Journal of Neuro-Ophthalmology, September 2013, Volume 33, Issue 3

Idiopathic Sclerosing Orbital Inflammation: Presentation of an Unusual Case With Isolated Bilateral Optic Nerve Involvement

with worsening myasthenic symptoms, including ocular complaints, might benefit from repeat mediastinal imaging to rule out recurrent or residual thymoma (6). Cynthia Ishin Tung, MD Department of Ophthalmology The University of Texas Medical Branch, Galveston, TX Department of Ophthalmology The Methodist Hospital, Houston, TX Diana Chao, MD Department of Ophthalmology The University of Texas Medical Branch, Galveston, TX Department of Ophthalmology The Methodist Hospital, Houston, TX Nagham Al-zubidi, MD Department of Ophthalmology The Methodist Hospital, Houston, TX Monica Yalamanchili Saint Bonaventure University Saint Bonaventure, NY Andrew Go Lee, MD Department of Ophthalmology The University of Texas Medical Branch, Galveston, TX Department of Ophthalmology The Methodist Hospital, Houston, TX Baylor College of Medicine, Houston, TX Departments of Ophthalmology Neurology, and Neurosurgery Weill Cornell Medical College, New York, NY Department of Ophthalmology The University of Iowa Hospitals and Clinics Iowa City, IA [e-mail] Supported in part by an unrestricted grant from the Research to Prevent Blindness to the University of Texas Medical Branch, Galveston, TX. The authors report no conflicts of interest. REFERENCES 1. Bruce BB, Kupersmith MJ. Safety of prednisone for ocular myasthenia gravis. J Neuroophthalmol. 2012;32:212-215. 2. Masaoka A, Yamakawa Y, Niwa H, Fukai I, Kondo S, Kobayashi M, Fujii Y, Monden Y. Extended thymectomy for myasthenia gravis patients: a 20-year review. Ann Thorac Surg. 1996;62:853-859. 3. Roberts PF, Venuta F, Rendina E, De Giacomo T, Coloni GF, Follette DM, Richman DP, Benfield JR. Thymectomy in the treatment of ocular myasthenia gravis. J Thor Cardiovasc Surg. 2001;122:562-568. 4. Masaoka A, Yamakawa Y, Niwa H, Fukai I, Saito Y, Tokudome S, Nakahara K, Fujii Y. Thymectomy and malignancy. Eur J Cardiothorac Surg. 1994;8:251-253. 5. Liu W, Tong T, Ji Z, Zhang Z. Long-term prognostic analysis of thymectomized patients with myasthenia gravis. Chinese Med J. 2002;11:235-237. 6. Ito M, Fujimura S, Monden Y, Watanabe Y, Shimizu N, Hirono T, Iuchi K, Tsubota N, Masaoka A. A retrospective group study on post-thymectomy myasthenia gravis. Nippon Kyobu Geka Gakkai Zasshi. 1992;40:189-193. Idiopathic Sclerosing Orbital Inflammation: Presentation of an Unusual Case With Isolated Bilateral Optic Nerve Involvement We read with great interest the report by Levin et al (1) regarding the role of the optic nerve biopsy in the management of progressive optic neurop-athy. We had the opportunity to evaluate a 50-year-old man with progressive deterioration of vision in his left eye for 2 months with minimal periocular pain. Visual acuity was 20/20, right eye, and no light perception, left eye. The right fundus was normal but there was marked optic disc edema in the left eye (Fig. 1). Magnetic reso-nance imaging (MRI) of the brain and orbits demon-strated enhancement and thickening of the left optic nerve (Fig. 2). An extensive work-up including hemato-logic tests, lumbar puncture, and computed tomography of chest, abdomen, and pelvis was unremarkable. The patient was given 1 g of methylprednisolone intra-venously for 3 days followed by a tapering dose of oral steroids over several months. Although there was im-provement in the left optic disc edema, vision remained unchanged. Four months after onset of symptoms, the patient reported vision loss in the right eye. Acuity was 20/40, right eye, and no light perception, left eye. Funduscopy revealed right optic disc edema and left optic atrophy. MRI demonstrated thickening of the right optic with marked enhancement following intravenous contrast. Prednisone was restarted at a dose of 1 mg/kg/d, and there was rapid improvement in the vision in the right eye with resolution of right optic disc edema. 308 Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 307-318 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Given involvement of the previously unaffected right eye, a biopsy of the left optic nerve was performed. Histopathology showed primarily fibrosis and an inflam-matory infiltrate consisting of lymphocytes, histiocytes, and plasma cells (Fig. 3). No granulomas were identified. Stains for infectious, immune, and neoplastic disorders were nondiagnostic. Based on these results, a diagnosis of idiopathic sclerosing orbital inflammation (ISOI) was made. ISOI is a rare condition characterized by marked fibrosis and some inflammatory infiltrate (2-4). However, unlike idiopathic orbital inflammation, which has an acute clinical onset, the progression of ISOI generally has a chronic and indolent course (5). In our patient, there was consecutive involvement of both optic nerves. There are reports of ISOI associated with systemic idiopathic fibrosis and increased serum levels of IgG4 (6,7). Our patient did not show in-creased serum levels of IgG4, and the biopsy specimen was negative for the presence of IgG4. Alberto Galvez-Ruiz, MD, PhD Department of Ophthalmology, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, [e-mail] Imtiaz Chaudhry, MD Department of Ophthalmology, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia Hind Al Katan, MD Department of Pathology, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia The author reports no conflicts of interest. REFERENCES 1. Levin MH, Ney JJ, Venneti S, Moster ML, Balcer LJ, Volpe NJ, Gausas RE, Liu GT, Vagefi MR, Galetta SL. Optic nerve biopsy in the management of progressive optic neuropathy. J Neuroophthalmol. 