Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Graves' Ophthalmopathy Although the frequency of ophthalmopathy related to thyroid disease is difficult to estimate, 80% of those patients with bilateral exophthalmos will have Graves' disease. The eye findings associated with Graves' disease can occur before, during, or long after the thyroid disease has been discovered or treated. While the orbitopathy is most commonly associated with hyperthyroid states, it can be seen in both euthyroid and even hypothyroid patients. Graves' ophthalmopathy, or endocrine ophthalmopathy is an immunologically-mediated inflammatory process associated with one of three thyroid disorders: 1) Graves' hyperthyroidism (80%); 2) Hashimoto's thyroiditis (10% to 15%); or 3) unclassified thyroid immunologic abnormality (5%). The link between thyroid disease and the orbital pathology involves both humoral and cellular immune reactions. However, the exact mechanism remains unknown. Clinically, the classic signs of hyperthyroidism can be found in the presence of proptosis. Other eye findings include: lid lag or retraction; periorbital edema; extra-ocular muscle involvement causing diplopia; and, the most feared complication, visual loss. The evaluation of these patients begins with a thorough endocrine work-up in conjunction with a complete ophthalmic examination. Helpful radiologic studies include orbital ultrasound or magnetic resonance imaging (MRI). However, the study of choice is orbital computerized tomography (CT scanning). Perhaps the most difficult aspect in the care of these patients is the fact that there is no stepwise progression of disease. Each patient presents with a unique constellation of orbital findings, and no finding or set of findings can predict which patient will develop visual impairment. Management must therefore, be individualized and begins with control of the concomitant thyroid disease. In the majority of cases, the ophthalmic complications can be managed with local measures only. In approximately 10% of cases further therapy is required, such as systemic glucocorticoids or orbital radiotherapy. Surgical orbital decompression is reserved for those patients in which vision is threatened, where there is the presence of severe cosmetic deformity, or failure of medical management. Multiple surgical approaches are available for orbital decompression, with the combined transorbital-transnasal method used most frequently at Baylor College of Medicine. This method, as well as the surgical experience at our institution over the past five years, was discussed in detail. Case Presentation A 62-year-old female smoker was diagnosed with hyperthyroidism in May by an outlying physician. The patient was treated with radioactive iodine (I 131) for thyroid ablation in June. In October, she developed bilateral periorbital edema, epiphora, and diplopia. The patient was then referred to the Baylor Ophthalmology Department. Initial evaluation revealed her acuity to be 20/20 bilaterally with normal color vision. Computerized visual field examinations were normal. There was minimal proptosis and slit lamp examination reveled moderate conjunctival hyperemia and chemosis. There was marked limitation of elevation in both eyes. An MRI revealed enlarged superior, medial, and inferior rectus muscles bilaterally as well as increased retrobulbar fat. The patient was managed conservatively and with close follow-up. Presenting one year later with a foreign-body sensation in her right eye, L.W. was noted to have a 4 mm bilateral increase in proptosis. Her vision in the right eye had decreased to 20/50. She had also lost red color perception in the affected eye. A combined right transconjunctival orbitotomy and transnasal complete ethmoidectomy was then performed by the Ophthalmology and Otolaryngology services. Two weeks postoperatively, the patient's vision had improved to 20/25 in the right eye and red color perception had returned. However, there was no change in her diplopia. Bibliography Bahn RS, Garrity JA, Bartley GB, Gorman CA. Diagnostic evaluation of Graves' ophthalmopathy. Endocrinol Metab Clin North Am 1988;17:527-545. Bleeker GM. Changes in the orbital tissues and muscles dysthyroid ophthalmopathy. Eye 1988;2:193-197. Calcaterra TC, Thompson