Clinical Observation
Optic Nerve Sheath Fenestration for the Treatment of Papilledema Secondary to Cerebral Venous Thrombosis Jennifer Murdock, MD, Jonathan H. Tzu, MD, Norman J. Schatz, MD, Wendy W. Lee, MD
Abstract: A 16-year-old adolescent girl with multiple risk factors for thrombosis presented with acute onset of headache, decreased vision, and papilledema. Evaluation demonstrated cerebral venous thrombosis (CVT) involving the left transverse and sigmoid sinuses and left internal jugular vein. Following bilateral optic nerve sheath fenestration (ONSF), she experienced improvement in vision and resolution of papilledema. In selected cases, ONSF is an effective surgical option for the treatment of papilledema due to CVT after medical treatment has failed. Journal of Neuro-Ophthalmology 2014;34:67–69 doi: 10.1097/WNO.0000000000000087 © 2013 by North American Neuro-Ophthalmology Society
C
erebral venous thrombosis (CVT) may be precipitated by multiple risk factors and present clinically in a highly variable manner. The sequelae of CVT are usually due to effects of increased intracranial pressure (ICP) and cerebral infarction. The most common clinical findings are the presence of focal neurologic signs or partial seizures (1). Papilledema secondary to increased ICP is another manifestation, reported in 50% of patients (1,2). Early detection and treatment of papilledema is important to prevent progressive loss of vision (3). Although medical management with carbonic anhydrase inhibitors, corticosteroids, and lumbar drainage is the initial treatment for increased ICP, surgery, including optic nerve sheath fenestration (ONSF) and ventriculoperitoneal shunting, may be required (2,4). CVT is rare in the adult population with reported prevalence of 1.32 cases per 100,000. It is even more uncommon in children occurring in 0.67 cases per 100,000 University of Miami Miller School of Medicine (JM), Miami, Florida; and Bascom Palmer Eye Institute (JHT, NJS, WWL), University of Miami Miller School of Medicine, Miami, Florida. The authors report no conflicts of interest. Address correspondence to Jennifer Murdock, MD, 1688 West Avenue #406, Miami Beach, FL 33139; E-mail: jmurdock@med. miami.edu Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
non-neonates (5,6). We describe a pediatric patient with multiple risk factors for thrombosis who developed papilledema and vision loss secondary to CVT. She was treated successfully with anticoagulation and ONSFs.
CASE REPORT A 16-year-old adolescent girl complained of throbbing frontal headaches with associated nausea and vomiting for 1 week. Her only medication was oral contraceptive pills, which she began taking 2 weeks previously. Magnetic resonance venography (MRV) revealed thrombosis of the left transverse and sigmoid sinuses and the left internal jugular vein. The patient was immediately started on subcutaneous enosparain sodium, and acetazolamide was added 12 days later, with the dose gradually increased to 1000 mg/d. Hematological studies demonstrated positive Factor V Leiden and prothrombin G20210A mutations, elevated Factor XII, and mildly lowered antithrombin III and protein S levels. Three weeks later, she developed horizontal diplopia and decreased vision. Opening pressure on lumbar puncture (LP) was 50 cm H2O and cerebrospinal fluid (CSF) analysis was normal. One month after the initial presentation, neuroophthalmologic evaluation revealed visual acuity of 20/80, right eye, and 20/200, left eye. Automated visual field (Humphery Visual Analyzer; Carl Zeiss Meditec, Dublin, CA; central 30-2 Swedish interactive threshold algorithm) showed enlarged blind spots and paracentral scotomas bilaterally (Fig. 1A). There was marked papilledema in each eye. The patient was referred to our institution for ONSF. At the time of our evaluation, visual acuity was 20/100, right eye, and 20/200, left eye. Pupillary reactions were normal, ocular motility revealed an abduction defect of the left eye, and high-grade bilateral papilledema was present (Fig. 1A). Medications consisted 48 mg of subcutaneous enoxaparin and 500 mg of acetazolamide, both taken twice daily. 67
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Clinical Observation
FIG. 1. Automated visual fields and appearance of optic discs: (A) preoperatively, (B) 1 week after right optic nerve sheath fenestration (ONSF) and 2 weeks after left ONSF, (C) 7 weeks after right ONSF and 8 weeks after left ONSF.
