The results of the statistical survey on Japanese patients with malignant exophthalmos performed in 1978 are reported.While 15,442 patients with Graves' disease were seen in 9 thyroid clinics, 121 cases (0.8%) were diagnosed as malignant exophthalmos. Exophthalmos was present in 30.1% of 8,801 cases of Graves' disease.Two hundred and twelve patients with malignant exophthalmos reported from 13 institutions were analyzed. The male-female ratio of the patients was 1 : 1.06, and 22.2% of them was not associated with hyperthyroidism. The age distribution of the patients' first visit peaked at the 5th decade.A family history of thyroid disorder was found in 11.8%. Twenty-one percent of the patients had previous treatments for hyperthyroidism. Aggravation of eye symptoms was reported in some patients treated with antithyroid drugs or radioiodine. Goiter was not palpable in 16% of the cases, and estimated weight of the thyroid was smaller in patients without hyperthyroidism. The consistency of the goiter was elastic firm in 35% of the cases. Thyroid histology revealed Hashimoto's thyroiditis in 6 out of 20 patients studied. Thyroid function varied widely from hypothyroid to hyperthyroid ranges, being mostly in euthyroid or mildly thyrotoxic ranges. Few patients showed a normal TRH-test or T3 suppression test. Antithyroglobulin and antimicrosomal antibodies by TRC were detected in 17.8% and 63.5%, respectively.The grade of proptosis ranged up to 29mm with the maximum right-left difference of 7mm. The incidences of eye symptoms were as follows : pain 30.5%, conjunctival injection and chemosis 89.3%, diplopia 66.3%, restriction of eye motion 51.6%, lagophthalmos 27.0%, corneal lesion 44.4%, visual field defect 9.4%. Upper and lateral gaze was most often limited.Abnormalities on funduscopy, roentgen study and CT were found in 18%, 5%, and 94% respectively. A follow up study up to 5 years revealed amelioration with treatment in 36%, no change in 55%.The treatment reported effective included adrenocortical steroids, radiation and thyroid hormones, however, the effect of steroids and radiation on proptosis was considered temporary in most cases.
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