|Year : 1990 | Volume
| Issue : 2 | Page : 61-63
Management of progressive exophthalmos
S Kumar1, Chowdhury Subhankar2, SC Sen3
1 Chief of Endocrinology unit and Reader of Medicine, Medical College Hospitals, Calcutta, India
2 Dept. of Medicine, Medical College Hospitals, Calcutta, India
3 Professor of Ophthalmology, Regional Institute of Ophthalmology, Medical College Hospitals, Calcutta, India
20, B Garcha 1st lane, Calcutta-700 019
Source of Support: None, Conflict of Interest: None
The present study reveals our experience in the medical management of 30 cases of progressive/malignant exophthalmos, employing costicosteroid, b-blocker, diuretic and a preparation containing proteolytic enzymes. There was encouraging improvement in terms of symptoms, proptosis and optic nerve compression, while reduction of ophthalmoplegia was not so satisfactory. However, none of the patients needed decompression surgery.
|How to cite this article:|
Kumar S, Subhankar C, Sen S C. Management of progressive exophthalmos. Indian J Ophthalmol 1990;38:61-3
| Introduction|| |
Exophthalmos in Graves' disease and also idiopathic exophthalmos often have an eventual self-limiting course , Nonetheless, the exophthalmos can progress rapidly and become the major clinical concern because of possible damage to the cornea through exposure keratitis and damage to vision owing to increased intraorbital pressure . So, it becomes necessary to treat the exophthalmos actively in order to salvage the function of the eye. We record here our experience in the management of 30 cases of progressive/malignant exophthalmos using a multi-pronged approach.
| Material and methods|| |
30 cases of progressive/malignant exophthalmos seen jointly by the Endocrinology and Ophthalmology Depts. of Medical College Hospital, Calcutta are included in the study. All `Surgical' causes of exophthalmos were excluded by plain X-ray, ultrasonogram or C.T. scan, when there was a clinical suspicion and especially when the patient was euthyroid and/or, the exophthalmos was unilateral. Orbital inflammatory disease was excluded on clinical grounds like, lack of pain on ocular movement, absence of fever or, leucocytosis and lack of response to antibiotics. The study was confined to cases of clinical Graves' disease and idiopathic exophthalmos; the latter group is also often included in the broad category of dysthyroid or endocrine exophthalmos/ophthalmopathy, as many of these patients have subclinical thyrotoxicosis (diagnosed by special tests like, T3 suppression of Radioactive Iodine uptake or, TSH response to TRH) or, subsequently develop thyroid dysfunction or, goiter. Among the patients 17 (56.7%) were male and 13(43.3%) female. The age range was from 32 years to 54 years. 11 (36.7%) patients had clinical thyrotoxicosis, while the remaining 19 (63.3%) had so-called idiopathic exophthalmos. 18 (60%) patients had unilateral, 11 (36.7%) had bilateral asymmetrical and 1 (3.3%) had bilateral symmetrical exophthalmos. In the thyrotoxicosis group 7(3.6%) were female while in the idiopathic group only 6 ( 31.6%) were female. The investigations in each patient included:
1.Thyroid function studies employing serum T 3 , T4 & TSH values.
2.Ophthalmologic check up like:
(b)Record of Visual acuity.
(c) Record of field of vision, by perimetry and scotometry.
(d)Record of eyeball movements and
(e)Exophthalmometry-which gives an index of the severity of proptosis.
3.Monitoring of blood sugar, serum electrolytes and adreno cortical function., besides, in 4 out of the 18 patients who had evidence of optic nerve compression (like abnormality on ophthalmoscopy, visual acuity, perimetry or scotometry) visual evoked potential (V. E. P.) was also recorded and showed reduction in amplitude with distortion of the wave form.
The ophthalmologic check ups were performed once at the start of the treatment regime, at weekly intervals till the end of the regime, at monthly internals for 6 months after stoppage of treatment and then at 3 month intervals for 6 months after stoppage of treatment and then at 3 month intervals for a maximum of 5 years. Patients with clinical thyrotoxicosis had antithyroid treatment as relevant; also wherever required, local ophthalmic treatment was administered. The treatment regime under discussion was embarked upon only in progressive/malignant exophthalmos causing symptoms like pain, itching, lacrimation, redness, or, exophthalmoplegia, threatening visual loss or, primarily for cosmetic reasons. At the start of treatment 6 (20%) patients had early exposure keratitis, 18 (60%) had signs of optic nerve compression; 20 (66.7%) . had exophthalmoplegia, while 6 (20%) were treated purely for cosmetic reasons.
