Macroglossia

DEFINITION.—Hypertrophy of the tongue.

SYNONYMS.—Megaloglossia, Glossoptosis, Prolapsus linguæ, Lingua propendula, Chronic prolapse of the tongue, Chronic intumescence of the tongue.

HISTORY.—This rare affection has been long known, the first cases on record being in the works of Galen. Other cases have been recorded by Celsus and Avicenna. Among more modern recorders may be mentioned Scaliger (1570), Bartholin (1680), Benedict and Pencer; among recent recorders, Lassus, Percy, Harris, Humphrey, Gayraud, W. Fairlie Clarke, Bryant, and the French dictionaries in present process of publication; to all of which the reader is referred for bibliographic, descriptive, and illustrative details.

Mémoire de l'Institut National, 18—, an VI. t. i.

Dict. Sci. Méd., t. xxvii.

Am. Journ. Med. Sci., vol. vii., 1830, p. 17; vol. xx., 1837, p. 15—both illustrated.

Trans. Med.-Chir. Soc. London, 1853, p. 113.

Thèse de Montpellier, No. 68, 1865.

Diseases of the Tongue, London, 1873.

"Surgical Affections of the Tongue," Guy's Hosp. Reports, 1883, p. 102 et seq.

ETIOLOGY.—This affection is usually congenital, at least to a certain extent, and augments with the growth of the child. It has been attributed, on apparently insufficient grounds, to injury received during parturition. It is probably intra-uterine in origin. Though encountered in both sexes, the majority of recorded cases have been in females. In summing up these observations, it appears that the affection often attracts little or no attention until dentition is in progress. The hypertrophy begins to augment rapidly during the second or third year of age, or a year or two later in some cases.

The gradual increase of the congenital deformity during infancy has been attributed to hypernutrition from local irritation produced by habits of sucking on the organ, induced, in some subjects, by forcible efforts at suction from a short nipple. Similarly, the rapid augmentation of volume noted as occurring during the period of dentition or a little later has been attributed to hypernutrition excited by irritation suffered by the protruding organ from the lower row of teeth. Cases commencing at this age have been supposed to be due exclusively to tongue-sucking. In some instances, due to this cause apparently, the deformity is associated with idiocy (Lawson). Convulsions, epileptic seizures, and whooping cough have been regarded by some writers as occasional causes of the deformity. Indeed, idiocy and cretinism are not infrequent co-associates with the deformity (Parrot). It has been observed likewise in anencephalous monsters (Brissot, idem).

Trans. Clin. Soc. London, vol. v. p. 158.

Gaz. Méd. Paris, Dec. 10 and 17, 1881; Lond. Med. Record, Mar. 15, 1882, p. 113.

SYMPTOMS, COURSE, DURATION, TERMINATIONS, COMPLICATIONS, AND SEQUELÆ.—The prominent symptom of macroglossia is the enlarged tongue protruding beyond the mouth. The resemblance of the protruding tongue of a child with macroglossia to the tongue hanging from the mouth of a calf gave rise to the name lingua vitulina by which it has sometimes been designated. In some instances, where the enlargement is but moderate, the organ can be retained within the mouth. When bilateral, the enlargement may be symmetrical, or may interest one side of the tongue more than the other. When the enlargement is confined to the free portion of the tongue, it interferes little with respiration and with movements of suction. When occupying the base of the organ, it may seriously embarrass respiration, and even produce suffocation in some instances (Clarke). The mouth being maintained open, saliva dribbles away constantly except during alimentation. Thirst is often very great in consequence of this, and of the desiccation of the tongue and of the walls of the mouth by the unmodified air to which they are continuously exposed. The tongue is usually free from pain.

