29-year-old woman presents with subacute vision loss and headaches
The patient was previously diagnosed with idiopathic intracranial hypertension.
A 29-year-old obese white woman with a known history of idiopathic intracranial hypertension, or IIH, presented to the New England Eye Center with 1 month of progressively decreasing vision in her right eye and positional headaches. She was diagnosed with IIH 5 years prior, after presenting with transient visual obscurations in her right eye and headaches. She was noncompliant with oral medications and had a recent 45-pound weight gain.
On examination, best corrected visual acuity was 20/200 in the right eye and 20/20 in the left eye. The patient had an afferent pupillary defect and color desaturation in the right eye. Humphrey visual field 30-2 testing revealed paracentral loss and an inferotemporal scotoma in the right eye; testing was normal in the left eye. Fundus examination was notable for bilateral optic disc edema and venous tortuosity. There was a superotemporal peripapillary subretinal hemorrhage with subretinal fluid and exudate in the right eye (Figure 1a), which was consistent with choroidal neovascularization on fluorescein angiography (Figure 1g). Spectral-domain OCT of the macula revealed a distorted foveal contour with intraretinal and subretinal fluid in the right eye (Figure 1d).
What is your diagnosis?
Blurred vision, headaches
The differential diagnosis of blurred vision in a young woman with bilateral optic disc edema includes papilledema secondary to increased intracranial pressure, papillitis, hypertensive optic neuropathy, diabetic papillopathy, optic disc infiltration (secondary to sarcoidosis, tuberculosis, inflammatory disease or tumor), and idiopathic intracranial hypertension, among others.
Although the patient already had a diagnosis of IIH, considering the unilaterality of symptoms and choroidal neovascularization were atypical, repeat neuroimaging with MRI and magnetic resonance venography of the brain and orbits with gadolinium was obtained. Imaging was unremarkable for a secondary cause of increased intracranial pressure, and she was diagnosed with papilledema-associated peripapillary choroidal neovascularization (PP-CNV) secondary to IIH.
The patient was started on 1 g of acetazolamide daily, and she was given an intravitreal injection of Lucentis (ranibizumab, Genentech) in the right eye. Four weeks later, her BCVA was 20/100, and she reported improvement in symptoms. On examination, there was regression of the choroidal neovascular membrane and subretinal hemorrhage in the right eye (Figure 1b), with development of subretinal exudate. SD-OCT revealed an improvement in central retinal thickness from 462 µm to 207 µm, with resolution of the subretinal fluid (Figures 1e and 1g). She was continued on 1 g of oral acetazolamide to treat the persistent papilledema and was given a second ranibizumab injection.
Six weeks after the second injection, her symptoms had improved and her vision was stable at 20/100. She had resolution of subfoveal fluid and improvement in subretinal exudate (Figures 1c and 1f). She received a third ranibizumab injection in the right eye for mild residual fluorescein leakage of the peripapillary choroidal neovascularization.
Five months after presentation, the patient’s vision improved to 20/60 with complete fibrotic involution of the PP-CNV.
PP-CNV secondary to disc edema is uncommon and to our knowledge has been described in IIH only 17 times. In all cases, long-standing disc edema was present before the onset of PP-CNV.
The pathogenesis of choroidal neovascularization in the setting of papilledema is unknown; however, resolution after VEGF therapy supports the idea that the initial PP-CNV formation in IIH is VEGF driven and may represent a process that is unique to chronic papilledema. Studies demonstrate that crowding of neural and vascular elements in the peripapillary area may increase the potential space between Bruch’s membrane and the optic disc, resulting in deformation and dehiscence of Bruch’s membrane and allowing inward vessel growth from the choriocapillaris. Further, the edematous optic nerve may disrupt axoplasmic flow and produce relative anoxia, resulting in release of angiogenic factors.
The natural history of PP-CNV associated with papilledema in IIH varies considerably. Despite lowering intracranial pressure with resolution of papilledema, expansion of PP-CNV into the fovea has been reported. Therefore, treatment to prevent permanent vision loss is indicated.
Anti-VEGF agents are effective to treat PP-CNV from other causes, such as age-related maculopathy. Although possibly resulting from different pathologic mechanisms, four case reports have shown positive results with Avastin (bevacizumab, Genentech) inducing regression of PP-CNV in IIH, with long-term stability of results after one to two injections.
In our patient, complete involution of PP-CNV after three injections combined with treatment of papilledema supports the idea that prolonged anti-VEGF therapy may not be required in this underlying etiology.
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- For more information:
- Kendra Klein, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Kristen E. Dunbar, MD, and Kendra Klein, MD. They can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston MA 02111; website: www.neec.com.