Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
Case Reports
Coincidental Optic Nerve Meningioma and Thyroid Eye Disease Aakriti Garg, B.A.*, Payal Patel, M.D.*, Angela Lignelli, M.D.†, Edward Baron, M.D.‡, and Michael Kazim, M.D.*
Abstract: A 57-year-old woman with diabetes mellitus, hypertension, obesity, and Graves disease presented with clinical evidence of thyroid eye disease (TED) and optic neuropathy. She was referred when a tapered dose of steroids prompted worsening of her TED. CT and MRI were consistent with TED and bilateral optic nerve meningioma. To the authors’ knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. When investigating the etiology of TEDassociated optic neuropathy, careful attention to orbital imaging is required because coexisting pathology may exist.
T
hyroid eye disease (TED) is an autoimmune disorder usually associated with Graves disease that most commonly affects women more than men in their fourth and fifth decades.1 Optic nerve sheath meningioma also typically affects middleaged women.2 Herein, the authors report the clinical examination, CT, and MRI of a 57-year-old woman having optic neuropathy and diagnosed with concurrent TED and optic nerve meningioma. This case report complied with policies established by the Health Insurance Portability and Accountability Act and the Institutional Review Board at Columbia University Medical Center. Oral informed consent was obtained from the patient.
CASE REPORT A 57-year-old woman with TED presented with complaints of blurred vision of 1-week duration. Approximately 1 year prior to presentation, the patient developed the characteristics of TED, which included eyelid swelling and mild blurring of vision. Soon after, she developed palpitations and diaphoresis and was ultimately diagnosed with Graves hyperthyroidism and was treated with methimazole. Her ocular symptoms progressed to include retrobulbar pain and diplopia, and oral prednisone (80 mg) was prescribed by her general ophthalmologist. She was referred when a tapering dose of corticosteroids prompted worsening of her TED. Her medical history included glaucoma, Type-2 diabetes mellitus, hypertension, and obesity. On presentation, best-corrected visual acuity was 20/25 OD and 20/20 OS. She identified 3/6 Hardy Rand and Rittler pseudoisochromatic color plates OU but was noted to have 5% relative red desaturation *Department of Ophthalmology, Edward S Harkness Eye Institute, and †Department of Radiology, Columbia University College of Physicians and Surgeons, New York; and ‡Department of Ophthalmology, Albert Einstein College of Medicine, Bronx, New York, U.S.A. Accepted for publication December 13, 2013. This work was supported by grants from the Doris Duke Charitable Foundation to Columbia University (A.G.) and the Orbital Disease, Education, and Research Foundation. Presented at International Thyroid Eye Disease Society Meeting on June 28 and 29, 2013 in Vancouver, BC, Canada. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Michael Kazim, M.D., Columbia University Harkness Eye Institute, 630 West 165th Street, New York, NY 10032. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000113
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OD. There was no afferent pupil defect noted. Extraocular movements were severely limited bilaterally in all directions of gaze. Hertel exophthalmometry measured 28 mm OD and 25 mm OS. Bilateral upper and lower eyelid edema and lagophthalmos were present. There was chemosis, 2+ conjunctival injection, and exposure keratopathy OU. Fundoscopic examination revealed optic nerve cupping OU with cup-to-disc ratio of 0.5, and no evidence of retinopathy. She had pretibial myxedema. Humphrey visual field testing demonstrated bilateral inferior scotomas. Noncontrast CT revealed bilateral fusiform enlargement of all but the lateral rectus muscles, sparing the tendons, consistent with TED, and compatible with apical compression of the optic nerve. Additionally noted were bilateral circumferential optic nerve calcifications with a tram track appearance, suggesting optic meningioma (Fig. 1). This diagnosis was further supported by MRI, which demonstrated bilateral enhancement of the optic nerve sheaths apically extending to the chiasm (Fig. 2). Given the patient’s comorbidities, the authors recommended a short course of corticosteroids to suppress orbital inflammation followed by orbital bone decompression surgery. They suggested that treatment of the meningioma be deferred until the stable phase of TED was ensured, and compressive optic neuropathy (CON) due to TED maximally reversed. The patient elected follow up with her neuroophthalmologist. Her vision ultimately declined to 20/40 OD and 20/30 OS, and she underwent decompression surgery. Postsurgically, there was improvement in vision by 3 lines OD and an increase in color vision by 1 Hardy Rand and Rittler pseudoisochromatic color plate OU.
DISCUSSION To the authors’ knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. The latter diagnosed by the presence of linear calcification of the optic nerve sheath accompanied by an unenhanced optic nerve, the so-called “tram track sign.”3,4 The active phase of TED typically lasts 6 to 18 months.5 Corticosteroids are typically reserved for severe orbitopathy including CON and used in concert with external beam radiotherapy to increase treatment efficacy and durability.1 In this patient, management was complicated by the comorbidities (hypertension, glaucoma, obesity, and Type-2 diabetes mellitus) and bilateral optic nerve meningioma. Long-term corticosteroid treatment of CON was therefore not advised. Instead, the authors suggested corticosteroids to provide urgent reversal of orbital inflammation to improve the safety of the anticipated decompression. Despite the routine adjunctive use of orbital radiotherapy in cases of steroid responsive CON, they recommended against its use in this case. They thought that the 20 Gy typically recommended for CON-TED would complicate or preclude the future use of the 40 to 50 Gy indicated for vision loss associated with optic meningioma.1,6 This case illustrates the importance of careful attention to orbital imaging when investigating the etiology of optic neuropathy in TED patients because coexisting pathology may exist.
