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Macroglossia Management Depends on the Cause; Many Patients Will Need Long Term Psychological Support

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Macroglossia Management Depends on the Cause; Many Patients Will Need Long Term Psychological Support nologies in routine practice. They are typically tackled which we practise medicine, and this can be addressed only through written guidelines, policies, and protocols backed by the medical profession. up with either exhortations to do the right thing or threats Information technology will soon pervade health care, of penalties. But are these enough to produce sustainable and many doctors are interested in learning how it will improvements in the care of patients? Clinical medicine is affect them and their patients. Next month's conference on now technically and organisationally so complex that it is the topic, organised by the BMA in conjunction with the difficult to do the right thing repeatedly day after day, British Medical Informatics Society, will attempt to raise patient after patient, even ifwe know what we ought to do. the awareness and understanding among practising The knowledge about what works has to be supported by doctors ofthe benefits and disbenefits ofmedical informat- effective processes for getting it done. Simply trying to ics. An equal partnership is needed between managers, work harder and faster is no longer an answer. informaticians, systems developers, and doctors. But The rationale for medical informatics is that information above all the profession should recognise that medical systems can provide an important part of the processes informatics goes to the heart of clinical practice and must needed to deliver effective and efficient health care. Their develop and take ownership of the clinical agenda. purpose is not to replace the skills of doctors but to aug- ANTHONY NOWLAN ment those skills and to help put the knowledge of what Consultant in research and development works into practice. Systems must be useful and usable in Medical Products Group, everyday care. They must cope with complex clinical infor- Hewlett-Packard Ltd, mation while being intuitive to use, and like any good tool Bristol BS12 6QZ they should be unremarkable. Unfortunately, such clinical 1 Smith R. Towards a knowledge based health service: priorities for health technology assessment. simplicity is notoriously difficult to achieve and requires BMJ 1994;309:217-8. considerable underlying technical complexity. Fortu- 2 Berwick D. Health services research and quality of care. Assignments for the 1990s. Med Care nately, recent advances in the design of computer hard- 1989;27:761-71. ware and software combined with an understanding of "The Heart of the Matter: The Vital Role of Information in clinical processes are making new types of solution Clinical Practice" is on 1-2 December. Details are available from possible. But technological advance alone is not sufficient the BMA Conference Unit, Box 295, London WC1H 9TE because much of the hard part is bound up with the way in (tel 0171 383 6605, fax 0171 383 6663). Macroglossia Management depends on the cause; many patients will need long term psychological support Macroglossia is traditionally defined as a resting tongue lesions in the floor of the mouth including tumours, that protrudes beyond the teeth or alveolar ridge. The Ludwig's angina (an infection in the sublingual and sub- diagnosis is usually based on this sign and comparison with mandibular space, usually with a dental cause), ranula (a an apparently normal tongue (objective measurements of mucus retention cyst considered to arise from the sub- size are unreliable). The term should be reserved for cases lingual salivary gland), and angioneurotic oedema. of long term painless enlargement of the tongue and is Pseudomacroglossia is usually self limiting and regresses distinct from rapid growth of the tongue due to acute with age. Apparent enlargement ofthe tongue secondary to parenchymatous glossitis.1 micrognathia is seen in Down's syndrome and the Pierre- The condition may be classified as true macroglossia, Robin syndrome2 and occasionally in conditions with which is associated with definitive histopathological find- underlying neuromuscular dysfunction such as cerebral ings, or pseudomacroglossia, which is a relative enlarge- palsy.4 ment ofthe tongue secondary to a small mandible and with The secondary effects of a grossly enlarged tongue merit no demonstrable histological abnormalities. True macro- serious consideration for active treatment. Prolonged glossia may be primary-characterised by hypertrophy or exposure can cause ulceration and necrosis of the mouth hyperplasia of the tongue muscles-or secondary-the and tip of the tongue.5 Maxillofacial abnormalities includ- result of infiltration of normal tissue with anomalous ing anterior open bite, prognathism, and an increased