JW. Antral-ethmoidal decompression of the orbit in Graves' disease: ten-year experience. Laryngoscope 1980;90:1941-1949. Carter KD, Frueh BR, Hessburg TP, Musch DC. Long-term efficacy of orbital decompression for compressive optic neuropathy of Graves' eye disease. Ophthalmology 1991;98:1435-1442. Fells P. Systemic management of dysthyroid ophthalmopathy. Eye 1988;2:198-200. Girod DA, Orcutt JC, Cummings CW. Orbital decompression for preservation of vision in Graves' ophthalmopathy. Arch Otolaryngol Head Neck Surg 1993;119:229-233. Guy JR, Fagien S, Donovan JP, Rubin ML. Methylprednisolone pulse therapy in severe dysthyroid optic neuropathy. Ophthalmology 1989;96:1048-1053. Hiromatsu Y, Fukazawa H, Wall JR. Cytotoxic mechanisms in autoimmune thyroid disorders and thyroid-associated ophthalmopathy. Endocrinol Metab Clin North Am 1987;16:269-86. Jones B. Caleb Hillier Parry. West Engl Med J 1991;106:101-102. Kadlubowski M, Irvine WJ, Rowland AC. Anti-muscle antibodies in Graves' ophthalmopathy. J Clin Lab Immunol 1987;24:105-111. Kahaly G, Moncayo R, Stover C, Beyer J. Relationship of eye muscle antibodies with HLA phenotypes and thyroid-stimulating immunoglobulins in endocrine orbitopathy. Res Exp Med 1991;191:147-144. Kahaly G, Stover C, Beyer J, Otto E. In vitro synthesis of glycosaminoglycans in endocrine ophthalmopathy. Acta Endocrinol 1992;127:397-402. Kazin M, Trokel S, Moore S. Treatment of acute Graves orbitopathy. Ophthalmology 1991;98:1443-1448. Kennedy DW, Goodstein ML, Miller NR, Zinreich SJ. Endoscopic transnasal orbital decompression. Arch Otolaryngol Head Neck Surg 1990;116:275-282. Lloyd WC III, Leone CR Jr. Supervoltage orbital radiotherapy in 36 cases of Graves' disease. Am J Ophthalmol 1992;113:374-380. Mazzaferri EL, Carusco DR. Disorders of the thyroid gland. In: Cummings DW, Fredrickson JM, editors. Otolaryngology - head and neck surgery, Vol 3, 2nd ed. St. Louis: Mosby, 1993:2414-2435. McDougall IR. Graves' disease: current concepts. Med Clin North Am 1991;75:79-95. Nunery WR, Ophthalmic Graves' disease: a dual theory of pathogenesis. Ophthalmol Clin North Am 1991;4:73-87. Olivari N. Transpalpebral decompression of endocrine ophthalmopathy (Graves' Disease) by removal of intraorbital fat: experience with 147 operations over 5 years. Plast Reconstr Surg 2991;87:627-641. Palmer D, Greenberg P, Cornell P, Parker RG. Radiation therapy for Graves' ophthalmopathy: a retrospective analysis. Int J Rad Oncol Biol Phys 1987;13:1815-1820. Patrinely JR, Osborn AG, Anderson RL, Whiting AS. Computed tomographic features of nonthyroid extraocular muscle enlargement. Ophthalmology 1989;96:1038-1047. Pearl RM, Vistnes L, Troxel S. Treatment of exophthalmos. Plast Reconstr Surg 1991;87:236-244. Petersen IA, Kriss JP, McDougall IR, Donaldson WW. Prognostic factors in the radiotherapy of Graves' ophthalmopathy. Int J Rad Oncol Biol Phys 1990;19:259-264. Roncevic R, Jackson IT. Surgical treatment of thyrotoxic exophthalmos. Plast Reconstr Surg 1989;84:754-760. Salvi M, Zhang Z-G, Haegert D, Woo M, Liberman A, Cadarso L, et al. Patients with endocrine ophthalmopathy not associated with overt thyroid disease have multiple thyroid immunological abnormalities. J Clin Endocrinol Metab 1990;70:89-94. Sandler HM, Rubenstein JH, Fowble BL, Sergott RC, Savino PJ, Bosley RM. Results of radiotherapy for thyroid ophthalmopathy. J Rad Oncol Biol Phys 1989;17:823-827. Stover C, Otto E, Beyer J, Kahaly G. Humoral immunity and retrobulbar fibroblasts in endocrine ophthalmopathy. Acta Endocrinol 1992;126:394-398. Taylor S. Graves of Graves' Disease, 1796-1853. J R Coll Physicians Lond 1986;20:298-300. Tyutyunikov A, Raikow RB, Kennerdell JS, Kazim M, Dalbow MH, Scalise D. Re-examination of peripheral blood T cell subsets in dysthyroid orbitopathy. Invest Ophthalmol Vis Sci 1992;33:2299-2303. Wall JR, How J, editors. Graves' ophthalmopathy. Boston: Blackwell Scientific, 1990. Warren JD, Spector JG, Burde R. Long-term follow-up and recent observations on 305 cases of orbital decompression for dysthyroid orbitopathy. Laryngoscope 1989;99:35-40. Weisman RA, Svaino PJ. Management of endocrine orbitopathy. Otolaryngol Clin North Am 1988;21:93-102. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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