An ONSF of the left eye was performed the following day. After disinsertion of the medical rectus muscle, careful retraction of the orbital soft tissues was performed. A rectangular window was made in the optic nerve sheath, with a large gush of CSF exiting the fenestration. Because our patient was anticoagulated, extra care was taken during the procedure to maintain hemostasis and she was hospitalized overnight for observation. One week later, the patient reported resolution of diplopia and visual acuity was 20/100, right eye, and 20/70, left eye. Extraocular movements were full bilaterally. On funduscopy, there was still significant edema of the right optic disc but left optic disc edema was much improved. Automated visual fields showed minimal improvement. A right ONSF with the same surgical technique and precautions was performed the following day. One week later, visual acuity was 20/200, right eye, and 20/60, left eye, with full extraocular movements. Visual fields and optic disc appearance were improved bilaterally (Fig. 1B). Two months later, visual acuity was 20/40 in each eye with continued resolution of papilledema and visual field defects (Fig. 1C). At 4 months, acuity was 20/40 bilaterally and visual fields were stable, with complete resolution of papilledema.
DISCUSSION Although there is general consensus that the initial treatment for CVT is anticoagulation, controversy exists regarding the management of the secondary sequelae, including elevated ICP (1). Carbonic anhydrase inhibitors and diuretics are considered first-line treatment (2,4). If the patient is anticoagulated, then serial LPs are contraindicated. In our patient, raised ICP led to papilledema and progressive visual loss despite medical therapy. Our report 68
demonstrates that ONSF is a potential treatment for visual loss secondary to CVT. In addition, our patient illustrates how CVT can have multiple causative factors. She had an unusual combination of predisposing conditions, including Factor V Leiden mutation, G20210A mutation, Factor XII deficiency, decreased antithrombin III and protein S levels, and use of oral contraceptive pills (7–9). Alsuhaibani et al. (10) reported significant improvement in papilledema and stability of the visual field in the fellow eye after unilateral ONSF in patients with idiopathic intracranial hypertension. Although we agree with the approach of operating on the eye with the more severe papilledema and visual field loss in attempting to avoid bilateral surgery, this was not the case in our patient. Rather, bilateral ONSFs were required. The surgical treatment of papilledema in the setting of CVT has its own unique challenges. The use of anticoagulation complicates performing ONSF due to the increased risk of bleeding during the surgical procedure. Careful dissection during the procedure is essential to maintain hemostasis within the orbit. We recommend overnight observation following surgery to monitor for complications of bleeding. Measures can be taken to decrease the risk of hemorrhage by working with a hematologist or perhaps switching the patient to enoxaparin sodium, which has a shorter half-life than heparin.
REFERENCES 1. Crassard I, Bousser MG. Cerebral venous thrombosis. J Neuroophthalmol. 2004;24:156–163. 2. Biousse V, Tong F, Newman NJ. Cerebral venous thrombosis. Curr Treat Options Neurol. 2003;5:409–420. 3. Lee AG, Wall M. Papiledema: are we any nearer to consensus on pathogenesis and treatment? Curr Neurol Neurosci Rep. 2012;12:334–339. 4. Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology. 2000;107:1907–1012. Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Clinical Observation 5. Coutinho JM, Zuurbier SM, Aramideh M, Stam J. The incidence of cerebral venous thrombosis; a cross-sectional study. Stroke. 2012;43:3375–3377. 6. deVeber G, Andrew M, Adams C, Bjornson B, Booth F, Buckley DJ, Camfield CS, David M, Humphreys P, Langevin P, MacDonald EA, Gillet J, Meaney B, Shevell M, Sinclair DB, Yager J; Canadian Pediatric Ischemic Stroke Study Group. Cerebral sinovenous thrombosis in children. N Engl J Med. 2001;345:417–423. 7. Biousse V, Conard J, Brouzes C, Horellou MH, Ameri A, Bousser MG. Frequency of the 20210 G/A mutation in the 3’-untranslated region of the prothrombin gene in 35 cases of cerebral venous thrombosis. Stroke. 1998;29:1398–400.
Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
8. Poort SR, Rosendal FR, Reitsma PH, Vertina RM. A common genetic variation in the 3’-untranslated region of the prothrombin gene is associated with elevated plasma prothrombin levels and an increase in venous thrombosis. Blood. 1996;88:3698–3703. 9. Bavikatty NR, Killeen AA, Akel N, Normolle D, Schmaier AH. Association of prothrombin G20210A mutation with factor V Leiden in a Midwestern American population. Am J Clin Pathol. 2000;114:272–275. 10. Alsuhaibani AH, Carter KD, Nerad JA, Lee AG. Effect of optic nerve sheath fenestration on papilledema of the operated and the contralateral nonoperated eyes in idiopathic intracranial hypertension. Ophthalmology. 2011;118:412–414.
