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Editorials

Macroglossia

BMJ 1994; 309 doi: https://doi.org/10.1136/bmj.309.6966.1386 (Published 26 November 1994) Cite this as: BMJ 1994;309:1386
  1. P Murthy,
  2. M R Laing

    Management depends on the cause; many patients will need long term psychological support

    Macroglossia is traditionally defined as a resting tongue that protrudes beyond the teeth or alveolar ridge. The diagnosis is usually based on this sign and comparison with an apparently normal tongue (objective measurements of size are unreliable). The term should be reserved for cases of long term painless enlargement of the tongue and is distinct from rapid growth of the tongue due to acute parenchymatous glossitis.1

    The condition may be classified as true macroglossia, which is associated with definitive histopathological findings, or pseudomacroglossia, which is a relative enlargement of the tongue secondary to a small mandible and with no demonstrable histological abnormalities. True macroglossia may be primary—characterised by hypertrophy or hyperplasia of the tongue muscles—or secondary—the result of infiltration of normal tissue with anomalous elements.2

    Given its varied causes, both physicians and surgeons may see macroglossia. Common causes of true macroglossia in children include hypothyroidism, lymphangioma, haemangioma, idiopathic hyperplasia, metabolic disorders, and chromosomal abnormalities including the Beckwith-Wiedemann syndrome.2 Amyloidosis is the commonest cause of macroglossia in adults, the tongue being involved in both its primary and secondary forms.3 Secondary macroglossia may also be a manifestation of both benign and malignant space occupying lesions, such as cystic hygroma, cysts in the lingual thyroglossal duct, dermoid cysts, neurofibromatosis, granular cell tumours, and rhabdomyosarcoma.2 Other important causes include acromegaly, angio-oedema, lymphoma, and chronic inflammatory conditions such as syphilis and tuberculosis.2 The tongue may be displaced upwards and forwards by lesions in the floor of the mouth including tumours, Ludwig's angina (an infection in the sublingual and submandibular space, usually with a dental cause), ranula (a mucus retention cyst considered to arise from the sublingual salivary gland), and angioneurotic oedema.

    Pseudomacroglossia is usually self limiting and regresses with age. Apparent enlargement of the tongue secondary to micrognathia is seen in Down's syndrome and the PierreRobin syndrome2 and occasionally in conditions with underlying neuromuscular dysfunction such as cerebral palsy.4

    The secondary effects of a grossly enlarged tongue merit serious consideration for active treatment. Prolonged exposure can cause ulceration and necrosis of the mouth and tip of the tongue.5 Maxillofacial abnormalities including anterior open bite, prognathism, and an increased angle between the ramus and body have been described.3 Noisy breathing, drooling, and the unsightly appearance of a protruberant tongue, particularly in children, can cause distress. Difficulties in swallowing due to limited movement of the enlarged tongue can lead to poor weight gain and failure to thrive.4 Problems with articulation occur, particularly the expression of consonants requiring the tip of the tongue to be in contact with the alveolar ridge or roof of the mouth. The most serious and life threatening complication is airway obstruction, which is more common in generalised or posterior lingual enlargement than in anterior enlargement of the tongue.4

    The evaluation of a patient with macroglossia should begin with a thorough history and physical examination, which may allow the recognition of a syndrome of which the enlarged tongue is one component. Assessment of the tongue should include examination for masses and changes in colour and consistency.5 Thyroid function tests, isotopic imaging of the thyroid gland, chromosomal studies, and urinary mucopolysaccharide assay may be indicated. Patients with chronic airway obstruction should be assessed for pulmonary hypertension and cardiac decompensation with electrocardiography, chest radiography, arterial blood gas analysis, and Doppler echocardiography. Computed tomography and magnetic resonance imaging may be useful to delineate soft tissues and to show the extent of tumours and other masses. Microscopic examination of tongue tissue in primary macroglossia may be unhelpful, but biopsy is useful for localised lesions of the tongue that occur in chronic granulomatous and neoplastic disorders. Biopsy of other potentially affected tissue (rectum, skin, gums) is indicated to diagnose definitively amyloidosis.

    The successful management of macroglossia requires a multidisciplinary approach. Medical management may be sufficient if the enlargement of the tongue is due to systemic disease, but surgical reduction offers the best functional and cosmetic results and minimises morbidity. Airway obstruction demands prompt intervention; tracheostomy is occasionally necessary. Surgery is indicated in almost all cases of secondary macroglossia, when the tongue is affected with neoplastic disease. In primary macroglossia in infants, prevention of speech and orthodontic problems may require surgical reduction of the tongue at an early stage, preferably before 7 months of age.6 Early management helps rehabilitation and reduces the risk of permanent maxillofacial abnormalities and abnormalities of speech.

    Conservative methods of treating macroglossia are of limited value. Thyroxine in cases of hypothyroidism and bromocriptine in cases of acromegaly have obvious therapeutic benefits. Corticosteroids can be life saving in acute airway obstruction and are useful postoperatively to reduce oedema.

    Reduction glossectomy has been the main surgical treatment for patients with symptomatic macroglossia.7 Excision should be conservative whenever possible, particularly with benign disease, to allow the tongue to fit comfortably in the oral cavity and restore normal occlusion.4 Surgical techniques offer a choice of a V shaped wedge resection, circumferential wedge resection, or a combined transoral and transcervical approach for grossly enlarged lesions.8 Whatever the technique, particular attention should be given to preventing acute airway obstruction; tracheostomy is usually required to cover the perioperative period.

    Patients with macroglossia face appreciable physical and psychological problems requiring support and rehabilitation. Secondary orthodontic care and speech therapy may have important roles in this. The stigma attached to an enlarged tongue protruding outside the mouth, labelling the patient (particularly a child) as having learning disabilities, causes substantial mental anguish to patients and their families. In older children these psychological burdens often result in depression and withdrawal. In some cases psychiatric help may be needed; in most cases long term counselling and support are essential to enable patients to achieve mental stability, overcome prejudice, and reintegrate into society.

    References

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