2012;32:313-320. FIG. 2. Postcontrast axial (A) and coronal (B) T1 magnetic resonance imaging with fat suppression shows thickening and enhancement of the left optic nerve. FIG. 3. Biopsy demonstrates optic nerve and sheath frag-ments with marked fibrosis accompanied by an in-flammatory infiltrate of lymphocytes, histiocytes, and plasma cells (periodic acid-Schiff, ·400). FIG. 1. Left fundus shows marked optic disc edema with peripapillary hemorrhages. Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 307-318 309 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 2. Hsuan JD, Selva D, McNab AA, Sullivan TJ, Saeed P, O'Donnell BA. Idiopathic sclerosing orbital inflammation. Arch Ophthalmol. 2006;124:1244-1250. 3. Rootman J, McCarthy M, White V, Harris G, Kennerdell J. Idiopathic sclerosing inflammation of the orbit. A distinct clinicopathologic entity. Ophthalmology. 1994;101:570-584. 4. Liu CH, Ma L, Ku WJ, Kao LY, Tsai YJ. Bilateral idiopathic sclerosing inflammation of the orbit: a report of three cases. Chang Gung Med J. 2004;27:758-765. 5. Brannan PA. A review of sclerosing idiopathic orbital inflammation. Curr Opin Ophthalmol. 2007;18:402-404. 6. Berry-Brincat A, Rose GE. Idiopathic orbital inflammation: a new dimension with the discovery of immunoglobulin G4-related disease. Curr Opin Ophthalmol. 2012;23:415-419. 7. Cheuk W, Yuen HK, Chan AC, Shih LY, Kuo TT, Ma MW, Lo YF, Chan WK, Chan JK. Ocular adnexal lymphoma associated with IgG41 chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol. 2008;32:1159-1167. Intracranial Hypertension in a Patient Preparing for Gestational Surrogacy With Leuprolide Acetate and Estrogen We read with great interest the editorial (1) and series of articles dealing with intracranial hypertension recently published in the Journal of Neuro-Ophthalmology. Gestational surrogacy, the process by which a genetically unrelated woman is implanted with an embryo, requires preparation with leuprolide acetate and estrogen. It is increasing as a management option for infertility. Intracra-nial hypertension is a rare adverse effect of sterility manage-ment with leuprolide acetate occurring after months or years of administration. We evaluated a 23-year-old mul-tiparous woman being prepared for gestational surrogacy with injections, daily leuprolide acetate and twice weekly estrogen, who developed intracranial hypertension within 7 days of starting therapy. She developed gradual onset of holocephalic pounding headache that increased in intensity over the course of 1 week and was made worse by maneu-vers that increase intracranial pressure such as laying flat. She also had blurry vision and binocular horizontal diplo-pia. She had been on this regimen the previous year for gestational surrogacy and tolerated it well. She was not obese and her weight had been stable. Visual acuity was 20/20, right eye, and 20/25, left eye, with normal color vision (Hardy-Rand-Rittler plates) and confrontation visual field testing. Extraocular movements demonstrated a right sixth nerve paresis, and funduscopy revealed bilateral optic disc swelling. Brain magnetic reso-nance imaging and MR venography were normal. Lumbar puncture was significant for an opening pressure of 440 mm of water. Closing pressure was 100 mm of water following removal of 20 mL of cerebrospinal fluid. Her headache improved immediately after the lumbar puncture and con-tinued to improve following cessation of hormone therapy. Leuprolide acetate is a synthetic gonadotrophin-releasing hormone or luteinizing hormone-releasing hor-mone analog used to treat sterility in women and prostate cancer in men (2). More recently, it has been used for gestational surrogacy. There are 2 reports describing in-tracranial hypertension in patients taking leuprolide, one in a patient on pulsatile pump for 2 years for sterility management and in another following discontinuation of the drug after 5 months of treatment (3,4). Fraunfelder and Edwards (5) suggested that cases in their review lacked sufficient data to determine whether intracranial hyperten-sion was due to leuprolide. In contrast, we documented intracranial hypertension occurring within 7 days of initiating treatment with leupro-lide. The role of the two doses of estrogen she received and of previous treatment from previous gestational surrogacy is unclear. Although our patient was in the age group affected by idiopathic intracranial hypertension, she did not have associ-ated comorbid conditions such as obesity or recent weight gain (6,7). Additionally, she improved rapidly with discontin-uation of hormonal therapy suggesting that daily leuprolide likely induced the intracranial hypertension. Considering the increasing numbers of gestational surrogacy, the lack of similar case reports would imply that intracranial hypertension is a rare occurrence in this context. However, this diagnosis should be considered if a patient being prepared for surrogacy has new onset of headache, and a fundus examination should be performed to look for papilledema. Joshua Alexander, DO, MPH Department of Neurosciences University of California San Diego San Diego California [e-mail] Leah Levi, MBBS Departments of Ophthalmology and Neurosciences University of California San Diego San Diego California The authors report no conflicts of interest. 310 Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 307-318 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.