Orbit, 2014; 33(1): 72–74 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2013.851252
C ASE REPORT
No Light Perception Vision From Compressive Thyroid Orbitopathy Dana M. Hornbeak1, Madhura A. Tamhankar1, and Lauren A. Eckstein2 1
Department of Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, USA and 2Kaiser Permanente Vandever Medical Offices, San Diego, California, USA
ABSTRACT Thyroid eye disease (TED) is an autoimmune inflammatory disorder that affects the extraocular soft tissues and causes eyelid retraction, proptosis and restrictive extraocular myopathy. Compressive optic neuropathy from extraocular muscle enlargement occurs in less than 5% of patients, in the majority of whom it develops within 18 months of the diagnosis of hyperthyroidism. Vision loss from compressive optic neuropathy in patients with thyroid eye disease is usually bilateral and insidious in onset and progression and is associated with diplopia and elevated intraocular pressure. To our knowledge, there have been no reported cases of acute vision loss to the level of no light perception secondary to thyroid orbitopathy. The authors report a 66-year-old Caucasian male with history of long-standing thyroid eye disease and massive proptosis who progressed from mild compressive symptoms to no light perception within days, despite being clinically stable for over 10 years. Keywords: Optic neuropathy, proptosis, thyroid orbitopathy, vision loss
INTRODUCTION
vision and visual fields in each eye. The patient noted blurry vision in the left eye for 5 days prior to complete loss of vision to NLP. He denied eye pain, headache, jaw claudication, weight loss, or scalp tenderness. He was on levothyroxine and was euthyroid at the time of presentation. He had quit smoking 2.5 years prior. Ophthalmic examination revealed a visual acuity of 20/25 in the right eye and NLP in the left eye, with a left relative afferent pupillary defect. External examination revealed bilateral massive proptosis (30 mm in the right eye and 35 mm in the left eye with Hertel’s exophthalmometry readings), and severe conjunctival injection (Figure 1). Intraocular pressures were 24 mm in each eye. Dilated fundus examination showed normal optic nerves bilaterally with the presence of few horizontal choroidal folds in the left macula. There were no signs of retinal detachment or vascular abnormalities. Humphrey visual field in the right eye showed peripheral constriction.
20 14
Compressive optic neuropathy is an uncommon vision threatening complication of thyroid eye disease, causing vision loss, which is usually insidious, bilateral, and slowly progressive. We report a patient who rapidly progressed to no light perception (NLP) vision despite being clinically stable for over 10 years.
Case Report A 66-year-old Caucasian male presented with acute vision loss in his left eye that he noticed upon awakening. He had a history of hyperthyroid Graves disease 10 years prior, which had been treated with radioactive iodine. For several years he had bilateral proptosis, dry eyes, and restrictive myopathy, which were stable. His prior ophthalmic examination 3 months prior had revealed normal
Received 16 December 2012; Revised 2 May 2013; Accepted 30 September 2013; Published online 7 November 2013 Correspondence: Dr. Madhura Tamhankar, Scheie Eye Institute, 51N, 39th Street, Philadelphia, PA 19104, USA. E-mail: [email protected]
72
Thyroid Associated Ophthalmopathy The patient was treated with high-dose intravenous methylprednisolone due to concern for compressive optic neuropathy. Magnetic resonance imaging of the brain and orbits showed massive enlargement of the extraocular muscles bilaterally, left greater than right, along with apical compression and stretching of the optic nerves (Figure 2). Magnetic resonance angiography (MRA) demonstrated normal orbital flow through the ophthalmic artery, with no evidence of orbital vascular abnormalities, dural carotid cavernous fistula, cavernous sinus thrombosis, or pituitary apoplexy. Laboratory studies showed normal CBC, ESR of 19, C-reactive protein of less than 0.5, and normal thyroid function studies. The patient underwent emergent three-wall orbital decompression of the left eye, followed by orbital decompression in the right eye as well. Vision recovered completely in the right eye and improved slightly in the left eye to counting fingers at 3 feet postoperatively.
