MACROGLOSSIA

Definition (1 of the following)

1.  extravasation of the lingual apex or lingual border onto or outside the dentition

2.  the impression of one or more teeth on the lingual border visualized when the mouth is open

3.  following surgery for correction, a relapse of increased interdental space, open bite deformity, and/or jaw deformation with malocclusion occurs.

Macroglossia causes :

1.  airway obstruction - usually worsened by lying supine

2.  speech impairment

3.  swallowing difficulties

4.  exposure and drying of the tongue with resultant glossitis and bleeding is common

5.  open bite deformity

6.  mandibular prognathism

7.  dental malalignment – teeth pushed forwards (proclined)

Classification

1.  Pseudomacroglossia

a.  Small oral cavity

i.  Low palate and decreased oral cavity volume displacing tongue

ii.  Transverse, vertical, or anterior/posterior deficiency in the maxillary or mandibular arches displacing the tongue

iii.  severe mandibular deficiency (retrognathism)

b.  Displacement

i.  Enlarged tonsils and/or adenoids displacing tongue

ii.  Neoplasms displacing the tongue

iii.  Habitual posturing of the tongue

iv.  Hypotonia of the tongue

2.  Congenital

a.  Lymphatic malformation

b.  Downs syndrome

c.  Beckwidth-Wiedemann syndrome

d.  Mucopolysaccharidoses

3.  Acquired

a.  Metabolic/endocrine

i.  Hypothyroidism

ii.  Diabetes

b.  Infection

i.  Syphilis

ii.  Tuberculosis

c.  Neoplastic

d.  Infiltrative

i.  Amyloidosis

ii.  Sarcoidosis

Anatomy

Blood supply

·  main artery - the lingual branch of the external carotid.

·  contributing arteries

1.  the tonsillar branch of the facial artery

2.  ascending palatine branch of the ascending pharyngeal artery.

·  An extensive submucosal plexus is responsible for the vigorous bleeding with even superficial wounds.

Treatment

·  Airways compromise dictate the need for urgent intervention ie tracheostomy

·  Partial glossectomy best undertaken between 6-24 months of age – too late may compromise speech development

Partial Glossectomy

·  Aim to preserve tongue function, along with the reduction of tongue mass.

·  Peripheral excisions (A & D below) leave the tongue globular and immobile.

·  V-shaped wedge taken from the tip of the tongue(B), will shorten but not narrow it, whereas an ellipse taken from the midline of the tongue will narrow it but not shorten it (C)

·  Incision K preserves the tip but leads to a smaller reduction

·  Keyhole excision (L) may give the best results – taking the tip does not seem to cause reduction in sensation

·  Care should be taken to avoid the neurovascular bundles which run inferolaterally

·  Even if both lingual arteries are severed, the tongue will not normally necrose because of the rich collateral blood supply and anastomoses.

·  Postoperative edema is often quite substantial, and sutures should not be too small or too tight because the swelling may cause them to cut through

·  Taste and tongue mobility are rarely affected by tongue reduction.

·  Speech can be affected, but this complication usually corrects itself within a matter of weeks and formal speech therapy is rarely needed.

·  Speech improvement has been reported as a result of tongue reduction.

Other procedures

·  Orthognatic/Orthodontics usually required to correct prognatism, open bite (combined LeFort 1 and BSSO)

Tongue-reduction procedures. A, Butlin and Ensign; B, Harris, Blair and Hendrick; C, Edgerton; D, Dingman and Grabb and Gupta; E, Egyedi and Obwegeser; F, Kole and Davalbhakta and Lamberty; G and H, Austerman and Machtens; I, Kruchinsky; J, Mixter ; K, Harada and Enomoto; L, Morgan et al and Kacker et al.