2018•BMC OphthalmologyOpen access

Extraocular muscle repositioning as the last therapeutic option for a patient with a severe course of Graves’ Ophthalmopathy: a case report

Andréa Rau, Matthias Klöpfer, Niklas Rommel, Mechthild Rau-Fornefeld, Andreas Kolk

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Abstract

BACKGROUND: Graves' disease is a common autoimmune inflammatory condition of the thyroid. About one in four of affected patients also develop orbital symptoms like exophthalmos, proptosis and diplopia - called Graves' Ophthalmopathy. Not all patients respond well to the standard therapy of systemic glucocorticoid administration. The inflammatory swelling of the intraorbital muscles can lead to pressure-induced damage of the optic nerve. Orbital decompression surgery is a therapeutic option for these patients with varying success. Other symptoms like the extreme malposition of the ocular globe are poorly addressed by decompression surgery and demand for different therapeutic approaches. CASE PRESENTATION: Presented is the case of a 46-year old patient with an acute exacerbation of Graves' ophthalmopathy. Clinically apparent was a convergent strabismus fixus with severe hypotropia of both eyes. The patient suffered from attacks of heavy retrobulbar pain and eyesight deteriorated dramatically. Since neither systemic glucocorticoid therapy nor orbital decompression surgery had helped to halt the progress of the disease, a decision was made in favour of the surgical release and repositioning of the inferior and medial rectus muscle as a final therapeutic option. Surgery of both eyes was performed consecutively within one week. Detailed descriptions and illustrations of the surgical steps and treatment outcome are provided supplemented by a discussion of the current literature. CONCLUSIONS: Graves' Ophthalmopathy is a variant and therapeutically challenging disease. Exceptional courses of the disease call for therapeutic approaches off the beaten track. Surgical extraocular muscle repositioning, which has not been described before in the context of Graves' Ophthalmopathy, proved to be effective in improving the patient's eyesight and quality of life. Furthermore, we regard the measurement of extraocular muscle volume as a valuable method to monitor the course of Graves' Ophthalmopathy.

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BACKGROUND: Graves' disease is a common autoimmune inflammatory condition of the thyroid. About one in four of affected patients also develop orbital symptoms like exophthalmos, proptosis and diplopia - called Graves' Ophthalmopathy. Not all patients respond well to the standard therapy of systemic glucocorticoid administration. The inflammatory swelling of the intraorbital muscles can lead to pressure-induced damage of the optic nerve. Orbital decompression surgery is a therapeutic option for these patients with varying success. Other symptoms like the extreme malposition of the ocular globe are poorly addressed by decompression surgery and demand for different therapeutic approaches. CASE PRESENTATION: Presented is the case of a 46-year old patient with an acute exacerbation of Graves' ophthalmopathy. Clinically apparent was a convergent strabismus fixus with severe hypotropia of both eyes. The patient suffered from attacks of heavy retrobulbar pain and eyesight deteriorated dramatically. Since neither systemic glucocorticoid therapy nor orbital decompression surgery had helped to halt the progress of the disease, a decision was made in favour of the surgical release and repositioning of the inferior and medial rectus muscle as a final therapeutic option. Surgery of both eyes was performed consecutively within one week. Detailed descriptions and illustrations of the surgical steps and treatment outcome are provided supplemented by a discussion of the current literature. CONCLUSIONS: Graves' Ophthalmopathy is a variant and therapeutically challenging disease. Exceptional courses of the disease call for therapeutic approaches off the beaten track. Surgical extraocular muscle repositioning, which has not been described before in the context of Graves' Ophthalmopathy, proved to be effective in improving the patient's eyesight and quality of life. Furthermore, we regard the measurement of extraocular muscle volume as a valuable method to monitor the course of Graves' Ophthalmopathy.

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Available abstract

BACKGROUND: Graves' disease is a common autoimmune inflammatory condition of the thyroid. About one in four of affected patients also develop orbital symptoms like exophthalmos, proptosis and diplopia - called Graves' Ophthalmopathy. Not all patients respond well to the standard therapy of systemic glucocorticoid administration. The inflammatory swelling of the intraorbital muscles can lead to pressure-induced damage of the optic nerve. Orbital decompression surgery is a therapeutic option for these patients with varying success. Other symptoms like the extreme malposition of the ocular globe are poorly addressed by decompression surgery and demand for different therapeutic approaches. CASE PRESENTATION: Presented is the case of a 46-year old patient with an acute exacerbation of Graves' ophthalmopathy. Clinically apparent was a convergent strabismus fixus with severe hypotropia of both eyes. The patient suffered from attacks of heavy retrobulbar pain and eyesight deteriorated dramatically. Since neither systemic glucocorticoid therapy nor orbital decompression surgery had helped to halt the progress of the disease, a decision was made in favour of the surgical release and repositioning of the inferior and medial rectus muscle as a final therapeutic option. Surgery of both eyes was performed consecutively within one week. Detailed descriptions and illustrations of the surgical steps and treatment outcome are provided supplemented by a discussion of the current literature. CONCLUSIONS: Graves' Ophthalmopathy is a variant and therapeutically challenging disease. Exceptional courses of the disease call for therapeutic approaches off the beaten track. Surgical extraocular muscle repositioning, which has not been described before in the context of Graves' Ophthalmopathy, proved to be effective in improving the patient's eyesight and quality of life. Furthermore, we regard the measurement of extraocular muscle volume as a valuable method to monitor the course of Graves' Ophthalmopathy.

Key concepts: Medicine, Diplopia, Extraocular muscles, Exophthalmos, Graves' disease, Graves' ophthalmopathy, Surgery, Strabismus surgery

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