Frozen Eye What Could it be? - QScience.com

QATAR MEDICAL JOURNAL | VOL. 15 / NO. 2 / NOVEMBER 2006

CASE

REPORT

Frozen Eye What Could it be? Yassin M.A., Khan F.Y., Al Ani A., Othman M.E. Department of Medicine, Hamad Medical Corporation Doha, Qatar

Abstract: Thyroid associated opthalmopathy (TAO) may precede, coincide, or follow the systemic complications of dysthyroidism it can present gradually as dry eyes, puffy eyelids, ocular pressure or pain, field loss, diplopia with 3rd, 4th, 6th nerve palsies we report a 22 year old Filipina female who presented with sudden onset of diplopia, pain, headache, with no sign or symptom of dysthyroidism and clinically sounds to have 3rd, 4th, and 6th, nerve palsies (left eye) the case represents diagnostic dilemma that's why found it worth to be reported.

well built in good general condition with stable vital signs, HEENT NAD, neither goiter nor lymphadenopathy were detected, no skin rash, examination of her eyes revealed paresis of the 3rd, 4th, and 6th cranial nerves on the left side, Figure 1 but her fifth cranial nerve was Intact, there was lower motor neuron lesion on the right side, which she claimed it's old since childhood the rest of The neurological examination wasn't remarkable. Examinations of the cardiovascular, respiratory, abdomen, Musclosketal systems weren't remarkable.

mmg

Introduction: Ophthlmoplegia is interested subject for both discussion as well as clinical examination some times it is very easy to diagnose the underlying a etiology and sometimes represent a diagnostic dilemma. Thyrotoxicosis is one of the causes, which sound easy to diagnose, but if you did not but in mind you can be easily missed will discuss a case of ophthalmoplegia in which we faced diagnostic dilemma.

Case Report: Our patient is 22 year old Filipina female with benign past medical history who presented through Accident and Emergency with history of inability to move her left eye for five days duration associated with headache and diplopia on looking to the left side the condition associated with pain as well. The course was sudden there was no history of similar condition before, loss of consciousness or deterioration in level of consciousness, abnormal movement, weakness in mastication, loss of weight, heat intolerance, palpitation, excessive sweating, and change in bowel habits. Patient is single has no sexual partner and not using oral contraceptive pills. She has no history of skin rash, joint pain, serositis. On examination a young female

Address for correspondence: Mohammed

Abdeldaem

Yassin, MD

Department of Medicine, Hamad Medical Corporation P. O. Box 3050, Doha, Qatar Fax: (+974)4392273; E-mail: [email protected]

54

Figure 1: Before treatment Lab works showed CBC, urea and electrolyte were normal, ESR was 16 mm/hr, CRP1 mg/1, LP done and revealed protein of 0.19 g/1 CSF glucose 3.6 mmol/1 serum glucose 5.9 mmol/1 WBC< 5 RBC 3/ul no organism could be seen by Gram stain, tensilon test as well as anti acetylcholine antibodies were negative since there was a great suspicion of cavernous sinus thrombosis urgent CT scan of the brain was With exaggerated enhancement of retro orbital fat. no orbital lesion optic nerves are with in normal any significant Proptosis, thyroid scan revealed diffusely increase uptake (diffuse hyperthyroidism), TFT showed TSH < 0.01miu/l. FT4 23.8 pmol/1 FT3 8.2 pmol/1 so finally patient was diagnosed as case of thyrotoxicosis started on neomercazole, as well as oral prednisolone follow up in the clinic showed great improvement (Figure 2). Patient given prednisolone orally as 1 mg/kg, showed partial improvement in one month and total recovery in 10 weeks after which prednisolon was tapered till discontinued there was no relapse after stopping the prednisolone, she was kept on neomercazol since beginning (dose of 15 mg BD).

Frozen Eye What Could it be?

