British Journnf of Piasfic Surgery (r973), 26, 27+-276

AMYLOIDOSIS: REPORT OF A CASE PRESENTING

WITH MACROGLOSSIA

By K. A. HICKS, F.D.S.R.C.S.(Eng.) and W. R. DICKIE, F.R.C.S.(Edin.)

Northern Ireland Plastic and Maxilla-Facial Service, The Ulster Hospital, Belfast

AMYLOIDOSIS is an unusual condition, characterised by the deposition of an amorphous eosinophilic material between the cells and in the walls of the blood vessels of the affected organs. Any organ, including the tongue, may be affected by amyloidosis but it is rare for macroglossia to be as gross as in the case reported here.

CASE REPORT

A 68-year-old male with macroglossia was referred with a tentative diagnosis of sarcoidosis. Two years previously he had first noticed bilateral submandibular swellings which gradually increased in size. Nine months later, his tongue started to enlarge and 15 months later it had become so large that it protruded continuously from his mouth (Fig. I). He was unable to take solid food and had lost 42 pounds in weight. His inability to close his lips and excessive salivation resulted in a constant dribble of saliva. Coherent speech was almost impossible. He gave no history of any chronic disease apart from osteoarthritis.

FIG. I. The tongue at rest protruding from the mouth.

FIG. 2. Dorsal furrowing on actively protruded tongue.

His father and mother had died at 75 and 80 years of age respectively. He has 2 brothers and 3 sisters, all over 60 years of age, and all alive with no known overt disease.

He was a pale thin man with a fast, irregular pulse. The massive symmetrical enlargement of the tongue held the jaws widely open and displaced the submandibular salivary glands downwards. Firm enlarged discrete lymph nodes were palpable in the axillae and groins but neither the liver nor the spleen was clinically enlarged.

The tongue was firm with slight pitting on pressure but still mobile; when fully protruded, longitudinal furrows appeared on the dorsum (Fig. 2). The enlargement involved mainly the anterior two-thirds of the tongue on which the filiform papillae were absent and the fungiform papillae swollen. The jaws were edentulous and the maxillary tuberosities flattened buccally from pressure of the tongue.

274

AMYLOIDOSIS 275 Chest X-rays showed a left ventricular enlargement and old tubercular lesions at the apices

‘of the lungs. The submandibular sialogram showed a normal ductal pattern. The sedimentation rate was increased to 50 mm./1 hour and the serum alkaline phosphatase

to 29 King Armstrong units. Other haematological and biochemical tests were normal and there was no Bence Jones protein in the urine.

FIG. 3. Section of tongue showing deposition of amyloid in the wall of a blood vessel.

FIG. 4. The patient 3 months after operation.

FIG. 5. Intra-oral view showing the tongue 3 months after tongue reduction.

A biopsy of an axillary lymph node demonstrated focal deposits of amyloid. A bone marrow biopsy showed normal marrow; the plasma cells were normal in appearance and accounted for 3-5 per cent of the total nucleated cells.

At operation a large wedge measuring 90 mm. by 80 mm. was removed from the anterior two-thirds of the tongue. Two smaller wedges were taken from the sides of the tongue stump to reduce the bulk of the remaining tissue. The muscle in the anterior part of the floor of the

276 BRITISH JOURNAL OF PLASTIC SURGERY

mouth was normal but the remainder was friable and atrophic; this made it difficult to control the slight but prolonged ooze from small blood vessels even after ligation of the lingual arteries. The wound was closed with chromic catgut sutures.

Post-operatively there was difficulty in closing the jaws due to muscle spasm but this improved rapidly during the first week with concomitant improvements in speech and control of saliva.

Microscopic examination of the specimen showed a superficial oedema immediately below the dermis. Deep to this there was a diffuse infiltration of amyloid around and between the muscle fibres which were atrophic. Deposition of amyloid was also marked around the blood vessels (Fig. 3).

When reviewed 3 months after operation, the patient remained well and had gained 20 pounds in weight (Fig. 4). The tongue was soundly healed. There was no suggestion of further deposition of amyloid (Fig. 5). The serum alkaline phosphatase was 16 King Armstrong units and the sedimentation rate had dropped to within normal limits.

DISCUSSION

Symmers (1956) classified amyloidosis into three main groups on clinicopathological findings: generalised secondary amyloidosis, generalised primary amyloidosis and localised amyloidosis.

Macroglossia is a common finding in primary amyloidosis. Rukavina (1956) in a

review of 154 cases and Briggs (1961) in an analysis of 20 cases report a 20 per cent incidence of macroglossia. Macroglossia can also occur, but is less common, in secondary amyloidosis (Brandt et al., 1968) and amyloidosis associated with multiple myeloma (Cahn, 1957). There have been no recorded cases of localised amyloidosis of the tongue in recent literature.

Macroglossia in myelomatosis with amyloidosis may precede the bone lesions and an absence of Bence Jones proteinuria does not exclude the possibility of the former condition. A sternal marrow biopsy should therefore be part of the routine investigation (Buchanan et al., 1969).

When the patient was first examined the appearance and consistency of the tongue suggested that some of the enlargement might be due to oedema. Subsequent histo- pathological examination confirmed the presence of superficial oedema due to the obstructive effect of the amyloid infiltration.

SUMMARY

A case of amyloidosis of the tongue causing macroglossia in an elderly man is described together with the technique used for surgical reduction.

REFERENCES

BRANDT, K., CATHCART, E. S. and COHEN, A. S. (1968). prognosis of 42 patients with amyloidosis.

A clinical analysis of the course and

BRIGGS, G. W. (1961). Amyloidosis. American Journal of Medicine, 44, 955-969.

Annals of Internal Medicine, 55, 943-957. BUCHANAN, J., GIBSON, I. I. J. M., GIBSON, T. and RUSSELL, A. R.

in Myelomatosis. (1969).

British Journal of Plastic Surgery, 22, 157-160. Macroglossia

CAHN, L. (1957). Oral amyloid as a complication of myelomatosis. Oral Surgery, Oral Medicine, Oral Pathology, IO, 735-742.

RUKAVINA, J. G., BLOCK, W. D., JACKSON, C. E., FALLS, H. F., CARET, J. H. and CIJRTIS, A. C. (1956). Primary systemic amyloidosis: a review and an expenmental, genetic and clinical study of 29 cases with particular emphasis on the familial form. Medicine, 35, 239-334.

SYMKEYE;:. ST. C. (1956). Primary amyloidosis: review. Journal of Cfinicaf Pathology, 9,