REFERENCES 1. Zoumalan CI, Cockerham KP, Turbin RE, et al. Efficacy of corticosteroids and external beam radiation in the management of moderate to severe thyroid eye disease. J Neuroophthalmol 2007;27:205–14. 2. Shapey J, Sabin HI, Danesh-Meyer HV, et al. Diagnosis and management of optic nerve sheath meningiomas. J Clin Neurosci 2013;20:1045–56.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
Case Reports
FIG. 1. CT without contrast shows bilateral fusiform enlargement of the right lateral and both medial rectus muscle sparing the tendons in a pattern consistent with thyroid eye disease and optic nerve calcifications with a tram track appearance, suggestive of optic nerve meningioma.
3. Baehring JM. Tram track sign. J Neurooncol 2007;85:75. 4. Kanamalla US. The optic nerve tram-track sign. Radiology 2003;227:718–9. 5. Dolman PJ. Evaluating Graves’ orbitopathy. Best Pract Res Clin Endocrinol Metab 2012;26:229–48. 6. Turbin RE, Thompson CR, Kennerdell JS, et al. A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapy. Ophthalmology 2002;109:890–9; discussion 899–900.
No Intense 18F-Fluorodeoxyglucose Uptake in Positron Emission Tomography of a Metastatic Orbital Tumor From Breast Carcinoma Hiromichi Matsuda, M.D.*, Yasuhiro Takahashi, M.D., Ph.D.*, Kouhei Nakata, M.D., Ph.D.†, Atsuko Kitamura, M.D., Ph.D.‡, and Hirohiko Kakizaki, M.D., Ph.D.* FIG. 2. Postfat saturation T1-weighted MRI shows enhancement of optic chiasm and dura extending laterally on the left.
*Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi; †Department of Radiology, Wakayama Medical University, Wakayama; and ‡Department of Pathology, Aichi Medical University Hospital, Nagakute, Aichi, Japan Accepted for publication December 25, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Hirohiko Kakizaki, M.D., Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi 480-1195, Japan. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000115
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e95
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Case Reports
Coincidental Optic Nerve Meningioma and Thyroid Eye Disease Aakriti Garg, B.A.*, Payal Patel, M.D.*, Angela Lignelli, M.D.†, Edward Baron, M.D.‡, and Michael Kazim, M.D.*
Abstract: A 57-year-old woman with diabetes mellitus, hypertension, obesity, and Graves disease presented with clinical evidence of thyroid eye disease (TED) and optic neuropathy. She was referred when a tapered dose of steroids prompted worsening of her TED. CT and MRI were consistent with TED and bilateral optic nerve meningioma. To the authors’ knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. When investigating the etiology of TEDassociated optic neuropathy, careful attention to orbital imaging is required because coexisting pathology may exist.
T
hyroid eye disease (TED) is an autoimmune disorder usually associated with Graves disease that most commonly affects women more than men in their fourth and fifth decades.1 Optic nerve sheath meningioma also typically affects middleaged women.2 Herein, the authors report the clinical examination, CT, and MRI of a 57-year-old woman having optic neuropathy and diagnosed with concurrent TED and optic nerve meningioma. This case report complied with policies established by the Health Insurance Portability and Accountability Act and the Institutional Review Board at Columbia University Medical Center. Oral informed consent was obtained from the patient.