Figure 2: After treatment

Discussion: Thyroid associated ophthalmopathy (TAO), frequently called Graves's ophthalmopathy, is an organ specific autoimmune process that is strongly associated with dysthyrodism. T-cell lymphocytes are believed to react against thyroid follicular cells with shared antigenic epitopes in the retro orbital space. Whether T-cells are involved in a cell-mediated or a humoral immune response is not certain. Although evidence for cellular and humeral immunity against various orbital antigens exists, the nature of the primary antigen(s) that is recognized by immunocompetent cells and auto antibodies has not been definitively determined. TAO patients may complain of the following ocular symptoms: Dry eyes, Puffy eyelids, Angry-looking eyes, Bulging eyes, Diplopia, Visual loss, Field loss,yschromatopsia, Photopsia on up gaze, Ocular pressure or pain. Symptoms of hyperthyroidism or Symptoms of hypothyroidism. TAO is the most common cause of unilateral and bilateral proptosis in adults. Proptosis occurs because the orbital contents are confined within the bony orbit, and decompression can only occur anteriorly. Unilateral proptosis of TAO usually reflects asymmetric muscle involvement as in our patientLacrimal gland enlargement is not uncommon Lid retraction may occur in both the upper and lower lids because of a sympathetically innervated tarsal muscle in both lids. If eyelid retraction is absent, then TAO may be diagnosed only if (1) proptosis, optic nerve involvement, or restrictive extra ocular myopathy is associated with thyroid dysfunction or abnormal regulation, and (2) no other confounding ophthalmic features are apparent. Serum TSH (thyrotrophic) is useful to establish the diagnosis of hyperthyroidism or hypothyroidism. Usually, the TSH is low in hyperthyroidism in our patient it was (TSH < 01miu/l) and high in hypothyroidism. Other blood tests that may be useful include calculated free T4 (thyroxine) index, thyroidstimulating immunoglobulin, antithyroid antibodies, and serum T3 (triiodothyronine). The introduction of direct assays for TSH, free T4, and free T3 has superseded the usefulness of total T4 and T3 resin uptake testing. Antithyroid peroxidase antibody is believed to be more sensitive than antimicrosomal antibody in the detection of autoimmune thyroid disease. Ultrasound, CT scan, and MRI can be performed, as in our

Yassin MA.,

et.al.

patient who underwent an MRI, which is showed below (Figure 3). The natural history of Graves' ophthalmopathy is variable and must be considered in the context of concomitant antithyroid therapy. In some patients, for example, ophthalmopathy changes little for many years. In others, it may worsen or improve, or, in a few patients, follow a course characterized by exacerbations and remissions. These variations make it difficult to reach conclusions about the efficacy of treatment0*. Treatment of patients with Graves' ophthalmopathy has three components. Reversal of hyperthyroidism, if present, Symptomatic treatment, Treatment with a glucocorticoid, orbital irradiation, and orbital decompression, surgery to reduce inflammation in the periorbital tissues. Other immunosuppressive drugs (mostly cyclosporine), have not been proven to be effective(2) .octeriotide, a somatostatin analog, was no more effective than placebo in one randomized trial(3). In a second randomized trial, octreotide-LAR did not improve a composite scale of disease activity and severity, but was associated with reduced proptosis(4). Graves' ophthalmopathy may worsen or first become apparent after treatment of hyperthyroidism, depending upon the treatment. A randomized trial, for example, evaluated 168 patients with Graves' hyperthyroidism, 22 of whom had preexisting ophthalmopathy^. Correction of the hyperthyroidism was associated with new onset of ophthalmopathy in 22 (15 percent) and worsening of the ophthalmopathy in 8 (36 percent). The worsening was manifested in most patients by an extra 1 to 2 mm of proptosis.Local measures including eye shades, artificial tears (saline eye drops), and raising the head of the bed at night often lead to sufficient relief of eye symptoms .The optimal dose of prednisolone is uncertain. Some physicians initiate therapy with a high dose, such as 100 mg/day. However, doses of 30 to 40 mg/day appear to be as effective and have fewer side effects in our case we gave lmg/kg oral prednisolone. Improvement usually occurs within four weeks as in our patient who showed partial improvement in her eye movement in four weeks and total improvement in 10 weeks time. About onehalf of patients have a good response to prednisone by the end of six months; those patients with less muscle swelling are more likely to respond(6). In some patients the eye disease worsens when the dose of prednisoloneis reduced which was not the case in our patient. These are the patients who require therapy to prevent the side effects of the glucocorticoid from becoming more dangerous than the eye disease. In particular, bisphosphonate therapy should be initiated early in patients receiving high-dose prednisone therapy (7) . Intravenous glucocorticoid pulse therapy has also been used. Radiotherapy kills retro orbital T cells. The value of orbital radiation is controversial. In two trials it was more effective than glucocorticoid therapy(8,9) and in a third it was better than shamirradiation 0 ^. However, in a fourth trial in which one eye was


