muscular stimulus
between the alveolar arches, these do not develop
transversely and the mandible does not grow in an
anterior direction, producing as a result a severe

4. • This syndrome shows no
predilection for gender and has
no genetic implications. Its
etiology must be searched for in
some sort of fetal cell traumatism
in the first few weeks of
gestation.
• Neither language nor swallowing
are sensibly affected by this
condition.

5. MACROGLOSSIA
(TONGUE HYPERTROPHY, PROLAPSUS OF THE TONGUE,
ENLARGED TONGUE, PSEUDOMACROGLOSSIA)
• Macroglossia, meaning large tongue, has been a documented anatomical
anomaly for several centuries.
• Although the exact incidence of macroglossia is unknown , some congenital
syndromes often express macroglossia in their phenotypes, most commonly
down syndrome and beckwith-wiedemann syndrome . In beckwith-
wiedemann syndrome, 97.5% of patients have macroglossia.
• The two broadest categories under the heading of macroglossia are true
macroglossia and pseudomacroglossia.

6. • Physical examination of the oral cavity
and head morphology is helpful to
deduce true macroglossia from
pseudomacroglossia.
• Severe retrognathia and unusually
small maxillary and/or mandibular size
may indicate the latter.
• In addition, check tongue tone and
mobility to rule out simple atonia or
hypotonia indicating poor posturing of
the tongue— as is commonly
observed in down syndrome

7. TREATMENT
• The goal of nearly all surgery is to return the patient to an anatomically
and physiologically normal condition; and this applies to macroglossia as
well. The goal is to reduce tongue size and thereby improve function.
Those main functions include articulation, mastication, deglutition,
protection of the airway, and gustation. Of these, only gustation is not
often improved with surgical intervention.

8. ANKYLOGLOSSIA OR TONGUE-TIE
• Ankyloglossia, or tongue-tie as it is more commonly known, is said to exist when
the inferior frenulum attaches to the bottom of the tongue and subsequently
restricts free movement of the tongue.
• At one time, such restriction was believed to cause speech problems and it was
routine to clip the membranous frenulum (frenulectomy) to free the tongue tip.
• Ankyloglossia occurs in approximately 1.7% of all neonates without preference for
either gender and is reported to be transitory.

9. • With growth, the frenulum lengthens so normal tongue
function is established.
• The criterion for diagnosis is based upon observation of
lingual mobility; no current specific indications for surgery
are emphasized in either the dental or medical literature
reviewed
• Simple incision of the frenulum may result in the
development of scar tissue and further restriction of tongue
movement
• Preventing speech defects or improving a child’s
articulation may be another reason to consider surgical
intervention
• Treatment. Frenulectomy is recommended.

10. CLEFT TONGUE
• A completely cleft or bifid tongue is a rare condition that is
apparently due to lack of merging of the lateral lingual
swellings of this organ.
• A partially cleft tongue is considerably more common and is
manifested simply as a deep groove in the midline of the
dorsal surface .
• The partial cleft results because of incomplete merging and
failure of groove obliteration by underlying mesenchymal
proliferation.
• It is of little clinical significance except that food debris and
microorganisms may collect in the base of the cleft and cause
irritation.

12. FISSURED TONGUE (SCROTAL TONGUE,
LINGUA PLICATA)
• Fissured tongue is a condition
frequently seen in the general
population and it is characterized by
grooves that vary in depth and are
noted along the dorsal and lateral
aspects of the tongue .
• Although a definitive etiology is
unknown, a polygenic mode of
inheritance is suspected because
the condition is seen clustering in
families who are affected.

13. • Patients are usually asymptomatic,
and the condition is initially noted
on routine intraoral examination as
an incidental finding.
• Fissured tongue is also seen in
melkersson-rosenthal syndrome
and down syndrome and in
frequent association with benign
migratory glossitis (geographic
tongue).

14. • Melkersson-rosenthal syndrome is a rare condition consisting of a triad of
persistent or recurring lip or facial swelling, intermittent seventh (facial)
nerve paralysis (bell’s palsy), and a fissured tongue. The etiology of this
condition is also unknown
• Fissured tongue is a totally benign condition and is considered by most to
be a variant of normal tongue architecture. No predilection for any particular
race appears to exist. Some reports have shown a slight male predilection.
• Although fissured tongue may be diagnosed initially during childhood, it is
diagnosed more frequently in adulthood. The prominence of the condition
appears to increase with increasing age.

