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MAIN Sir, The presentation of thyroid eye disease is usually closely temporally related to the diagnosis of thyrotoxicosis. A very small percentage of patients with thyroid eye disease
develop sight-threatening disease due to compressive optic neuropathy. We report a case of severe thyroid eye disease with sight loss presenting 14 years after the diagnosis of
thyrotoxicosis. CASE REPORT A 57-year-old Caucasian female, who smoked five cigarettes a day, presented with a severe reduction in right visual acuity associated with proptosis. Fourteen
years previously, she had been treated with carbimazole and radioactive iodine for thyrotoxicosis. Subsequent hypothyroidism was treated with thyroxine. She had controlled hypertension and
seasonal episodes of bronchitis. There was a strong family history of thyroid problems with her mother and sister being on replacement thyroxine treatment, although the underlying thyroid
condition was unknown. Her ophthalmological symptoms had started approximately 9 months previously, shortly after the death of her father, initially with right lower lid swelling followed 3
months later by horizontal diplopia and conjunctival injection. She then developed blurred right vision and she was referred to the local Eye Department where it was felt unlikely that she
had thyroid eye disease in view of the long interval between her thyrotoxicosis and the onset of her eye problems. Three months later, she developed right proptosis with reduced vision and
following a CT scan was started on 100 μg prednisolone reducing to 10 μg over 3 weeks. The left vision deteriorated, and the steroid dosage was increased to a maintenance dose of 25 μg. She
was referred to our service for further opinion, and presented with a corrected Snellen visual acuity of 2/60 in her right and 6/12 in her left eye. She was unable to identify any of the
Ishihara test plates with her right eye and scored only 10 out of 17 with the left eye. There was right proptosis with upper and lower lid retraction and restricted extraocular movements
worse on the right side (Figure 1). The anterior segments were normal. Intraocular pressures increased significantly on upgaze. She had a right afferent pupillary defect, nasal swelling of
the right optic disc, absent spontaneous venous pulsation of both optic discs, and extensive arteriovenous nipping. Electrodiagnostic tests were reported as consistent with a diagnosis of a
right optic neuropathy. The MRI scan showed compression of the right optic nerve at the orbital apex, with bilateral proptosis more marked on the right side due to asymmetrical enlargement
of the extraocular muscles (Figure 2). Blood results showed a neutrophilia, normal haemoglobin and platelet concentrations with normal blood chemistry. Thyroid function tests had recently
shown an elevated free thyroxine concentration with an associated low TSH level and her thyroxine had been reduced to 75 _μ_g. The patient was started on a 2-day course of intravenous
methylprednisolone and a three-wall orbital decompression was advised. COMMENT Although the majority of patients with thyroid eye disease usually present very soon after the diagnosis of
thyrotoxicosis, it is important to remember that, rarely, late presentation can occur. Anecdotally, it has been suggested that these patients can often identify a stressful and identifiable
precipitating factor. Such an event was noted in our patient. Only 5% of patients have the complete spectrum of clinical features with eyelid retraction, exophthalmos, extraocular muscle
involvement, optic nerve dysfunction and hyperthyroidism.1 The close temporal relationship of the diagnosis of Graves' disease and thyroid eye disease is well known and it has been
found in 81% of patients to develop in the 18 months prior to and after the diagnosis of thyroid dysfunction.2 It has been reported that 69.5% of patients requiring decompression surgery
develop thyroid eye disease in the year before or after diagnosis.3 Ophthalmopathy is rarely diagnosed more than 6 months before the diagnosis of thyrotoxicosis.4 In one report no patient
developed eye problems more than 4 years after the diagnosis of hyperthyroidism.4 It has been suggested that thyroid eye disease develops at a time when thyroid autoimmunity also exists,
thus strongly suggesting a common factor in the pathogenesis of thyroidal and ocular expressions of Graves' disease.5 This patient is a rare case of severe sight-threatening thyroid eye
disease presenting 14 years after diagnosis of thyrotoxicosis. While the vast majority of patients who develop thyroid eye disease will do so in an 18-month period before or after the time
of systemic diagnosis, it is important to be aware that sight-threatening thyroid eye disease may present many years after the initial diagnosis and treatment of thyrotoxicosis. Prompt
diagnosis and treatment of this condition is likely to preserve sight. REFERENCES * Bartley GB, Fatourechi B, Kadrmas EF, Jacobsen SJ, Ilstrup DM, Garrity JA et al. Clinical features of
Graves' ophthalmopathy in an incidence cohort. _Am J Ophthalmopathy_ 1996; 121(3): 284–290. Article CAS Google Scholar * Gorman CA . Temporal relationship between onset of
Graves' ophthalmopathy and diagnosis of thyrotoxicosis. _Mayo Clin Proc_ 1983; 58: 515–519. CAS PubMed Google Scholar * Garrity JA, Fatourechi V, Bergstralh EJ, Bartley GB, Beatty
CW, DeSanto LW et al. Results of transantral orbital decompression in 428 patients with severe Graves' ophthalmopathy. _Am J Ophthalmol_ 1993; 116: 533–547. Article CAS Google Scholar
* Bartley GB, Fatourechi V, Kadrmas EF, Jacobsen SJ, Ilstrup DM, Garrity JA et al. The chronology of Graves' ophthalmopathy in an incidence cohort. _Am J Ophthalmol_ 1996; 121:
426–434. Article CAS Google Scholar * Wiersinga WM, Smit T, van der Gaag R, Koornneef L . Temporal relationship between onset of Graves' ophthalmopathy and onset of thyroidal
Graves' disease. _J Endocrinol Invest_ 1988; 11(8): 615–619. Article CAS Google Scholar Download references AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Moorfields Eye Hospital,
City Road, London, EC1V 2PD, UK B Manzouri, A Bates, G E Rose & G G W Adams Authors * B Manzouri View author publications You can also search for this author inPubMed Google Scholar * A
Bates View author publications You can also search for this author inPubMed Google Scholar * G E Rose View author publications You can also search for this author inPubMed Google Scholar * G
G W Adams View author publications You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to B Manzouri. RIGHTS AND PERMISSIONS Reprints and
permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Manzouri, B., Bates, A., Rose, G. _et al._ Late presentation of severe thyroid eye disease. _Eye_ 18, 1021–1023 (2004).
https://doi.org/10.1038/sj.eye.6701396 Download citation * Published: 16 April 2004 * Issue Date: 01 October 2004 * DOI: https://doi.org/10.1038/sj.eye.6701396 SHARE THIS ARTICLE Anyone you
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