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Show Reply-Spontaneous Cerebrospinal Fluid Otorrhea and Rhinorrhea in Idiopathic Intracranial Hypertension Patients We thank Vaphiades et al for their interesting com-ments. Our manuscript (1) describes 4 patients with idiopathic intracranial hypertension (IIH) and increased intracranial pressure (ICP) (range, 270-370 mm H2O) that developed an unusual complication of spontaneous rhinor-rhea or otorrhea (one as the initial chief complaint and the others later in the clinical course). Our study does not address the issue of whether most cases of spontaneous cerebrospinal fluid (CSF) leak occur in patients with high ICP or not. In a recent publication by Chaaban et al (2), it was reported that the majority of spontaneous CSF leaks are secondary to increased ICP, and that successful treatment of elevated ICP together with endoscopic repair can provide cure for most patients. This conclusion contradicts an ear-lier publication by Shugar et al (3) who have reported that 55% of cases of nontraumatic rhinorrhea described in the literature are associated with normal ICP and 45% with elevated pressure. Spontaneous rhinorrhea or otorrhea is rare in patients with IIH. One goal of our report was to raise awareness among clinicians to these unusual manifes-tations. Gad Dotan Eldar Rosenfeld Tali Jonas Kimji Anat Kesler REFERENCES 1. Rosenfeld E, Dotan G, Kimchi TJ, Kesler A. Spontaneous cerebrospinal fluid otorrhea and rhinorrhea in idiopathic intracranial hypertension patients. J Neuroophthalmol. 2013;33:113-116. 2. Chaaban MR, Illing E, Riley KO, Woodworth BA. Spontaneous cerebrospinal fluid leak repair: a five-year prospective evaluation. Laryngoscope. [published ahead of print June 20, 2013] doi: 10.1002/lary.24160. 3. Shugar JM, Som PM, Eisman W, Biller HF. Non-traumatic cerebrospinal fluid rhinorrhea. Laryngoscope. 1981;91:114-120. Pseudotumor Cerebri Syndrome Associated With Giant Arachnoid Granulation In the March 2013 issue of the Journal, we read with great interest the articles dealing with various disorders initially thought to be idiopathic intracranial hypertension, includ-ing spinal leptomeningeal lymphoma (1) and Sheehan syn-drome (2). We describe a rare case of pseudotumor cerebri (PTC), associated with a giant arachnoid granulation (GAG) in the transverse venous sinus. A 26-year-old healthy, nonobese man complained of horizontal diplopia for 1 month. Visual acuity was 20/25 in each eye, pupillary reactions were normal, and ocular motility revealed bilateral abduction deficits. Funduscopy demon-strated bilateral papilledema, and automated visual fields showed enlarged blind spots. Optical coherence tomography of the peripapillary retinal nerve fiber layer (RNFL) con-firmed optic disc edema, with average RNFL thickness of 171 mm for the right eye and 275 mm for the left eye. Computed tomography (CT) of the brain revealed no mass lesion or hydrocephalus but a hypodense filling defect at the origin of the right transverse sinus (Fig. 1A). Contrast-enhanced magnetic resonance imaging (MRI) and magnetic resonance venography confirmed a 3.5-cm filling defect, noted to be isointense to cerebrospinal fluid (CSF) (Fig. 1B, C). Because these findings initially were inter-preted as subacute or chronic venous sinus thrombosis, a hypercoagulability work-up was initiated and the patient was started on daily aspirin (81 mg). On further review of the neuroimaging studies and based on the filling defect being isointense to CSF, the diagnosis was changed to GAG of the transverse sinus. Lumbar puncture revealed an elevated opening pressure of 56 cm water, with normal CSF composition. The diagnosis of PTC was made, and treatment was initiated with oral acetazolamide 500 mg twice daily. Despite titrating doses of acetazolamide up to 1,000 mg twice daily, the patient's dip-lopia persisted, and he developed more severe headaches with worsening papilledema. Because of progressive symptoms and signs, intervention via an endovascular approach was offered to the patient. Cerebral venography revealed intact venous flow around the intraluminal obstruction in the right transverse sinus. Ma-nometry showed pressure proximal to the GAG of 14 mm Hg and 3 mm Hg distal to the GAG. Because the appearance and pressures within the left transverse and superior sagittal sinuses were normal, stenting was not performed. Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423 417 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. The patient continued on medical treatment alone, with his doses of acetazolamide increased to 1,000 mg 3 times daily. A repeated lumbar puncture showed opening pressure of 32 cm water. His headaches and papilledema slowly improved and were resolved by 6 months. Repeat MRI revealed no change in the size of the hypodense filling defect in the right transverse sinus. Arachnoid granulations filter CSF across the lining of the arachnoid into the cerebral venous system. GAGs are believed to be a normal variant (3,4), with an estimated prevalence of 0.3%-1.0% in adults (4). GAGs may mimic other entities and may be misdiag-nosed as venous sinus thrombosis. However, venous sinus thrombosis is hyperintense on both CT and T1 axial MRI and enhances with contrast. Alternatively, GAGs are hypo-intense on CT and T1 axial MRI and do not enhance (4). Although usually asymptomatic, GAGs have been reported in association with increased intracranial pressure (ICP) and PTC in only a handful of cases (5-8). Arjona et al (6) described a 51-year-old nonobese man who presented with transient visual obscurations and bilateral papilledema. The patient was found to have a GAG at the right transverse sinus-sigmoid sinus junction, with hypoplasia of the left transverse sinus. Opening pressure on lumbar puncture was 27 cm water. Unfortunately, the authors did not discuss the management of this case but believed GAG to be responsible for the elevated ICP. Choi et al (7) reported a 66-year-old woman with occipital headaches who was initially diagnosed as having dural sinus thrombosis. MRI confirmed the diag-nosis of GAG, and a normal venous pressure gradient was found across the symptomatic lesion. The patient's headaches were managed with medical therapy. Zheng et al (8) described a unique case of a patient with GAG in the dom-inant left transverse sinus and elevated pressure proximal to the GAG. Stenting of the transverse sinus reduced the pres-sure gradient across the lesion, as well as the ICP, and led to symptomatic improvement. The cause of GAG formation is uncertain. It has been postulated that increased CSF volume and pressure may cause hypertrophy of the arachnoid granulations, with subsequent formation of a GAG (4). However, if this were the case, then many more patients with ICP from various etiologies would develop GAGs. Alternately, they may represent hyperplasia of preexisting arachnoid granulations or benign neoplasm of mesenchymal origin (3). The clinical course of our patient raises the question of whether the GAG caused PTC or whether its presence was purely coincidental. Normal venous sinus pressures proxi-mal to the GAG make it less likely that GAG raised venous sinus pressure and led to increased ICP. With medical therapy alone, PTC completely resolved in our patient. There is still the possibility that the GAG had caused increased ICP, and venous collaterals developed with time, facilitating venous outflow. We did not perform a follow-up cerebral venogram after our patient's PTC resolved to eval-uate this possibility. Kevin I. Rosenberg, MD Rudrani Banik, MD Department of Ophthalmology, The New York Eye and Ear Infirmary, New York, New York The authors report no conflicts of interest. FIG. 1. A. CT reveals a hypodense filling defect at the origin of the right transverse sinus adjacent to the torcular, with involvement of the proximal left transverse sinus (arrow). B. T1 axial MRI shows the hypodense filling defect (arrow) at the origin of the right transverse sinus, corresponding to the CT image. C. T2 axial MRI shows a hyperintense signal (arrow) corresponding to the filling defect. CT, computed tomography; MRI, magnetic resonance imaging. 