elements.2 angle between the ramus and body have been described.3 Given its varied causes, both physicians and surgeons Noisy breathing, drooling, and the unsightly appearance of may see macroglossia. Common causes of true macro- a protruberant tongue, particularly in children, can cause glossia in children include hypothyroidism, lymphan- distress. Difficulties in swallowing due to limited move- gioma, haemangioma, idiopathic hyperplasia, metabolic ment of the enlarged tongue can lead to poor weight gain disorders, and chromosomal abnormalities including the and failure to thrive.4 Problems with articulation occur, Beckwith-Wiedemann syndrome.2 Amyloidosis is the com- particularly the expression of consonants requiring the tip monest cause of macroglossia in adults, the tongue being of the tongue to be in contact with the alveolar ridge or involved in both its primary and secondary forms.3 roof of the mouth. The most serious and life threatening Secondary macroglossia may also be a manifestation of complication is airway obstruction, which is more com- both benign and malignant space occupying lesions, such mon in generalised or posterior lingual enlargement than as cystic hygroma, cysts in the lingual thyroglossal duct, in anterior enlargement of the tongue.4 dermoid cysts, neurofibromatosis, granular cell tumours, The evaluation of a patient with macroglossia should and rhabdomyosarcoma.2 Other important causes include begin with a thorough history and physical examination, acromegaly, angio-oedema, lymphoma, and chronic which may allow the recognition of a syndrome of which inflammatory conditions such as syphilis and tuberculosis.2 the enlarged tongue is one component. Assessment of the The tongue may be displaced upwards and forwards by tongue should include examination for masses and 1386 BMJ VOLUME 309 26 NOVEMBER 1994 changes in colour and consistency.5 Thyroid function tests, ment for patients with symptomatic macroglossia.7 isotopic imaging of the thyroid gland, chromosomal Excision should be conservative whenever possible, partic- studies, and urinary mucopolysaccharide assay may be ularly with benign disease, to allow the tongue to fit com- indicated. Patients with chronic airway obstruction should fortably in the oral cavity and restore normal occlusion.4 be assessed for pulmonary hypertension and cardiac Surgical techniques offer a choice of a V shaped wedge decompensation with electrocardiography, chest radio- resection, circumferential wedge resection, or a combined graphy, arterial blood gas analysis, and Doppler echocar- transoral and transcervical approach for grossly enlarged diography. Computed tomography and magnetic lesions.8 Whatever the technique, particular attention resonance imaging may be useful to delineate soft tissues should be given to preventing acute airway obstruction; and to show the extent of tumours and other masses. tracheostomy is usually required to cover the perioperative Microscopic examination of tongue tissue in primary period. macroglossia may be unhelpful, but biopsy is useful for Patients with macroglossia face appreciable physical and localised lesions of the tongue that occur in chronic granu- psychological problems requiring support and rehabili- lomatous and neoplastic disorders. Biopsy of other poten- tation. Secondary orthodontic care and speech therapy tially affected tissue (rectum, skin, gums) is indicated to may have important roles in this. The stigma attached to diagnose definitively amyloidosis. an enlarged tongue protruding outside the mouth, The successful management of macroglossia requires a labelling the patient (particularly a child) as having learn- multidisciplinary approach. Medical management may be ing disabilities, causes substantial mental anguish to sufficient if the enlargement of the tongue is due to patients and their families. In older children these psycho- systemic disease, but surgical reduction offers the best logical burdens often result in depression and withdrawal. functional and cosmetic results and minimises morbidity. In some cases psychiatric help may be needed; in most Airway obstruction demands prompt intervention; cases long term counselling and support are essential to tracheostomy is occasionally necessary. Surgery is indi- enable patients to achieve mental stability, overcome prej- cated in almost all cases of secondary macroglossia, when udice, and reintegrate into society. the tongue is affected with neoplastic disease. In primary P MURTHY macroglossia in infants, prevention of speech and ortho- Registrar in otolaryngology M R LAING dontic problems may require surgical reduction of the Consultant otolaryngologist tongue at an early stage, preferably before 7 months of Raigmore Hospital NHS Trust, age.6 Early management helps rehabilitation and reduces Inverness IV2 3UJ the risk of permanent maxillofacial abnormalities and abnormalities of speech.
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