69
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Optic Nerve Sheath Fenestration for the Treatment of Papilledema Secondary to Cerebral Venous Thrombosis Jennifer Murdock, MD, Jonathan H. Tzu, MD, Norman J. Schatz, MD, Wendy W. Lee, MD
Abstract: A 16-year-old adolescent girl with multiple risk factors for thrombosis presented with acute onset of headache, decreased vision, and papilledema. Evaluation demonstrated cerebral venous thrombosis (CVT) involving the left transverse and sigmoid sinuses and left internal jugular vein. Following bilateral optic nerve sheath fenestration (ONSF), she experienced improvement in vision and resolution of papilledema. In selected cases, ONSF is an effective surgical option for the treatment of papilledema due to CVT after medical treatment has failed. Journal of Neuro-Ophthalmology 2014;34:67–69 doi: 10.1097/WNO.0000000000000087 © 2013 by North American Neuro-Ophthalmology Society
C
erebral venous thrombosis (CVT) may be precipitated by multiple risk factors and present clinically in a highly variable manner. The sequelae of CVT are usually due to effects of increased intracranial pressure (ICP) and cerebral infarction. The most common clinical findings are the presence of focal neurologic signs or partial seizures (1). Papilledema secondary to increased ICP is another manifestation, reported in 50% of patients (1,2). Early detection and treatment of papilledema is important to prevent progressive loss of vision (3). Although medical management with carbonic anhydrase inhibitors, corticosteroids, and lumbar drainage is the initial treatment for increased ICP, surgery, including optic nerve sheath fenestration (ONSF) and ventriculoperitoneal shunting, may be required (2,4). CVT is rare in the adult population with reported prevalence of 1.32 cases per 100,000. It is even more uncommon in children occurring in 0.67 cases per 100,000 University of Miami Miller School of Medicine (JM), Miami, Florida; and Bascom Palmer Eye Institute (JHT, NJS, WWL), University of Miami Miller School of Medicine, Miami, Florida. The authors report no conflicts of interest. Address correspondence to Jennifer Murdock, MD, 1688 West Avenue #406, Miami Beach, FL 33139; E-mail: jmurdock@med. miami.edu Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
non-neonates (5,6). We describe a pediatric patient with multiple risk factors for thrombosis who developed papilledema and vision loss secondary to CVT. She was treated successfully with anticoagulation and ONSFs.
CASE REPORT A 16-year-old adolescent girl complained of throbbing frontal headaches with associated nausea and vomiting for 1 week. Her only medication was oral contraceptive pills, which she began taking 2 weeks previously. Magnetic resonance venography (MRV) revealed thrombosis of the left transverse and sigmoid sinuses and the left internal jugular vein. The patient was immediately started on subcutaneous enosparain sodium, and acetazolamide was added 12 days later, with the dose gradually increased to 1000 mg/d. Hematological studies demonstrated positive Factor V Leiden and prothrombin G20210A mutations, elevated Factor XII, and mildly lowered antithrombin III and protein S levels. Three weeks later, she developed horizontal diplopia and decreased vision. Opening pressure on lumbar puncture (LP) was 50 cm H2O and cerebrospinal fluid (CSF) analysis was normal. One month after the initial presentation, neuroophthalmologic evaluation revealed visual acuity of 20/80, right eye, and 20/200, left eye. Automated visual field (Humphery Visual Analyzer; Carl Zeiss Meditec, Dublin, CA; central 30-2 Swedish interactive threshold algorithm) showed enlarged blind spots and paracentral scotomas bilaterally (Fig. 1A). There was marked papilledema in each eye. The patient was referred to our institution for ONSF. At the time of our evaluation, visual acuity was 20/100, right eye, and 20/200, left eye. Pupillary reactions were normal, ocular motility revealed an abduction defect of the left eye, and high-grade bilateral papilledema was present (Fig. 1A). Medications consisted 48 mg of subcutaneous enoxaparin and 500 mg of acetazolamide, both taken twice daily. 67
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Clinical Observation
FIG. 1. Automated visual fields and appearance of optic discs: (A) preoperatively, (B) 1 week after right optic nerve sheath fenestration (ONSF) and 2 weeks after left ONSF, (C) 7 weeks after right ONSF and 8 weeks after left ONSF.