The regime consisted of :- Inj. Dexamethasone 8 mg IM BD x 1 wk, .then, 4 mg IM BD upto 4wks. Tab Frusemide 40 mg BD x 3 days, then 40 mg OD upto 4 wks. Tab Proporanolol 20 mg TDS x 4 wks Tab Trypsin & Chymotrypsin (in ratio 6:1) equivalent to 100,000 units proteolytic activity OD AC x 4 wks. + Potassium supplement.
Blood pressure, blood sugar and serum electrolytes were monitored during therapy and adrenocortical function was evaluated on stoppage of the treatment. History of peptic ulcer was enquired into in each case. The full treatment could be offered to each patient. Blood sugar increased in 4 patients, but it was readily controlled in all by dietary restriction, which also could be withdrawn within 4 wks of stopping this regime.
| Results|| |
The clinical response was assessed in terms of (a) improvement of symptoms like pain, itching, watering and visual impairment, (b) reduction of proptosis (determined from exophthalmometric reading), (c) reduction of features of optic nerve compression (determined from ophthalmoscopy, record of visual acuity, perimetry, scotometry and V.E.P.) and (d) reduction of ophthalmoplegic element. The response was graded as excellent, good, fair or insignificant in each patient, which in case of reduction of proptosis was considered for more than 3.5 mm, 2 to 3.5 mm, 0.5 to 2 mm and less than 0.5 mm respectively at the end of 4 weeks of treatment.
Symptom improvement, reduction of proptosis, reduction of optic nerve compression and improvement in ophthalmoplegia were excellent or good in 76.7%, 66.7%, 77.7% and 20% of the cases respectively and insignificant in 3.3%, 6.67%, nil and 65% of the cases respectively. In no case was decompression surgery required. During the follow up there was a recurrence in 9 i.e., 30% of the cases, which necessitated a second course of treatment. All such recurrences were within the first 6 months of the first treatment and responded promptly to the second course of treatment.
| Discussion|| |
The present study showed a worthwhile response of progressive/malignant exophthalmos to a combination therapy utilising corticosteroid, beta-blocker, diuretic and a preparation containing trypsin and chymotrypsin. We have not come across such an integrated multi-pronged approach to the treatment of progressive exophthalmos in the literature. The study revealed satisfactory response in terms of symptom relief, reduction of proptosis and features of optic nerve compression; but improvement in ophthalmoplegia was unsatisfactory or, insignificant in 65% of the cases. Probably the myopathic features are related to fibrosis in the muscles concerned and hence were not reversible.
Corticosteroids have been the cornerstone of therapy for malignant exophthalmos and probably act by their immunosuppressive and antiinflammatory properties  Rather large doses of prednisolone 100 mg or, its equivalent daily are required. Some studies have shown that patients who benefited from steroid therapy were immunologically different from non-responders and showed, during therapy, an increase in T-cell count and good responses to non specific mitogens sub . Such immunologic studies were, however, not done in the present study. Parenteral steroid was used in preference to oral route in order to ensure better bioavailability and also to reduce risks of acute gastritis and peptic ulceration. Other immunosuppressives like azathioprine, methotrexate, cyclophosphamide have also been used, usually in combination with corticosteroid. More recently, cyclosporin has also given a favourable response with improved motility, reduction of proptosis and decreased swelling of orbital muscles as demonstrated by C.T. Scan sub .
Plasma exchange has also been tried, but in one study of 18 patients followed up for an average of 13 months no significant consistent improvement was noted. 
Radiotherapy is another modality of treatment, usually reserved for patients who fail to respond to or, have unacceptable side affects from systemic corticosteroids. While progression of the congestive element is altered and a slight decrease in proptosis is observed, there is usually no improvement in the myopathy . In this study no case required radiotherapy.
Surgical decompression of the orbit is the last resort and can bring about significant reduction of proptosis, but it may take 6-12 months to become apparent  Fortunately none needed decompression surgery in our study.
Beta-blocker was used in this study as there is evidence of sympathetic overactivity in cases of exophthalmos, especially when associated with thyrotoxicosis.
The rationale of our use of diuretic was to provide symptomatic improvement by reducing orbital fluid content. The combination of proteolytic enzymes probably helped by breaking down any inflammatory exudate.
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[Table - 1], [Table - 2], [Table - 3]