In some subjects, although the tongue, left to itself, protruded considerably, it has been found quite practicable to maintain it within the cavity of the mouth by means of bandages or other appliances secured to the back and top of the head. These bandages are removed from time to time to give relief from the restraint and to permit food and drink to be taken. Systematic compression, indeed, has been induced in this way in some instances, and has produced considerable diminution in the size of the organ—sufficient to maintain its concealment without the aid of an appliance. When the tongue cannot be retained within the mouth the patient becomes unable to close the jaws. Hence saliva dribbles constantly, save when food or drink is being taken. The protruded portion of the tongue undergoes a livid discoloration, sometimes diffuse, sometimes disseminated. Though sometimes remaining comparatively soft in texture, it usually becomes hard, dry, rough, fissured, ulcerated and sanious, covered with desiccating layers of mucus and epithelium, and marked by indentations made by the edges of the teeth, which sometimes seem almost to strangle it. Mastication, deglutition, and articulation often become very difficult, and respiration also, but less frequently. The lower lip becomes much everted. The larynx and hyoid bone become drawn upward and forward by the weight of the organ. The configuration of the lower jaw undergoes considerable change, and the teeth become pressed out of position. Dislocation of the jaw from this cause has been noticed (Chalk).

Trans. Path. Soc. London, vol. viii. p. 305.

These symptoms undergo aggravation with the growth of the subject, and, while presenting general features of resemblance in all cases, vary considerably in individual instances. Great difficulty is encountered, as a rule, in taking food, and mastication has to be performed very slowly. In some instances mastication can be performed satisfactorily by the molars, owing to a compensatory curvature of the lower jaw, even though the anterior portions of the jaw may remain permanently separated (Harris). Some patients get along by using their fingers to push the bolus far enough back to permit of its deglutition. Some have used a cup with a long tube slightly curved to convey fluids to the back part of the mouth for a similar purpose. Some have been systematically fed by means of a catheter passed through a nasal passage and thus on into the oesophagus. The difficulties in nourishing patients reduce some of them to extreme emaciation.

Notwithstanding all these drawbacks, quite a number of cases are on record where the patients have reached well into adult life before being submitted to radical measures for relief. One patient is recorded as having reached the age of eighty, having worn for some sixty-five years a silver shield to conceal her deformity (Clarke).

PATHOLOGY AND PATHOLOGICAL ANATOMY.—The hypertrophy may involve all the structures of the tongue, but usually implicates the muscular tissue especially (Sédillot, Paget, Bouisson). In a case published by W. Fairlie Clarke it was found on microscopic examination that the papillæ as well as the mucous and submucous tissues were somewhat enlarged and thickened, while the bundles of muscular fibre were slightly coarser than natural. Maas reports a unilateral case of macroglossia in a male child two months of age associated with hyperdevelopment of the entire left side of the body. In some instances the blood-vessels and lymphatics are chiefly involved (Virchow, Billroth, Maas), two cases of which have been described by Virchow as cavernous lymphatic tumors.

Arch. klin. Chir., p. 413, Bd. xiii. Heft 3.

Hilliard reports a congenital case from vicious growth, removed at fourteen months of age. Microscopic sections showed the large lacunæ filled with corpuscles, blood-pigment in different stages of degeneration, and the papillæ much hypertrophied. Winiwarter reports a congenital macroglossia associated with congenital cysts of the neck.

Brit. Med. Journ., Nov. 26, 1870, p. 591.

Arch. klin. Chir., 1874, Bd. xvi. Heft 3.

Sometimes there is very little true muscular hypertrophy, as in a case quoted by Bryant which was presented to the Pathological Society of London in 1872 by M. H. Arnott. In this specimen the epithelial covering was very thick and the papillæ enlarged. The blood-vessels were larger than usual, and there were large irregular spaces, thin-walled and filled with blood or clear fluid. "A few vesicular bodies which may have been enlarged lymphatics were also present"—probably cross-sections of lymphatic vessels.

The size that may be attained even in young children seems incredible, three and four inches protruding from the mouth in some instances. The free portion is more bulky than the intra-oral portion. One case reported "as thick as an arm" probably refers to the arm of the child. As a rule, both sides of the tongue are involved; exceptionally, the affection is unilateral.

In most instances the hypertrophy occupied the free portion of the tongue chiefly, the base of the organ having been implicated in but a few.

FIG. 14.

Chronic Intumescence of the Tongue (Harris).

FIG. 15.

Hypertrophy of the Tongue (Harris), before operation and after. A, odontolith.

DIAGNOSIS.—The presence of the tongue outside of the mouth speaks for itself (Figs. 14 and 15). The age of the patient, usually a young child, the history of the case if it present in the adult, suffice to differentiate macroglossia from the tumefaction of glossitis on the one hand and from certain protruding tumors and malignant diseases on the other. Hypertrophy of the tongue following chronic glossitis, syphilitic or non-specific, must not be confounded with the congenital or idiopathic affection under consideration.