An ONSF of the left eye was performed the following day. After disinsertion of the medical rectus muscle, careful retraction of the orbital soft tissues was performed. A rectangular window was made in the optic nerve sheath, with a large gush of CSF exiting the fenestration. Because our patient was anticoagulated, extra care was taken during the procedure to maintain hemostasis and she was hospitalized overnight for observation. One week later, the patient reported resolution of diplopia and visual acuity was 20/100, right eye, and 20/70, left eye. Extraocular movements were full bilaterally. On funduscopy, there was still significant edema of the right optic disc but left optic disc edema was much improved. Automated visual fields showed minimal improvement. A right ONSF with the same surgical technique and precautions was performed the following day. One week later, visual acuity was 20/200, right eye, and 20/60, left eye, with full extraocular movements. Visual fields and optic disc appearance were improved bilaterally (Fig. 1B). Two months later, visual acuity was 20/40 in each eye with continued resolution of papilledema and visual field defects (Fig. 1C). At 4 months, acuity was 20/40 bilaterally and visual fields were stable, with complete resolution of papilledema.
DISCUSSION Although there is general consensus that the initial treatment for CVT is anticoagulation, controversy exists regarding the management of the secondary sequelae, including elevated ICP (1). Carbonic anhydrase inhibitors and diuretics are considered first-line treatment (2,4). If the patient is anticoagulated, then serial LPs are contraindicated. In our patient, raised ICP led to papilledema and progressive visual loss despite medical therapy. Our report 68
demonstrates that ONSF is a potential treatment for visual loss secondary to CVT. In addition, our patient illustrates how CVT can have multiple causative factors. She had an unusual combination of predisposing conditions, including Factor V Leiden mutation, G20210A mutation, Factor XII deficiency, decreased antithrombin III and protein S levels, and use of oral contraceptive pills (7–9). Alsuhaibani et al. (10) reported significant improvement in papilledema and stability of the visual field in the fellow eye after unilateral ONSF in patients with idiopathic intracranial hypertension. Although we agree with the approach of operating on the eye with the more severe papilledema and visual field loss in attempting to avoid bilateral surgery, this was not the case in our patient. Rather, bilateral ONSFs were required. The surgical treatment of papilledema in the setting of CVT has its own unique challenges. The use of anticoagulation complicates performing ONSF due to the increased risk of bleeding during the surgical procedure. Careful dissection during the procedure is essential to maintain hemostasis within the orbit. We recommend overnight observation following surgery to monitor for complications of bleeding. Measures can be taken to decrease the risk of hemorrhage by working with a hematologist or perhaps switching the patient to enoxaparin sodium, which has a shorter half-life than heparin.
REFERENCES 1. Crassard I, Bousser MG. Cerebral venous thrombosis. J Neuroophthalmol. 2004;24:156–163. 2. Biousse V, Tong F, Newman NJ. Cerebral venous thrombosis. Curr Treat Options Neurol. 2003;5:409–420. 3. Lee AG, Wall M. Papiledema: are we any nearer to consensus on pathogenesis and treatment? Curr Neurol Neurosci Rep. 2012;12:334–339. 4. Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Ophthalmology. 2000;107:1907–1012. Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
Clinical Observation 5. Coutinho JM, Zuurbier SM, Aramideh M, Stam J. The incidence of cerebral venous thrombosis; a cross-sectional study. Stroke. 2012;43:3375–3377. 6. deVeber G, Andrew M, Adams C, Bjornson B, Booth F, Buckley DJ, Camfield CS, David M, Humphreys P, Langevin P, MacDonald EA, Gillet J, Meaney B, Shevell M, Sinclair DB, Yager J; Canadian Pediatric Ischemic Stroke Study Group. Cerebral sinovenous thrombosis in children. N Engl J Med. 2001;345:417–423. 7. Biousse V, Conard J, Brouzes C, Horellou MH, Ameri A, Bousser MG. Frequency of the 20210 G/A mutation in the 3’-untranslated region of the prothrombin gene in 35 cases of cerebral venous thrombosis. Stroke. 1998;29:1398–400.
Murdock et al: J Neuro-Ophthalmol 2014; 34: 67-69
8. Poort SR, Rosendal FR, Reitsma PH, Vertina RM. A common genetic variation in the 3’-untranslated region of the prothrombin gene is associated with elevated plasma prothrombin levels and an increase in venous thrombosis. Blood. 1996;88:3698–3703. 9. Bavikatty NR, Killeen AA, Akel N, Normolle D, Schmaier AH. Association of prothrombin G20210A mutation with factor V Leiden in a Midwestern American population. Am J Clin Pathol. 2000;114:272–275. 10. Alsuhaibani AH, Carter KD, Nerad JA, Lee AG. Effect of optic nerve sheath fenestration on papilledema of the operated and the contralateral nonoperated eyes in idiopathic intracranial hypertension. Ophthalmology. 2011;118:412–414.
69
Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.
