Pathophysiology: Aglossia

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Pathophysiology

Developmental and structural abnormalities of the tongue are common


characteristics. The different morphological variations that might happen
throughout the development of tongue are [5]: aglossia, microglossia,
macroglossia, ankyloglossia, cleft tongue, pentafid tongue, fissured tongue,
geographic tongue, hairy tongue, median rhomboid glossitis.

 Aglossia: congenital absence of the tongue is very rare. Usually, the tongue
is absent in cases of gross underdevelopment or maldevelopment of the 1 st
visceral arches.

 Microglossia: it’s rare developmental condition and is also called


hypoglossia. Its defining feature may be a rudimentary or abnormally small
tongue. It results in restricted muscular movement and is related to
syndromes like Hanhart syndrome.

 Macroglossia: Macroglossia is an infrequently encountered condition


characterized by tongue enlargement, seen in association with other
congenital defects leading to syndromes such as down syndrome (trisomy
21), Beckwith-Wiedemann syndrome.

 Ankyloglossia: It occurs due to failure in cellular degeneration leading to a


longer anchorage between the tongue and floor of the mouth; this is
commonly known as "tongue-tied" and demonstrates an abnormally short
lingual frenulum. Ankyloglossia can range in severity from mild to complete
ankyloglossia in which the tongue gets fused to the floor of the mouth
restricting its free movement.[6] A short lingual frenulum leading to tongue-
tie is also associated with several genetic syndromes such as related
Robinow syndrome, oral-facial-digital syndrome Type I, Opitz syndrome,
and Van der Woude syndrome.

 Cleft tongue: It is also known as bifid tongue and occurs when the lateral
swellings fail to merge. It can be partial or complete. The former is a more
common entity and is manifested as a deep groove on the dorsal surface of
the tongue in the midline. It occurs when the mesenchymal proliferation
interferes with the merging leading to failure of the obliteration of the
groove. There are reports of bifid tongue in syndromic cases like Opitz G
BBB syndrome, oral-facial-digital syndrome type I, Klippel–Feil anomaly,
and Larsen syndrome.[7]

 Pentafid tongue: Disturbance in the mesodermal penetration and


mesenchymal fusion during the development of tongue development is
responsible for this malformation.

 Fissured tongue: It is also known as scrotal tongue or lingua fissurata. It is


congenital anomaly manifested as grooves oriented anteroposteriorly on the
dorsal aspect of the tongue with multiple branches extending towards the
lateral aspect. The grooves range from 2 to 6 mm in depth. In a severe form
of the fissured tongue, when the grooves are extremely prominent and
interconnected, the tongue may appear to be lobulated. It can also present in
association with down syndrome or Melkerson-Rosenthal syndrome (a triad
of fissure, granulomatous cheilitis, and cranial nerve VII paralysis).

 Geographic tongue: Geographic tongue, also known as lingua geographica


or benign migratory glossitis is an inflammatory disorder caused by loss of
filiform papillae.

 Hairy tongue: It is also known as a black hairy tongue and characteristically


demonstrates the accumulation of excess keratin on the filiform papillae on
the dorsal surface of the tongue leading to the formation of elongated strands
resembling hair. This condition most commonly affects the midline just
anterior to the circumvallate papillae, usually sparing the lateral and anterior
borders.

 Median rhomboid glossitis: It is a condition that presents in the midline of


the dorsal surface of the tongue, just in front of the circumvallate papillae. It
presents as a well-demarcated, symmetric, depapillated area. However, it
also occasionally appears in the paramedial location.

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