CASE REPORT A 57-year-old woman with TED presented with complaints of blurred vision of 1-week duration. Approximately 1 year prior to presentation, the patient developed the characteristics of TED, which included eyelid swelling and mild blurring of vision. Soon after, she developed palpitations and diaphoresis and was ultimately diagnosed with Graves hyperthyroidism and was treated with methimazole. Her ocular symptoms progressed to include retrobulbar pain and diplopia, and oral prednisone (80 mg) was prescribed by her general ophthalmologist. She was referred when a tapering dose of corticosteroids prompted worsening of her TED. Her medical history included glaucoma, Type-2 diabetes mellitus, hypertension, and obesity. On presentation, best-corrected visual acuity was 20/25 OD and 20/20 OS. She identified 3/6 Hardy Rand and Rittler pseudoisochromatic color plates OU but was noted to have 5% relative red desaturation *Department of Ophthalmology, Edward S Harkness Eye Institute, and †Department of Radiology, Columbia University College of Physicians and Surgeons, New York; and ‡Department of Ophthalmology, Albert Einstein College of Medicine, Bronx, New York, U.S.A. Accepted for publication December 13, 2013. This work was supported by grants from the Doris Duke Charitable Foundation to Columbia University (A.G.) and the Orbital Disease, Education, and Research Foundation. Presented at International Thyroid Eye Disease Society Meeting on June 28 and 29, 2013 in Vancouver, BC, Canada. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Michael Kazim, M.D., Columbia University Harkness Eye Institute, 630 West 165th Street, New York, NY 10032. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000113
e94
OD. There was no afferent pupil defect noted. Extraocular movements were severely limited bilaterally in all directions of gaze. Hertel exophthalmometry measured 28 mm OD and 25 mm OS. Bilateral upper and lower eyelid edema and lagophthalmos were present. There was chemosis, 2+ conjunctival injection, and exposure keratopathy OU. Fundoscopic examination revealed optic nerve cupping OU with cup-to-disc ratio of 0.5, and no evidence of retinopathy. She had pretibial myxedema. Humphrey visual field testing demonstrated bilateral inferior scotomas. Noncontrast CT revealed bilateral fusiform enlargement of all but the lateral rectus muscles, sparing the tendons, consistent with TED, and compatible with apical compression of the optic nerve. Additionally noted were bilateral circumferential optic nerve calcifications with a tram track appearance, suggesting optic meningioma (Fig. 1). This diagnosis was further supported by MRI, which demonstrated bilateral enhancement of the optic nerve sheaths apically extending to the chiasm (Fig. 2). Given the patient’s comorbidities, the authors recommended a short course of corticosteroids to suppress orbital inflammation followed by orbital bone decompression surgery. They suggested that treatment of the meningioma be deferred until the stable phase of TED was ensured, and compressive optic neuropathy (CON) due to TED maximally reversed. The patient elected follow up with her neuroophthalmologist. Her vision ultimately declined to 20/40 OD and 20/30 OS, and she underwent decompression surgery. Postsurgically, there was improvement in vision by 3 lines OD and an increase in color vision by 1 Hardy Rand and Rittler pseudoisochromatic color plate OU.
DISCUSSION To the authors’ knowledge, this is the first reported case of concurrent TED and unsuspected bilateral optic nerve meningioma. The latter diagnosed by the presence of linear calcification of the optic nerve sheath accompanied by an unenhanced optic nerve, the so-called “tram track sign.”3,4 The active phase of TED typically lasts 6 to 18 months.5 Corticosteroids are typically reserved for severe orbitopathy including CON and used in concert with external beam radiotherapy to increase treatment efficacy and durability.1 In this patient, management was complicated by the comorbidities (hypertension, glaucoma, obesity, and Type-2 diabetes mellitus) and bilateral optic nerve meningioma. Long-term corticosteroid treatment of CON was therefore not advised. Instead, the authors suggested corticosteroids to provide urgent reversal of orbital inflammation to improve the safety of the anticipated decompression. Despite the routine adjunctive use of orbital radiotherapy in cases of steroid responsive CON, they recommended against its use in this case. They thought that the 20 Gy typically recommended for CON-TED would complicate or preclude the future use of the 40 to 50 Gy indicated for vision loss associated with optic meningioma.1,6 This case illustrates the importance of careful attention to orbital imaging when investigating the etiology of optic neuropathy in TED patients because coexisting pathology may exist.
REFERENCES 1. Zoumalan CI, Cockerham KP, Turbin RE, et al. Efficacy of corticosteroids and external beam radiation in the management of moderate to severe thyroid eye disease. J Neuroophthalmol 2007;27:205–14. 2. Shapey J, Sabin HI, Danesh-Meyer HV, et al. Diagnosis and management of optic nerve sheath meningiomas. J Clin Neurosci 2013;20:1045–56.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
Case Reports
FIG. 1. CT without contrast shows bilateral fusiform enlargement of the right lateral and both medial rectus muscle sparing the tendons in a pattern consistent with thyroid eye disease and optic nerve calcifications with a tram track appearance, suggestive of optic nerve meningioma.
3. Baehring JM. Tram track sign. J Neurooncol 2007;85:75. 4. Kanamalla US. The optic nerve tram-track sign. Radiology 2003;227:718–9. 5. Dolman PJ. Evaluating Graves’ orbitopathy. Best Pract Res Clin Endocrinol Metab 2012;26:229–48. 6. Turbin RE, Thompson CR, Kennerdell JS, et al. A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapy. Ophthalmology 2002;109:890–9; discussion 899–900.
No Intense 18F-Fluorodeoxyglucose Uptake in Positron Emission Tomography of a Metastatic Orbital Tumor From Breast Carcinoma Hiromichi Matsuda, M.D.*, Yasuhiro Takahashi, M.D., Ph.D.*, Kouhei Nakata, M.D., Ph.D.†, Atsuko Kitamura, M.D., Ph.D.‡, and Hirohiko Kakizaki, M.D., Ph.D.* FIG. 2. Postfat saturation T1-weighted MRI shows enhancement of optic chiasm and dura extending laterally on the left.
*Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi; †Department of Radiology, Wakayama Medical University, Wakayama; and ‡Department of Pathology, Aichi Medical University Hospital, Nagakute, Aichi, Japan Accepted for publication December 25, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Hirohiko Kakizaki, M.D., Department of Ophthalmology, Aichi Medical University, Nagakute, Aichi 480-1195, Japan. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000115
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e95
Copyright © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