15. • The lesions are usually asymptomatic unless debris is entrapped within
the fissure or when it occurs in association with geographic tongue (a
common finding).
• On clinical examination, fissured tongue affects the dorsum and often
extends to the lateral borders of the tongue.
• The depth of the fissures varies but has been noted to be up to 6 mm in
diameter. When particularly prominent, the fissures or grooves may be
interconnected, separating the tongue dorsum into what may appear to be
several lobules.
• Although a specific etiology has not been elicited, a polygenic or
autosomal dominant mode of inheritance is suspected because this
condition is seen with increased frequency in families with an affected

16. • A biopsy is rarely performed on a fissured tongue because of
its characteristic diagnostic clinical appearance; however,
histologic examination has shown an increase in the
thickness of the lamina propria, loss of filiform papillae of the
surface mucosa, hyperplasia of the rete pegs, neutrophilic
microabscesses within the epithelium, and a mixed
inflammatory infiltrate in the lamina propria.
• Treatment: No definitive therapy or medication is required.

17. MEDIAN RHOMBOID GLOSSITIS
• Embryologically the tongue is formed by two lateral
processes (lingual tubercles) meeting in the midline and
fusing above a central structure from the first and
second branchial arches, the tuberculum impar.
• The posterior dorsal point of fusion is occasionally
defective, leaving a rhomboid-shaped, smooth
erythematous mucosa lacking in papillae or taste buds.
• This median rhomboid glossitis is a focal area of
susceptibility to recurring or chronic atrophic
candidiasis, prompting a recent shift towards the use of
posterior midline atrophic candidiasis as a more

18. • The erythematous clinical appearance; moreover, is due primarily to the
absence of filiform papillae, rather than to local inflammatory changes, as
first suggested in 1914 by brocq and pautrier
• Median rhomboid glossitis presents in the posterior midline of the dorsum of
the tongue, just anterior to the v-shaped grouping of the circumvallate
papillae.
• Most cases are not diagnosed until the middle age of the affected patient,
but the entity is, of course, present in childhood.
• There appears to be a 3 : 1 male predilection.
• Those lesions with atrophic candidiasis are usually more erythematous but
some respond with excess keratin production, and therefore, show a white
surface change.

19. • Lesions are typically less than 2 cm in
greatest dimension and most
demonstrate a smooth, flat surface,
although it is not unusual for the surface
to be lobulated.
• Occasional lesions are located
somewhat anterior to the usual location.
None have been reported posterior to
the circumvallate papillae.

20. • Median rhomboid glossitis shows a smooth or nodular surface covered by
atrophic stratified squamous epithelium overlying a moderately fibrosed
stromawith somewhat dilated capillaries.
• Fungiform and filiform papillae are not seen, although surface nodules may
mimic or perhaps represent anlage of these structures. A mild to moderately
intense chronic inflammatory cell infiltrate may be seen within subepithelial
and deeper fibrovascular tissues.

21. • Chronic candida infection may result in excess
surface keratin or extreme elongation of rete
processes and premature keratin production
with individual cells or as epithelial pearls
(dyskeratosis) deep in the processes.
• Silver staining for fungus will often reveal
candida hyphae and spores in the superficial
layers of the epithelium. This pseudo epithelio
matous hyperplasia may be quite pronounced.
• No treatment is necessary for median
rhomboid glossitis, but nodular cases are
often removed for microscopic evaluation.

22. • Recurrence after removal is not expected, although those cases with
pseudoepitheliomatous hyperplasia should be followed closely for at least a
year after biopsy to be certain of the benign diagnosis.
• Antifungal therapy (topical troches or systemic medication) will reduce
clinical erythema and inflammation due to candida infection.

23. BENIGN MIGRATORY GLOSSITIS
(GEOGRAPHIC TONGUE)
• Benign migratory glossitis
is a psoriasiform mucositis
of the dorsum of the
tongue. Its dominant
characteristics is a
constantly changing
pattern of serpiginous
white lines surrounding
areas of smooth,
depapillated mucosa.

24. • The changing appearance has led some to call this the wandering rash
of the tongue, with the depapillated areas have reminded others of
continental outlines on a globe, hence the use of the popular term
geographic tongue
• The etiology of benign migratory glossitis is unknown, but it does seem
to become more prominent during conditions of psychological stress and
it is found with increased frequency (10%) in persons with psoriasis of
the skin.

