Macroglossia, has been documented by strict cephalometric analysis, as a large tongue. Ueyama5 and others defined macroglossia as occurring when 1 of the following 3 criteria is met: (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. The 2 broadest categories under the heading of macroglossia are true enlargement and pseudomacroglossia.

A 4 yr old tribal boy presented to us with a massive swollen protruded tongue. Parents used a handkerchief to hide the swelling and prevent cosmetic embarrassment. This presented as a small swelling since the age of one year and progressed henceforth to the massive enlargement. Due to this massive protruded swelling patient was unable to close his mouth, unable to articulate properly, could eat with difficulty. His parents seeked medical treatment due to social embarrassment and development of complications like excoriations and ulcers. The patient did not show any signs of delayed milestones, endocrine or congenital anomalies. We decided to do a wedge resection of the tongue. The pathology report confirmed it as a lymphangioma (Fig. 2).

The congenital syndromes that express macroglossia in their phenotypes, most commonly are Down syndrome (1 per 700 live births) and Beckwith-Weidmann syndrome (0.07 per 1000 live births). In Beckwith-Weidmann syndrome,1 97.5% of patients have macroglossia. The literature documents only 2 families with autosomal dominant inheritance of isolated macroglossia. In 1963 Beckwith reported three cases of a new syndrome consisting of macroglossia, omphalocoele, visceromegaly, cytomegaly of adrenal cortex and hyperplasia of gonadal interstitial cells. In 1964 Weidman6,7 independently reported the syndrome. Beckwith-Wiedmann syndrome (BWS) is characterized by foetal gigantism, visceromegaly, macroglossia, microcephaly, nevus flammeus, diaphragmatic hernia, hemihypertrophy, pancreatic and adrenal hyperplasia and omphalocoele. Increased incidence of intra-abdominal tumours and hypoglycaemia are also described. Adrenal manifestations of BWS are adrenal cortical cytomegaly, adrenal cortical cysts, adrenal adenomas, carcinoma, neuroblastoma, adrenal calcifications and adrenal haemorrhages. Benign haemorrhagic adrenocortical macrocysts (BHAM) are a rare manifestation of BWS .8

In Sweden, a young female patient was treated by amputating the protruded portion of the tongue. Her recovery allowed for normal swallowing. Surgical resection did not gain favour widely until after 1900. Prior to that time, the use of various ligatures dominated the attempts at surgical reduction. Tying a wire around the tongue was particularly gruesome and painful with the necrotic tongue taking up to 2 weeks to slough off.

Fig. 1 : Massive swollen protruded tongue.

The most common ligature instrument was the écraseur (crusher in French) described in their article by Ring et al.4 An instrument that resembled a snare, the écraseur had, instead of a wire loop, one made of chain links like those found on a chain saw or bicycle. At the end of the snare handle was a screw that tightened the chain. This instrument was applied across the portion of the tongue that was to be removed and tightened one link every hour until the necrosed portion was removed. Sometimes, the écraseur was tightened at a rate of 1 notch every 2 minutes until the écraseur cut through the tongue rather than necrosing it. The complications reported were great, but those who survived did well in terms of swallowing and speech.

In 1900, Butlin and Spencer severely condemned all previous treatments and stated, “There is only one treatment—wedge shaped excision.” This remains the standard today, although it has been modified in a number of different ways since then.

Fig. 3 : The mass measuring 6 x 6 cm.

Several studies document the role of the tongue in shaping the oral cavity. Just as reduced pressure of the tongue on the palate and mandible may lead to an adenoid facies, increased pressure on the surrounding anatomy can have opposite effects. Upper incisors can be pushed horizontally inducing forward maxillary growth. Other morphologic changes include open bite deformities, prognathism, class III malocclusion, anterior and/or posterior crossbites, buccal tipping of posterior teeth, accentuated curve of Spee in the maxillary arch, reverse curve of Spee in the mandibular arch, increased transverse width of mandibular and/or maxillary arches. Furthermore, difficulty with mastication may lead to temporomandibular joint pain.

If the tongue protrudes beyond the lips and is exposed to the air, drying of the tongue with resultant glossitis and bleeding is common.

One form of medical therapy has shown promise. The Castillo-Morales orofacial therapy2 developed in the mid 1970s has been most successful in cases of pseudomacroglossia where the problem is hypotonicity. A manual stimulation and facilitation treatment is performed using a palatal prosthesis. The prosthesis or plate has a pair of electrical stimulators in it. When placed in the mouth a reflexive action of the tongue to seek this foreign body occurs, moving the tongue backward and upward to meet it. When the tongue meets the plate, it activates the lingual electrode that further stimulates the tongue into the backward and upward position, rather than the hypotonic position of downward and forward.

The keyhole method of resection has been the most popular resection type over the last 50 years. It reduces not only the anterior-posterior dimension of the tongue but also its width, yet the classic description of this procedure involves the resection of the tip of the tongue and a T-line closure. Although this allows a greater resection of the anterior extent of the tongue, it also prevents the use of the important tip musculature for articulation and other fine motor movements. Mixter et al3 also reported that this method of reduction could lead to an ankylosed globular tongue with an insensitive tip. He advocated central debulking of the tongue using a W-shaped incision in the middle two-thirds. The greatest threat in this type of resection is more significant and prolonged swelling.