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Show Journal of Neuro- Ophthalmology 14( 4): 193- 195, 1994. i 1994 Raven Press, Ltd., New York Macular Subretinal Neovascular Membrane Associated with Pseudotumor Cerebri Yonca A. Akova, M. D., Tiilay Kansu, M. D., Zeliha Yazar, M. D., ( lagla Atabay, M. D., Yasemin Karagoz, M. D., and Sunay Duman, M. D. We report on the development of juxtapapillary subretinal neovascular membrane and permanent severe visual loss in a patient with pseudotumor cerebri. The patient was managed by a lumboperitoneal shunt. After surgery, despite resolving papilledema and intracranial pressure control, the membrane had enlarged rapidly to involve the foveal avascular zone, and resulted in rapid visual loss. The membrane slowly regressed, and was replaced by fibrous tissue at the ninth month, causing permanent severe visual loss. Key Words: Pseudotumor cerebri- Intracranial pres- Pseudotumor cerebri is characterized by an increased intracranial pressure with normal neurora-diologic studies and cerebrospinal components. Papilledema that accompanies pseudotumor cerebri may cause permanent visual loss, and the most common causes for visual loss as a result of pseudotumor cerebri include secondary optic atrophy, visual field loss, and ischemic optic neuropathy ( 1). On the contrary, the most uncommon reason for visual loss is subretinal peripapillary neovascular membrane ( PSRNVM) development, and subsequent hemorrhage. There are only a few reports in the literature in this subject, and the relationship between intracranial pressure levels, and neovascular membrane development ( 2- 4). We report a patient with pseudotumor cerebri who developed PSRNVM and very rapid visual loss due to macular involvement. This severe visual loss was permanent in spite of intracranial pressure control and involution of membrane. Department of Ophthalmology ( Y. A. A., Z. Y., Y. K., S. D.), Neuro- ophthalmology Unit, Ankara Hospital, Cebeci; and Department of Neurology ( Y. A. A., T. K., C. A., Y. K.), Neuro-ophthalmology Unit, Hacettepe University, School of Medicine, Sihhiye, Ankara, Turkey. Address correspondence and reprint requests to Dr. Yonca Aydin Akova, 23 Sokak, No: l/ 8, Aydin Apt, Eryaman, 06793 Ankara, Turkey. CASE REPORT A 33- year- old white female was seen in Hacettepe University Neuro- ophthalmology Unit for evaluation of severe headaches. The patient had a history of long- standing headaches, intermittent dizziness, and transient obscuration of vision bilaterally associated with posture changes. The patient's physical and neurologic examination were found unremarkable. The blood pressure was within normal limits ( 130/ 85 mmHg). Neuro- ophthalmic examination revealed a visual acuity of 20/ 20 each eye. Slit- lamp examination, intraocular pressure, ocular motility, and pupillary reactions were normal. Color vision testing with Ishihara plates was normal ( 11.5/ 12). In fundus examination both optic disks showed edema, hyper- 293 294 Y. A. AKOVA ET AL. emia, blurring of the margins, and obscuration of the vessels margins. No spontaneous venous pulsation was seen on either optic disk. Visual field examination disclosed an enlarged blind spot in both eyes. Results of computed axial tomography of the orbits and brain showed no abnormality. Initial lumbar puncture revealed an opening pressure of 250 mmH20, cerebrospinal fluid ( CSF) was clear and without cells. Microbiologic and biochemical assays were unremarkable. Two days later, a repeated lumbar puncture showed an opening pressure of 300 mmH20. A diagnosis of pseudotumor was established, and the patient was given oral acetazolamide, 250 mg four times a day. During the follow- up, the patient's headaches persisted, and optic disc edema did not resolve. Serial frequent lumbar punctures had failed to control the disease, and the patient was offered a lumboperi-toneal shunt operation. The patient declined the surgery. One month later, the patient returned complaining of rapid visual loss in the left eye. Visual acuity was measured 20/ 20 in the right eye and 20/ 100 in the left eye. Fundus examination revealed more pronounced optic disc edema especially in the left eye. Temporal to the disc, there was a PSRNVM and surrounding hemorrhage extending to the macula. Fundus fluorescein angiography ( FFA) confirmed the PSRNVM, and because it was threatening the foveola, the patient was offered argon laser photocoagulation ( Fig. 1). She requested lumboperitoneal shunt and the patient FIG. 1. Left eye. Fluorescein angiogram showing extensive peripapillary subretinal neovascular membrane at the temporal membrane at the temporal border of the optic disc, involving the foveal avascular zone. FIG. 2. Left eye after partial spontaneous involution of the subretinal neovascular membrane. was admitted to the Neurosurgery Department