DISCUSSION Compressive optic neuropathy is rare vision threatening complication of Graves orbitopathy that usually occurs during the acute phase of the disease although rarely, disease reactivation can occur several years later and despite euthyroid clinical status.1–5 It is
FIGURE 1. External appearance of the eyes showing bilateral massive proptosis and conjunctival injection.
73
heralded by vision loss that can be bilateral although is asymmetric and insidiously progressive. The degree of proptosis often does not correlate with optic nerve compression. The pathogenesis of optic neuropathy typically involves mechanical compression of the optic nerve, which in turn is due to extraocular muscle hypertrophy at the orbital apex and/or fibrosis of the annulus of Zinn.6 Other contributing mechanisms may include venous stasis and optic nerve ischemia. In severe cases of extraocular tissue enlargement, a compartment syndrome may develop within the bony orbit and orbital septum, producing compressive optic neuropathy.7 Rarely optic neuropathy can result from optic nerve stretching rather than compression.5,8 In our patient we believe the massive extraocular muscle enlargement contributed to optic nerve compression, although the rapid progression to no light perception vision is unusual. Due to the severity of vision loss and extraocular muscle hypertrophy, optic nerve ischemia was also suspected. However, MRA revealed normal orbital blood flow, and ophthalmoscopically the optic nerve head and retina appeared normal. It is also possible that the severe optic nerve stretching could have led to vision loss.5,8 Radiographically our patient did demonstrate optic nerve compression and stretching prior to presentation, although he had normal visual function and visual fields. The acute vision loss experienced by our patient can be explained by severe orbital venous congestion and resultant decreased perfusion pressure, leading to optic nerve ischemia. In summary, we report a unique case of a patient with stable thyroid orbitopathy who rapidly progressed to no light perception despite prior normal visual function for several years. Although vision loss from compressive optic neuropathy has been reported we did not come across any cases that presented with no light perception vision. In patients who demonstrate radiographic evidence of optic nerve compression despite normal visual function, one needs to maintain a high index of suspicion for the possibility of worsening of vision due to compressive optic
FIGURE 2. Coronal T1-weighted, gadolinium contrast enhanced MRI of the orbits showing massively thickened extra-ocular muscles in both orbits. !
2014 Informa Healthcare USA, Inc.
74 D. M. Hornbeak et al. neuropathy and orbital decompression should be considered preemptively in such patients.9,10
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Bahn RS. Graves ophthalmopathy. N Engl J Med 2010;362: 726–738. 2. Wiersinga WM, Bartalena L. Epidemiology and prevention of Graves’ophthalmopathy. Thyroid 2002;12: 855–860. 3. Wiersinga WM, Smit T, van der Gaag R, Koornneef L. Temporal relationship between onset of Graves’
4.
5. 6.
7.
8.
9.
10.
ophthalmopathy and onset of thyroidal Graves’ disease. J Endocrinol Invest 1988;11:615–619. Perros P, Crombie AL, Kendall-Taylor P. Natural history of thyroid associated ophthalmopathy. Clin Endocrinol 1995; 42:45–50. Robinson D, Wilcsek G, Sacks R. Orbit and orbital apex. Otolaryngol Clin North Am 2011;44:903–922. Kazim M, Trokel SL, Acaroglu G, Elliott A. Reversal of dysthyroid optic neuropathy following orbital fat decompression. Br J Ophthalmol 2000;84:600–605. Stan MN, Garrity JA, Bahn RS. The evaluation and treatment of Graves ophthalmopathy. Med Clin North Am 2012;96:311–328. Nugent RA, Belkin RI, Neigel JM, Rootman J, et al. Graves orbitopathy: correlation of CT and clinical findings. Radiology 1990;177:675–682. Chan LL, Tan HE, Fook-Chond S, Teo TH, et al. Graves ophthalmopathy: the bony orbit in optic neuropathy, its apical angular capacity, and impact on prediction of risk. Am J Neuroradiol 2009;30:597–602. Lee HB, Rodgers IR, Woog JJ. Evaluation and management of Graves’ orbitopathy. Otolaryngol Clin North Am 2006;39: 923–942.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
C ASE REPORT
