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IIH-associated choroidal neovascular membranes

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Title: Idiopathic intracranial hypertension (IIH)-associated choroidal neovascular membranes
Authors: Christopher D. Conrady, MD, PhD, Anastasia Neufeld, MD, M.E. Hartnett, MD, Kathleen Digre, MD
Date: 07/25/2017
Keywords/Main Subjects: Idiopathic intracranial hypertension; Choroidal neovascular membrane; Optic nerve swelling
Secondary CORE Category: Home / Neuro-Ophthalmology / Causes of Decreased Vision
Diagnosis: Choroidal neovascular membrane secondary to chronic optic disc swelling
Description of Case: BG is a 13-year-old, right-handed girl with past medical history of Hashimoto’s thyroiditis who developed acute blurring of the vision in the right eye while reading a book at school.  She was seen by outside providers who noted bilateral disc edema and referred her to the emergency department for further evaluation.   At time of presentation, she denied pulsatile tinnitus, transient visual obscurations, headache, and diplopia.  She denied any new medications but did endorse a 50 lb. weight gain.

On examination, visual acuity was count fingers at 4’ in the right eye and 20/20 in the left.  Intraocular pressure was 22 mmHg and 20 mmHg in the right and left eye respectively.  There was a small (0.3-0.6 log units), right-sided afferent pupillary defect .Hardy-Rand-Ritter color plates were significantly depressed in the right but not left eye.  Flicker fusion was severely depressed at 9 Hz in the right and

normal in the left at 31 Hz.  Anterior segment was unremarkable.  Dilated eye examination noted significant peripapillary hemorrhages and 360 degrees of edema of the optic nerve in the right eye (Figure 1).  The left eye was notable for stage II disc edema.

A Humphrey visual field of the left eye noted an enlarged blind spot, while a Goldman visual field noted a large central scotoma of the right eye (Figure 1c-d).  Fluorescein angiography noted a leaking lesion consistent with a peripapillary choroidal neovascular membrane (Figure 2a-c) with fluid extending into the fovea (Figure 2e).  B-scan of the globes did not identify optic nerve head drusen (Figure 2d).

The patient was referred to the retina service that performed an intravitreal avastin injection in the right eye.  Within a few months, vision returned in the right eye to 20/40 with significant improvement of cecocentral scotoma and resolution of the subretinal fluid (Figure 3a-e).

 

Figure 1: Acute vision loss in the right eye with bilateral disc edema.

(a-b) Fundus photographs of the left and right eye showed bilateral disc edema.  In the right eye (b), there is peripapillary subretinal blood and a choroidal neovascular membrane (yellow arrow).  (c) Humphrey visual field noted an enlarged blind spot in the left eye.  (d) Goldman visual field noted a central scotoma of the right eye.  Left column, left eye; right column, right eye.

 

Figure 2: Choroidal neovascular membrane with surrounding subretinal fluid and no signs of optic nerve head drusen of the right eye.

(a-c) Fluorescein angiogram of the right eye noted a peripapillary lesion that leaked late consistent with a choroidal neovascular membrane (red arrows).  (d) There were no signs of optic nerve head drusen on ultrasound.  (e) Macular OCT identified subretinal fluid extending from the optic nerve into the macula (yellow arrow).

 

 

Figure 3: Resolution of subretinal fluid with improvement in vision and visual field in the right eye.

(a) Follow up macular OCT of the right eye noted resolution of subretinal fluid in the right eye.  Optic disc edema (b-c) and visual fields were also improving in both eyes (d-e).  Left column, left eye; right column, right eye.

Summary of the Case:

  1. Choroidal neovascular membranes can complicate longstanding optic nerve edema in children.
  2. These membranes respond very well to anti-VEGF therapy.

Faculty Approval by: Dr. Kathleen Digre
Identifier: Moran_CORE_24138
Copyright statement: Copyright Conrady, ©2017. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/