for lumboperitoneal shunt, and postponed the laser therapy. Two weeks later, the patient returned with a complaint of sudden visual loss in the left eye to the level of 20/ 200 and FFA demonstrated that the membrane enlarged to foveal avascular zone despite rapid resolving papilledema. Because the membrane extended to the foveola, the photocoagulation therapy had to be canceled. During follow- up examinations papilledema and hemorrhage resolved totally. One year later, the patient's visual acuity was 20/ 100 in the left eye. The optic disc were normal. The membrane was partly regressed, and fibrosed ( Fig. 2). DISCUSSION A number of macular diseases have been reported in association with pseudotumor cerebri ( 3). These include choroidal folds, macular edema, macular stars, choroidal neovascularization, and hemorrhage. Juxtapapillary subretinal neovascular membrane formation in pseudotumor cerebri is very rare. The first case of subretinal neovascular membrane, which was treated by argon laser photocoagulation because of the extension toward to the fovea secondary to pseudotumor cerebri, was reported by Jamison ( 2). The patient's visual acuity deteriorated moderately following laser therapy. Morse and colleagues ( 4) reported the development of bilateral juxtapapillary subretinal neovascularization associated with recalcitrant pseudotumor cerebri. In one eye, neovascular membrane spontaneously involuted; the other eye was treated with photocoagulation because of rapid enlargement of the membrane and threatening of the / Neuro- Ophthalmol, Vol. 14, No. 4, 1994 PERIPAPILLARY MEMBRANE IN PSEUDOTUMOR CEREBRI 195 fovea. Morris and colleagues ( 5) have reported a patient with permanent visual loss secondary to subretinal hemorrhage associated with pseudotumor cerebri. Coppeto and coworkers ( 6) reported that bilateral PSRNVM formation with rapid enlargement of the membrane secondary to severe, recalcitrant pseudotumor cerebri. The membrane involuted spontaneously, despite persistence of elevated intracranial pressure, without causing any permanent visual loss. They claimed that PSRNVM associated with pseudotumor cerebri tend to involute spontaneously without causing serious visual loss, and the indications for photocoagulation of PSRNVM associated with pseudotumor cerebri should be very limited in comparison to therapy of PSRNVM associated with other underlying etiologies. In this case, PSRNVM developed secondary to a severe pseudotumor cerebri, and involved the foveal avascular zone very rapidly in spite of the control of the intracranial pressure. Membrane formation was unilateral and it caused permanent visual loss, and scarring in the fovea. Unilateral sub-retinal hemorrhage and permanent visual loss was reported in another case by Corbett and associates ( 1). This is the third case of PSRNVM in the literature causing permanent visual loss due to pseudotumor cerebri. It appears that the enlargement and involution of membrane as well as permanent damage to the vision may show variations from case to case. Visual loss may be very rapid, as seen in our patient. One should consider treating patients with pseudotumor cerebri aggressively to lower the intracranial pressure before causing PSRNVM. PSRNVM is a potential threat to central vision, and photocoagulation should be applied, if it threatens a foveal avascular zone. Once the membrane is closer than 200 fjim from the center of the foveal avascular zone, photocoagulation is no longer safe. We could not treat this patient because of her poor compliance and follow- up. After the involvement of avascular zone, we observed the involution of membrane, but it caused severe permanent visual loss, although it became fibrotic at long- term follow- up. In conclusion, this case represents permanent severe visual loss due to PSRNVM associated with chronic pseudotumor cerebri and shows very rapid enlargement. REFERENCES 1. Corbett JJ, Savino PJ, Kansu T, Schatz NJ, Orr LS, Hopson D. Visual loss in pseudotumor cerebri; Follow- up of 57 patients from five to 41 years and a profile of 14 patients with permanent severe visual loss. Arch Neurol 1982; 39: 461- 74. 2. Jamison RR. Subretinal neovascularization and papilledema associated with pseudotumor cerebri. Am J Ophthalmol 1978; 85: 78- 81. 3. Gittinger JW, Asdourian GK. Macular abnormalities in papilledema from pseudotumor cerebri. Ophthalmology 1989; 96: 192- 4. 4. Morse PH, Leveille AS, Antel JP, Burch JV. Bilateral juxta-papillary neovascularization associated with pseudotumor cerebri. Am J Ophthalmol 1981; 91: 312- 17. 5. Morris AT, Sanders MD. Macular changes resulting from papilledema. Br ] Ophthalmol 1980; 64: 211- 16. 6. Coppeto JR, Monteiro MLR. Juxtapapillary subretinal hemorrhages in pseudotumor cerebri. / Clin Neuro- ophthalmol 1985; 5: 45- 53. / Neuro- Ophthalmol, Vol. 14, No. 4, 1994 |