No Light Perception Vision From Compressive Thyroid Orbitopathy Dana M. Hornbeak1, Madhura A. Tamhankar1, and Lauren A. Eckstein2 1
Department of Ophthalmology, Scheie Eye Institute, Philadelphia, Pennsylvania, USA and 2Kaiser Permanente Vandever Medical Offices, San Diego, California, USA
ABSTRACT Thyroid eye disease (TED) is an autoimmune inflammatory disorder that affects the extraocular soft tissues and causes eyelid retraction, proptosis and restrictive extraocular myopathy. Compressive optic neuropathy from extraocular muscle enlargement occurs in less than 5% of patients, in the majority of whom it develops within 18 months of the diagnosis of hyperthyroidism. Vision loss from compressive optic neuropathy in patients with thyroid eye disease is usually bilateral and insidious in onset and progression and is associated with diplopia and elevated intraocular pressure. To our knowledge, there have been no reported cases of acute vision loss to the level of no light perception secondary to thyroid orbitopathy. The authors report a 66-year-old Caucasian male with history of long-standing thyroid eye disease and massive proptosis who progressed from mild compressive symptoms to no light perception within days, despite being clinically stable for over 10 years. Keywords: Optic neuropathy, proptosis, thyroid orbitopathy, vision loss
INTRODUCTION
vision and visual fields in each eye. The patient noted blurry vision in the left eye for 5 days prior to complete loss of vision to NLP. He denied eye pain, headache, jaw claudication, weight loss, or scalp tenderness. He was on levothyroxine and was euthyroid at the time of presentation. He had quit smoking 2.5 years prior. Ophthalmic examination revealed a visual acuity of 20/25 in the right eye and NLP in the left eye, with a left relative afferent pupillary defect. External examination revealed bilateral massive proptosis (30 mm in the right eye and 35 mm in the left eye with Hertel’s exophthalmometry readings), and severe conjunctival injection (Figure 1). Intraocular pressures were 24 mm in each eye. Dilated fundus examination showed normal optic nerves bilaterally with the presence of few horizontal choroidal folds in the left macula. There were no signs of retinal detachment or vascular abnormalities. Humphrey visual field in the right eye showed peripheral constriction.
20 14
Compressive optic neuropathy is an uncommon vision threatening complication of thyroid eye disease, causing vision loss, which is usually insidious, bilateral, and slowly progressive. We report a patient who rapidly progressed to no light perception (NLP) vision despite being clinically stable for over 10 years.
Case Report A 66-year-old Caucasian male presented with acute vision loss in his left eye that he noticed upon awakening. He had a history of hyperthyroid Graves disease 10 years prior, which had been treated with radioactive iodine. For several years he had bilateral proptosis, dry eyes, and restrictive myopathy, which were stable. His prior ophthalmic examination 3 months prior had revealed normal
Received 16 December 2012; Revised 2 May 2013; Accepted 30 September 2013; Published online 7 November 2013 Correspondence: Dr. Madhura Tamhankar, Scheie Eye Institute, 51N, 39th Street, Philadelphia, PA 19104, USA. E-mail: [email protected]
72
Thyroid Associated Ophthalmopathy The patient was treated with high-dose intravenous methylprednisolone due to concern for compressive optic neuropathy. Magnetic resonance imaging of the brain and orbits showed massive enlargement of the extraocular muscles bilaterally, left greater than right, along with apical compression and stretching of the optic nerves (Figure 2). Magnetic resonance angiography (MRA) demonstrated normal orbital flow through the ophthalmic artery, with no evidence of orbital vascular abnormalities, dural carotid cavernous fistula, cavernous sinus thrombosis, or pituitary apoplexy. Laboratory studies showed normal CBC, ESR of 19, C-reactive protein of less than 0.5, and normal thyroid function studies. The patient underwent emergent three-wall orbital decompression of the left eye, followed by orbital decompression in the right eye as well. Vision recovered completely in the right eye and improved slightly in the left eye to counting fingers at 3 feet postoperatively.
