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Show PHOTO ESSAY Subretinal Hemorrhage From a Peripapillary Choroidal Neovascular Membrane in Papilledema Caused by Idiopathic Intracranial Hypertension Busaba Sathornsumetee, MD, Adam Webb, MD, Donna L. Hill, MD, Nancy J. Newman, MD, and Valerie Biousse, MD FIG. 1. A. One week after initial presentation, chronic optic disc edema is evident in both eyes as well as a subretinal hemorrhage surrounding the left optic disc. B. Two months after left optic nerve sheath fenestration, optic disc edema has lessened in both eyes allowing better visualization of a peripapillary whitish elevation in the left eye. Departments of Ophthalmology ( BS, DLH, NJN, VB), Neurology ( AW, NJN, VB), and Neurological Surgery ( NJN), Emory University, Atlanta, Georgia. This study was supported in part by a departmental grant ( Department of Ophthalmology) from Research to Prevent Blindness, Inc., New York, NY, and by core grant P30- EY06360 ( Department of Ophthalmology) from the National Institutes of Health, Bethesda, Maryland. Dr. Newman is a recipient of a Research to Prevent Blindness Lew R. Wasserman Merit Award. Dr. Hill was supported by unrestricted educational grants from NovaVision and TEVA Neuroscience. Address correspondence to Valerie Biousse, MD, Neuro- Ophthalmology Unit, Emory Eye Center, 1365- B Clifton Rd., Atlanta, GA 30322; E- mail: vbiouss@ emory. edu Abstract: A 42- year- old man with idiopathic intracranial hypertension and chronic papilledema had severe visual loss in his left eye caused by subretinal bleeding from a peripapillary choroidal neovascular membrane ( CNVM). After optic nerve sheath fenestration in his left eye, the papilledema improved allowing improved visualization of the CNVM. Visual function did not improve after the surgery. CNVM can complicate chronic papilledema and account for sudden worsening of vision. The appropriate management of this type of CNVM is unresolved. J Neuro- Ophthalmol, Vol. 26, No. 3, 2006 197 J Neuro- Ophthalmol, Vol. 26, No. 3, 2006 Sathornsumetee et al ( J Neuro- Ophthalmol 2006; 26: 197- 199) A42- year- old man presented with a 1- month history of painless vision loss in both eyes. There were no other neurologic symptoms. Blood pressure was normal. Best- corrected visual acuity was 20/ 30 in the right eye and 20/ 300 in the left eye without relative afferent pupillary defect ( RAPD). Ophthalmoscopic examination showed bilateral disc edema. Neurologic examination was otherwise normal. Complete blood cell count, metabolic profile, erythrocyte sedimentation rate, and rapid plasma reagin were normal. Brain and orbit MRI with MRV was normal. Lumbar puncture revealed an opening pressure of 30 cm H20 and a normal formula. One week later, visual acuity was 20/ 30 in the right eye and 20/ 100 in the left eye, again without RAPD. Ophthalmoscopic examination showed persistent bilateral disc edema and a peripapillary temporal subretinal hemorrhage in the left eye extending to the fovea. A gray- white opacity present on the temporal edge of the optic disc merged with the optic disc edema ( Fig. 1A). Humphrey visual fields revealed an enlarged blind spot in the right eye with constriction, and an enlarged blind spot in the left eye with nasal depression. He was treated with acetazolamide, which he did not tolerate. Therefore, he underwent an uncomplicated optic nerve sheath fenestration in the left eye. Two months later, visual function had not improved in the left eye. He continued to deny any headache or associated symptoms. Visual acuity was 20/ 20 in the right eye and counting fingers at 3 feet in the left eye. There was a 0.6 log unit left RAPD. The visual field deficit was stable in the right eye and had worsened in the left eye. Ophthalmoscopic examination now showed a more obvious temporal peripapillary gray- white subretinal opacity and hemorrhage ( Fig. IB). The gray- white opacity took up intravenous fluorescein, confirming that it was a choroidal neovascular membrane ( Fig. 2). The subretinal hemorrhage blocked choroidal transmission. The patient refused further treatment. Nine months later, his visual function was unchanged. This case is presented to highlight the fact that a peripapillary choroidal neovascular membrane may be a cause of sudden visual loss in the setting of papilledema. The membrane was not initially obvious; it became more evident as disc edema resolved after optic nerve sheath fenestration. Peripapillary choroidal neovascular membrane is an exceedingly rare complication of chronic papilledema. Fewer than 15 cases have been reported ( 1- 9). The presumed pathogenesis is pressure deformity of the border of Bruch's FIG. 2. Fluorescein angiography confirms that the peripapillary whitish elevation is a leaking choroidal neovascular membrane. membrane at the level of the optic nerve head creating a discontinuity of the normal anatomic apposition of the chorioretinal layers. This anatomic dehiscence, coupled with hypoxia created by axonal swelling, may promote angiogenesis leading to the formation of a neovascular membrane ( 4). These peripapillary choroidal neovascular membranes have been treated with laser, photodynamic therapy, or surgery to prevent subfoveal extension ( 1,4,7,9). This treatment has resulted in regression of the membrane or improvement of visual acuity in approximately 50% of cases. Others have suggested that these membranes may not need treatment because they have a good prognosis and may even spontaneously regress once papilledema has improved ( 2,5,6,8,9). Among the 7 patients left untreated ( 2,5,6,8,9), 5 patients had a good visual outcome ( 2,5,6,9), 1 patient had extension of the choroidal neovascular membrane toward the fovea ( 9), and 1 patient with a subfoveal neovascular membrane had worsening of vision ( 8). None had recurrent hemorrhage. Although the optic nerve sheath fenestration resulted in dramatic improvement of the papilledema in our patient, the choroidal neovascular membrane had not resolved 9 months later. REFERENCES 1. Jamison RR. Subretinal neovascularization and papilledema associated with pseudotumor cerebri. Am J Ophthalmol 1978; 85: 78- 81. 2. Troost BT, Sufit RL, Grand MG. Sudden monocular visual loss in pseudotumor cerebri. Arch Neurol 1979; 36: 440- 2. 3. Morris AT, Sanders MD. Macular changes resulting from papilledema. Br J Ophthalmol 1980; 64: 211- 6. 198 © 2006 Lippincott Williams & Wilkins Subretinal Hemorrhage in Papilledema J Neuro- Ophthalmol, Vol. 26, No. 3, 2006 4. Morse PH, Leveille AS, Antel JP, et al. Bilateral juxtapapillary subretinal neovascularization associated with pseudotumor cerebri. Am J Ophthalmol 1981; 91: 312- 7. 5. Caballero- Presencia A, Diaz- Guia E, Martinez- Perez M, et al. Neovascularisation sous- retinienne juxtapapillaire bilaterale dans un cas de pseudotumor cerebri. JFr Ophthalmol 1986; 9: 105- 10. 6. Coppeto JR, Monteiro ML. Juxtapapillary subretinal hemorrhages in pseudotumor cerebri. J Clin Neuro- Ophthalmol 1985; 5: 45- 53. 7. Castellarin AA, Sugino IK, Nasir M, et al. Clinicopathological correlation of an excised choroidal neovascular membrane in pseudotumor cerebri. Br J Ophthalmol 1997; 81: 994- 1000. 8. Akova YA, Kansu T, Yazar Z, et al. Macular subretinal neovascular membrane associated with pseudotumor cerebri. J Neuroophthalmol 1994; 14: 193- 5. 9. Wendel L, Lee AG, Boldt HC, et al. Subretinal neovascular membrane in idiopathic intracranial hypertension. Am J Ophthalmol 1996; 141: 573^. 199 |