55

Yassin M.A.,

Frozen eye what could it be?

et.al.

not treated, radiation was no better than no t r e a t m e n t 0 0 . It is

are three major indications for orbital decompression in Graves'

m o s t o f t e n u s e d in p a t i e n t s in w h o m g l u c o c o r t i c o i d s are

ophthalmopathy03^

contraindicated, poorly tolerated, or cannot b e discontinued

irradiation fails to halt progression of the ophthalmopathy.

because of exacerbations of ophthalmopathy.Trials of combined

If loss of vision is threatened either by ulceration or infection

If g l u c o c o r t i c o i d t h e r a p y or o r b i t a l

radiation and glucocorticoid therapy have suggested that the

of the cornea or by changes in the retina or optic nerve. For

combination m a y be more effective than either alone ( 1 2 ) . There

cosmetic correction of severe proptosis.

References: 1. Burch, HB, Wartofsky, L. Graves' ophthalmopathy: current concepts regarding pathogenesis and management. Endocr Rev 1993; 14: 747. 2. Wiersinga, WM. Immunosuppressive treatment of Graves' ophthalmopathy. Thyroid 1992; 2: 229. 3. Dickinson, AJ, Vaidya, B, Miller, M, et al. Double-Blind, Placebo-Controlled Trial of Octreotide Long-Acting Repeatable (LAR) in Thyroid-Associated Ophthalmopathy. J Clin Endocrinal Metab 2004; 89: 5910. 4. Wemeau, JL, Caron, P, Beckers, A, et al. Octreotide (longacting release formulation) treatment in patients with graves' orbitopathy: clinical results of a four-month, randomized, placebo-controlled, double-blind study. J Clin Endocrinol Metab 2005; 90: 841. 5. Tallstedt, L, Lundell, G, Torring, O, et al. Occurrence of ophthalmopathy after treatment for Graves' hyperthyroidism. N Engl J Med 1992; 326: 1733. 6. Dickinson, AJ, Vaidya, B, Miller, M, et al. Double Blind, Placebo-Controlled Trial of Octreotide Long-Acting Repeatable (LAR) in Thyroid-Associated Ophthalmopathy. J Clin Endocrinol Metab 2004; 89: 5910.

7. Eastell, R, Devogelaer, JP, Peel, NF, et al. Prevention of bone loss with risedronate in glucocorticoid-treated rheumatoid arthritis patients. Osteoporos Int 2000; 11: 331. 8. Prummel, MF, Wiersinga, WM. Medical management of Graves'ophthalmopathy. Thyroid 1995; 5: 231. 9. Prummel, MF, Mourits, MP, Blank, L, et al. Randomized double blind controlled of prednisolone versus radiotherapy in Graves'ophthalmopathy. Lancet 1993; 342: 949. 10. Mourits, MP, van Kempen-Harteveld, ML, Garcia, and MB, et al. Radiotherapy for Graves' orbitopathy: randomised placebo-controlled study. Lancet 2000; 355: 1505. 11. Gorman, CA, Garrity, J A, Fatourechi, V, et al. A prospective, randomized, double blind, placebo-controlled study of orbital radiotherapy for Graves' ophthalmopathy. Ophthalmology 2001; 108: 1523. 12. Bartalena, L, Marcocci, C, Chiovato, L, et al. Orbital cobalt irradiation combined with systemic corticosteroids for Graves' ophthalmopathy: comparison with systemic corticosteroids alone. J Clin Endocrinol Metab 1983; 56: 1139. 13. Lyons, CJ, Rootman, J. Orbital decompression for disfiguring exophthalmos in thyroid orbitopathy. Ophthalmology 1994; 101: 223.

Call for publication in the First Emergency Medical Journal in the Middle East...

Original studies, case reports, and other communications in the emergency medicine and emergency surgery. For more information, please contact: The Editor-in-Chief, Middle East Journal of Emergency Medicine Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar Fax: (+974) 4392179; Tel. Nos.: (+974) 4392050/4392177 E-mail: [email protected] Website: www.hmc.org.qa/mejem

56