PROGNOSIS.—The prognosis is good as to relief from the deformity, provided the patient is submitted to surgical interference, and the prognosis of the operation depends upon the procedure selected. Sometimes additional operations are requisite to remedy the defects the lower jaw has sustained by prolonged depression. In comparatively young adults restoration of its position, configuration, and function seems likely to result spontaneously after the protruding portion of the tongue has been removed.

TREATMENT.—It has been maintained (Lassus) that the hypertrophy can be overcome by systematic compression of the tongue, by leeching the tongue, bandaging or strapping it, and forcibly maintaining it in the mouth by suitable retentive appliances. While it has been admitted that this plan may prove successful in cases of moderate enlargement of but few years' duration and unaccompanied with change in the shape of the lower jaw, the experience of more recent observers has been recorded as unfavorable, at least in pronounced cases. Clanny succeeded in this way with a child five years of age whose tongue protruded three inches. This plan is said to be very painful and irritating. It requires close watching on account of the difficulty of respiration which may ensue from thus blocking up the pharynx. It has been advised as a useful and sometimes an essential preliminary (Syme) to a radical procedure consisting in the excision of a V-shaped segment. This latter operation (Boyer) has been successfully performed by Howe, Harris, Humphry, Syme, and others. Frederici extended the incisions to the very base of the tongue. It has been performed both with knife and with scissors, the cut surfaces being united with sutures after ligation or torsion of the bleeding vessels. Re-enlargement ensued in one of Syme's cases operated upon in this way, and likewise in a case of Gies, requiring subsequent excision of the exuberant portions. Operations with the ligature, though sometimes successful (Fergusson, Hodgson), may be followed by fatal septicæmia during the slough (Liston), or, failing to strangulate the tongue sufficiently, may require the application of the knife, after all, to affect the separation (Harris).

Edinb. Med. and Surg. Journ., 1805, vol. i., cited by Clarke.

Arch. gén. de Méd., 1844; Edinb. Med. and Surg. Journ., p. 528, vol. lxiv., 1845.

Edinb. Med. Journ., 1857, vol. ii. p. 1057.

Arch. klin. Chir., 1873, p. 640.

Practical Surgery, London, 5th ed., p. 518.

Trans. Med.-Chir. Soc. London, 1858, p. 129.

Elements of Surgery, p. 334, Philada., 1842.

Am. Journ. Med. Sci., vol. vii. p. 17.

Excision with the incandescent loop of the galvano-cautery seems to be the most suitable procedure. Valerani operated in this manner without the loss of a drop of blood upon a congenital macroglossia in a child seven months of age. Maas operated in this way on a child two months of age. Fairlie Clarke, who removed a congenital macroglossia with the écraseur in a child five months of age, recommends operation before dentition begins—an opinion which appears to be justified by the belief that the pressure of the teeth contributes to the subsequent rapid enlargement of the organ. Nevertheless, the operation may be undertaken at any age. Several of those already cited were performed upon adults, and Stephen O'Sullivan excised the hypertrophied tongue of a female sixty-five years of age.

Giornale della Reale Accademia di Turino, fasc. 1518; London Med. Record, Sept. 15, 1876, p. 408.

Loc. cit.

Lancet, March 30, 1872, p. 432.

Dublin Journ. Med. Sci., Aug., 1875, p. 178.

Ignipuncture with the thermo-cautery of Paquelin has been successfully used of late by Helferich and by von Bruns of Tübingen. In the latter instance the subject was five years of age. Fourteen punctures were made from above downward at intervals of about one centimeter, and five were made transversely. Not a drop of blood was lost. On the third day secondary hemorrhage occurred from the intercommunication of three of the punctures; this was restrained by ferric chloride, and the case went on to a favorable conclusion. Surgical procedures must constitute our sole therapeutic reliance. The temporary subsidence of enlargement under the influence of mercury and the iodides seems sure to be followed, sooner or later, by reproduction of the deformity. It is therefore a waste of time to attempt cures by medication.

Centbl. f. Chir.; Med. Times and Gaz., Sept. 23, 1883.