DISCUSSION Compressive optic neuropathy is rare vision threatening complication of Graves orbitopathy that usually occurs during the acute phase of the disease although rarely, disease reactivation can occur several years later and despite euthyroid clinical status.1–5 It is
FIGURE 1. External appearance of the eyes showing bilateral massive proptosis and conjunctival injection.
73
heralded by vision loss that can be bilateral although is asymmetric and insidiously progressive. The degree of proptosis often does not correlate with optic nerve compression. The pathogenesis of optic neuropathy typically involves mechanical compression of the optic nerve, which in turn is due to extraocular muscle hypertrophy at the orbital apex and/or fibrosis of the annulus of Zinn.6 Other contributing mechanisms may include venous stasis and optic nerve ischemia. In severe cases of extraocular tissue enlargement, a compartment syndrome may develop within the bony orbit and orbital septum, producing compressive optic neuropathy.7 Rarely optic neuropathy can result from optic nerve stretching rather than compression.5,8 In our patient we believe the massive extraocular muscle enlargement contributed to optic nerve compression, although the rapid progression to no light perception vision is unusual. Due to the severity of vision loss and extraocular muscle hypertrophy, optic nerve ischemia was also suspected. However, MRA revealed normal orbital blood flow, and ophthalmoscopically the optic nerve head and retina appeared normal. It is also possible that the severe optic nerve stretching could have led to vision loss.5,8 Radiographically our patient did demonstrate optic nerve compression and stretching prior to presentation, although he had normal visual function and visual fields. The acute vision loss experienced by our patient can be explained by severe orbital venous congestion and resultant decreased perfusion pressure, leading to optic nerve ischemia. In summary, we report a unique case of a patient with stable thyroid orbitopathy who rapidly progressed to no light perception despite prior normal visual function for several years. Although vision loss from compressive optic neuropathy has been reported we did not come across any cases that presented with no light perception vision. In patients who demonstrate radiographic evidence of optic nerve compression despite normal visual function, one needs to maintain a high index of suspicion for the possibility of worsening of vision due to compressive optic
FIGURE 2. Coronal T1-weighted, gadolinium contrast enhanced MRI of the orbits showing massively thickened extra-ocular muscles in both orbits. !
2014 Informa Healthcare USA, Inc.
74 D. M. Hornbeak et al. neuropathy and orbital decompression should be considered preemptively in such patients.9,10
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Bahn RS. Graves ophthalmopathy. N Engl J Med 2010;362: 726–738. 2. Wiersinga WM, Bartalena L. Epidemiology and prevention of Graves’ophthalmopathy. Thyroid 2002;12: 855–860. 3. Wiersinga WM, Smit T, van der Gaag R, Koornneef L. Temporal relationship between onset of Graves’
4.
5. 6.
7.
8.
9.
10.
ophthalmopathy and onset of thyroidal Graves’ disease. J Endocrinol Invest 1988;11:615–619. Perros P, Crombie AL, Kendall-Taylor P. Natural history of thyroid associated ophthalmopathy. Clin Endocrinol 1995; 42:45–50. Robinson D, Wilcsek G, Sacks R. Orbit and orbital apex. Otolaryngol Clin North Am 2011;44:903–922. Kazim M, Trokel SL, Acaroglu G, Elliott A. Reversal of dysthyroid optic neuropathy following orbital fat decompression. Br J Ophthalmol 2000;84:600–605. Stan MN, Garrity JA, Bahn RS. The evaluation and treatment of Graves ophthalmopathy. Med Clin North Am 2012;96:311–328. Nugent RA, Belkin RI, Neigel JM, Rootman J, et al. Graves orbitopathy: correlation of CT and clinical findings. Radiology 1990;177:675–682. Chan LL, Tan HE, Fook-Chond S, Teo TH, et al. Graves ophthalmopathy: the bony orbit in optic neuropathy, its apical angular capacity, and impact on prediction of risk. Am J Neuroradiol 2009;30:597–602. Lee HB, Rodgers IR, Woog JJ. Evaluation and management of Graves’ orbitopathy. Otolaryngol Clin North Am 2006;39: 923–942.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
