Letters to the Editor
Reply—Spontaneous Cerebrospinal Fluid Otorrhea and Rhinorrhea in Idiopathic Intracranial Hypertension Patients
W
e thank Vaphiades et al for their interesting comments. 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 rhinorrhea 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 earlier 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 manifestations. 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
I
n 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, including spinal leptomeningeal lymphoma (1) and Sheehan syndrome (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 demonstrated bilateral papilledema, and automated visual fields showed enlarged blind spots. Optical coherence tomography of the peripapillary retinal nerve fiber layer (RNFL) confirmed 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). Contrastenhanced magnetic resonance imaging (MRI) and magnetic resonance venography confirmed a 3.5-cm filling defect, noted to be isointense to cerebrospinal fluid (CSF) Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423
(Fig. 1B, C). Because these findings initially were interpreted 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 diplopia 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. Manometry 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. 417
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Reply—Spontaneous Cerebrospinal Fluid Otorrhea and Rhinorrhea in Idiopathic Intracranial Hypertension Patients
W
e thank Vaphiades et al for their interesting comments. 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 rhinorrhea 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 earlier 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 manifestations. 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
I
n 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, including spinal leptomeningeal lymphoma (1) and Sheehan syndrome (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 demonstrated bilateral papilledema, and automated visual fields showed enlarged blind spots. Optical coherence tomography of the peripapillary retinal nerve fiber layer (RNFL) confirmed 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). Contrastenhanced magnetic resonance imaging (MRI) and magnetic resonance venography confirmed a 3.5-cm filling defect, noted to be isointense to cerebrospinal fluid (CSF) Letters to the Editor: J Neuro-Ophthalmol 2013; 33: 412-423
(Fig. 1B, C). Because these findings initially were interpreted 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 diplopia 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. Manometry 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. 417
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