25. • Approximately 1–2% of the population are
affected, although most cases are so mild that
they are never formally diagnosed.
• All of the microscopic features of psoriasis are
present in benign migratory glossitis and
migratory stomatitis, but these will not be
obvious unless the biopsy is taken from a
prominent serpiginous line at the periphery of a
depapillated patch.
• A thickened layer of keratin is infiltrated with
neutrophils, as are lower portions of the
epithelium to a lesser extent.

26. • These inflammatory cells often
produce small microabscesses,
called monro’s abscesses, in
the keratin and spinous layers.
• Rete ridges are typically thin
and considerably elongated,
with only a thin layer of
epithelium overlying connective
tissue papillae. When rete
ridges are not elongated, the
pathologist should consider
reiter’s syndrome as a
diagnostic possibility.

27. • Chronic inflammatory cells can be seen in variable numbers within the stroma
and silver or pas staining will often demonstrate candida hyphae or spores in the
superficial layers of the epithelium.
• There is no liquefactive degeneration of basal cells, as seen in lichenoid lesions,
and there is no ulceration except in cases of reiter’s syndrome.
• No treatment is usually necessary for benign migratory glossitis and stomatitis.
Symptomatic lesions can be treated with topical prednisolone and a topical or
systemic antifungal medication can be tried if a secondary candidiasis is
suspected.

28. HAIRY TONGUE (LINGUA NIGRA, LINGUA
VILLOSA, LINGUA VILLOSA NIGRA, BLACK
HAIRY TONGUE)
• Hairy tongue (lingua villosa) is a commonly observed
condition of defective desquamation of the filiform
papillae that results from a variety of precipitating
factors.
• The condition is most frequently referred to as black
hairy tongue (lingua villosa nigra); however, hairy
tongue may also appear brown, white, green, pink, or
any of a variety of hues depending on the specific
etiology and secondary factors (e.G. Use of colored
mouth washes, breath mints, candies).

29. • ETIOLOGY: The basic defect in hairy tongue is the hypertrophy of filiform
papillae on the dorsal surface of the tongue, usually due to a lack of
mechanical stimulation and debridement. This condition often occurs in
individuals with poor oral hygiene
• Contributory factors for hairy tongue are numerous and include tobacco
use and coffee or tea drinking. These factors account for the various
colors associated with the condition.

30. • CLINICAL FEATURES. Normal filiform papillae are approximately 1 mm in
length, whereas filiform papillae in hairy tongue are more than 15 mm in
length.
• Hairy tongue has been reported with greater frequency in males,
patients infected with human immunodeficiency virus (HIV), and those
who are HIV negative and use intravenous drugs.
• Hairy tongue is rarely symptomatic, although overgrowth of candida
albicans may result in glossopyrosis (burning tongue).

31. • Patients frequently complain of a tickling sensation in the soft palate and
the oropharynx during swallowing. In more severe cases, patients may
actually complain of a gagging sensation.
• Retention of oral debris between the elongated papillae may result in
halitosis.
• No racial predilection is associated with hairy tongue.
• Bacterial and fungal overgrowth play a role in the color of the tongue. The
only complication associated with hairy tongue is an occasional candidal
overgrowth, which often results in an uncomfortable glossopyrosis
(burning tongue). Altered taste sensation is a rare complication.

32. • DIFFERENTIAL
DIAGNOSIS. This
condition has to be
differentiated from
candidiasis, leukoplakia,
oral lichen planus and oral
hairy leukoplakia.
• HISTOPATHOLOGIC
FINDINGS In hairy tongue
consist of elongated
filiform papillae, with mild
hyperkeratosis and
occasional inflammatory

33. • The treatment of hairy tongue is variable. In many cases, brushing of
the tongue with a toothbrush or using a commercially available tongue
scraper is sufficient to remove elongated filiform papillae and retard
the growth of additional ones. Surgical removal of the papillae by
using electrodesiccation, carbon dioxide laser, or even scissors is the
treatment of last resort when less complicated therapies prove
ineffective. The prognosis for hairy tongue is excellent.

34. LINGUAL VARICES (LINGUAL OR SUBLINGUAL
VARICOSITIES)
• A varix is a dilated, tortuous vein, most
commonly a vein which is subjected to
increased hydrostatic pressure but poorly
supported by surrounding tissue.
• Varices involving the lingual ranine veins
are relatively common, appearing as red or
purple shotlike clusters of vessels on the
ventral surface and lateral borders of the
tongue as well as in the floor of the mouth.
• However, varices also do occur in other
oral sites such as the upper and lower lip,
buccal mucosa, and buccal commissure.