418 Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. REFERENCES 1. Ahmed RM, King J, Gibson J, Buckland ME, Gupta R, Gonzales M, Halmagyi M. Spinal leptomeningeal lymphoma presenting as pseudotumor syndrome. J Neuroophthalmol. 2013;33:13-16. 2. Moodley KK, Broad R, Chung K, Riordan-Eva P, Sibtain NA, Moran NF. Sheehan syndrome associated with raised intracranial pressure. J Neuroophthalmol. 2013;33:54-57. 3. Mamourian AC, Towfighi J. MR of giant arachnoid granulation, a normal variant presenting as a mass within the dural venous sinus. AJNR Am J Neuroradiol. 1995;16(suppl):901-904. 4. Roche J, Warner D. Arachnoid granulations in the transverse and sigmoid sinuses: CT, MR, and MR angiographic appearance of a normal anatomic variation. AJNRAm J Neuroradiol. 1996;17:677-683. 5. Peters SA, Frombach E, Heyer CM. Giant arachnoid granulation: differential diagnosis of acute headache. Australas Radiol. 2007;51(suppl 1):B18-B20. 6. Arjona A, Delgado F, Fernandez-Romero E. Intracranial hypertension secondary to giant arachnoid granulation, J Neurol Neurosurg Psychiatry. 2003;74:418. 7. Choi HJ, Cho CW, Kim YS, Cha JH. Giant arachnoid granulation misdiagnosed as transverse sinus thrombosis. J Korean Neurosurg Soc. 2008;43:48-50. 8. Zheng Z, Zhou M, Zhao B, Zhou D, He L. Pseudotumor cerebri syndrome and giant arachnoid granulation: treatment with venous sinus stenting. J Vasc Interv Radiol. 2010;21:927-929. Optic Perineuritis Secondary to Acute Retinal Necrosis Optic perineuritis (OPN) has been recognized as a form of idiopathic orbital inflammatory disease, where the specific target is the optic nerve sheath (1-4). Most reported cases are isolated and idiopathic, but some have been associated with specific infectious or inflammatory disorders, including Wegener granulomatosis, giant cell arteritis, syphilis, and viral meningitis (5-8). A recent report by Townsend et al (9) documented OPN as a pre-senting finding in a patient with leukemia. We evaluated a patient with acute retinal necrosis (ARN) who subse-quently developed OPN. A 67-year-old man reported decreased vision in his left eye. Examination revealed visual acuity of 20/80 with cells in the anterior chamber, multiple patchy areas of necrosis in the peripheral retina, retinal vasculitis, optic disc swelling and vitritis (Fig. 1A). He was diagnosed with ARN and treated with acyclovir 800 mg intravenously 3 times a day. Despite antiviral therapy, vision declined to 20/200 in the left eye. Ganciclovir (2 mg/0.1 mL) was injected into the vitreous cavity, and the patient was prescribed oral fam-ciclovir 500 mg, 3 times a day. On day 7, oral prednisolone (60 mg/day) was added. Prophylactic vitrectomy was per-formed with a silicone oil tamponade, and the patient's vision gradually improved to 20/100 (Fig. 1B). Systemic corticosteroids were tapered over 3 weeks and then discontinued, while maintaining oral famciclovir. After 3 days, the patient reported pain with left eye movement and had no light perception in the left eye. The fundus appearance was unchanged, and a fluorescein angiography showed no specific findings to explain the vision loss. Flash visual evoked potential was nonrecordable in the left eye. Although magnetic resonance image (MRI) of the brain appeared normal, there was enhancement of the left optic nerve sheath (Fig. 2). Cerebrospinal fluid analysis was un-remarkable. The patient was given high-dose intravenous methylprednisolone (1 g/day) for 3 days, followed by oral steroids and maintained on famciclovir. Visual acuity in the left eye recovered to 20/500 at 2 months, without further improvement over the following 10 months. Optic nerve involvement in ARN has been reported in 47%-57% of cases (10-12). Proposed mechanisms include intraneural vasculitis, direct viral invasion of the optic nerve, FIG. 1. A, Left fundus shows peripheral retinal necrosis, optic disc swelling, and vitreous haziness. B, After systemic therapy and vitrectomy with silicone oil tamponade, there is resolution of retinal necrosis and optic disc swelling with sheathing of the retinal vessels and optic disc pallor. Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423 419 Letters to the Editor Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
