A 15-year-old boy with a body mass index of 41 kg/m2 reported a 2-day history of a “black spot” in the center of his right visual field. His visual acuity was 20/200, right eye, and 20/30, left eye. There was no relative afferent pupillary defect but color vision was reduced in the right eye. Ophthalmoscopy revealed bilateral papilledema and subretinal blood temporal to the right optic disc extending beneath the fovea (Fig. 1A–B). Optical coherence tomography (Fig. 1C–D) and fluorescein angiography (Fig 1E) confirmed the presence of a peripapillary choroidal neovascular membrane (ppCNVM). Computed tomography and magnetic resonance imaging of the brain and magnetic resonance venography were unremarkable. Lumbar puncture opening pressure was 37 cm H2O and cerebrospinal fluid (CSF) composition was normal. Due to a possible sulfa compound allergy, acetazolamide was not prescribed. The patient was counseled on the importance of weight loss, and given an intravitreal injection of 1.25 mg/0.1 mL bevacizumab (Avastin; Genentech, South San Francisco, CA).
Four weeks later, his vision was 20/40 in the right eye and the papilledema was unchanged. The patient was started on 250 mg of acetazolamide 4 times daily when the allergy history was scrutinized and deemed unconvincing. He received a second and third injection of bevacizumab at 4-week intervals with improvement in the appearance of the peripapillary retina (Fig 2A). A month after the third injection, his visual acuity remained 20/40 but fluorescein angiography revealed minimal leakage from the ppCNVM (Fig 2C). The patient was given 3 additional monthly injections of intravitreal bevacizumab and with 7 months of follow-up, the acuity is 20/40 in the right eye, with diminished papilledema and the ppCNVM is inactive.
ppCNVM is an infrequent finding in patients with idiopathic intracranial hypertension (IIH), reported to occur in 0.5% of cases (1) and rare in the pediatric population (2). There is limited evidence to guide management and proposed treatments include measures to lower intracranial pressure and targeted therapy including argon laser photocoagulation, photodynamic therapy, surgical excision, and intravitreal injection of anti-vascular endothelial growth factor (VEGF) compounds (1–4).
In IIH, successful resolution of ppCNVM has been reported with lowering of intracranial pressure (2). However, at presentation our patient had subfoveal hemorrhage that called for urgent treatment. The reported allergy to sulfa compounds initially limited our treatment options. We did not feel that a surgical CSF diversion procedure was warranted given the modest level of visual impairment directly attributable to the CNVM. Our patient tolerated the intravitreal injections well with no local adverse reactions or known systemic complications although 6 injections were required. This differs from the rapid regression of ppCNVM after a single injection of intravitreal bevacizumab in a 30-year-old woman reported by Jamerson et al. (4). Their patient received concomitant oral acetazolamide which may have contributed to the rapid improvement. Sisk et al. (5) have provided the most data on the use of intravitreal bevacizumab in pediatric retinal and choroidal disorders. They showed efficacy and safety in 24 eyes where the goal was reduction of excess retinal fluid and exudation. Five or more injections were used in 5 of the 24 eyes. No ocular or systemic adverse events were reported.
Our case report supports the use of anti-VEGF agents for ppCNVM in pediatric patients with IIH. Since our patient was postpubescent, caution should be used in translating our observations to pre-pubescent patients with IIH where disease characteristics may differ.
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