Man presents with unilateral decreased vision
On initial examination, the left eye had a large subretinal hemorrhage extending from the nerve to the macula.
A 57-year-old Cambodian man with a medical history of diabetes, hypertension, hyperlipidemia and an unknown cardiac procedure presented with 2 weeks of acute blurry vision in the left eye. He reported having the flu 2 weeks prior with severe coughing. He had no eye pain or redness. He had no history of eye trauma or eye surgery. His medications included aspirin, atorvastatin, multiple antihypertensives and warfarin. He had no known family history of eye disease. He was a former cigarette smoker but did not drink alcohol or use illicit drugs.
The patient’s visual acuity on initial presentation was 20/20 in the right eye and 20/200 in the left eye. Pupils were symmetric with no afferent pupillary defect. IOPs were 10 mm Hg and 11 mm Hg in the right and left eyes, respectively. On anterior segment exam, both eyes had 1+ nuclear sclerotic and 2+ cortical spoking cataracts. On dilated fundus exam, the right eye was unremarkable. The left eye had a large subretinal hemorrhage extending from the nerve to the macula with adjacent inferior subretinal fluid and exudative retinal detachment (Figure 1). No retinal breaks were observed on 360° scleral depressed exam. OCT of the left eye showed subretinal fluid and hemorrhage, type 1 choroidal neovascular (CNV) membrane, multiple pigment epithelial detachments (PED), M-shaped polyps and PED notches (Figure 2). The choroid was thick on OCT. Fluorescein angiography (FA) of the left eye showed normal filling times, blockage from hemorrhage and no leakage (Figure 3). Indocyanine green angiography (ICG) showed a possible polyp in the right eye and a polyp in the temporal macula of the left eye (Figure 4).
The patient was given intravitreal Avastin (bevacizumab, Genentech) in the left eye. Other options discussed included pars plana vitrectomy with administration of tissue plasminogen activator and pneumatic displacement of submacular hemorrhage.
Three weeks later, he returned urgently with 1 day of severe left eye pain. He stated he had lost vision in the left eye 2 weeks prior.
Visual acuity at this examination was 20/25 in the right eye and no light perception in the left eye. The left pupil was fixed at 5 mm and had developed an afferent pupillary defect. IOP was 16 mm Hg in the right eye and 62 mm Hg in the left eye. Anterior segment exam of the right eye was unchanged. The ciliary body and scleral spur were visible on gonioscopy of the right eye. The left eye had diffuse conjunctival injection, microcystic corneal edema and diffuse stromal edema. The anterior chamber had 3+ red flare and 2+ red blood cells and was centrally deep. The iris was flat with no neovascularization. The angle was not visible on gonioscopy due to red-tinted haze, but there was no obvious neovascularization of the angle or peripheral anterior synechiae. Fundus exam was unchanged in the right eye. There was no posterior view in the left eye. B-scan ultrasound showed vitreous hemorrhage but no obvious retinal detachment (Figure 5).
Elevated IOP did not respond to medical therapy. Laser peripheral iridotomy (LPI) was performed on the left eye to rule out primary angle closure. IOP dropped to 22 mm Hg. The patient was started on maximum medical therapy. However, he returned the next day with recurrent elevated IOP to 58 mm Hg. The LPI was patent. After fluid was removed from the anterior chamber, the anterior chamber became shallow peripherally and centrally. The IOP quickly rose again to the 40s mm Hg.
The patient was taken to the operating room for emergency pars plana vitrectomy, anterior chamber washout and cyclophotocoagulation (CPC) for uncontrolled IOP and pain. Immediately after the trocars were placed, vitreous hemorrhage refluxed through the trocars, exiting the globe.
What is your diagnosis?
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Our patient was diagnosed with polypoidal choroidal vasculopathy (PCV) complicated by submacular hemorrhage with breakthrough vitreous hemorrhage. His elevated IOP was thought to be due to spillover hemorrhage into the anterior chamber, causing hemolytic glaucoma with a component of increased posterior pressure leading to angle closure from severe vitreous hemorrhage.
The differential for unilateral submacular hemorrhage with serosanguinous exudative retinal detachment includes valsalva retinopathy, retinal macroaneurysm and anticoagulant-related hemorrhage. In our patient, the presence of multiple polyps and type 1 CNV on OCT, bilateral polypoidal lesions on ICG, the aggressive course with breakthrough vitreous hemorrhage, as well as his Asian heritage, made PCV the most likely diagnosis.
Polypoidal choroidal vasculopathy is a type 1 CNV that is thought to be a variant of age-related macular degeneration. Risk factors include Asian heritage, cigarette smoking and a higher BMI. PCV is VEGF-mediated but does not respond as well to anti-VEGF therapy compared with AMD. PCV is also within the pachychoroid spectrum of disease.
PCV can present more aggressively than AMD with an acute decrease in vision; 30% to 63% of patients develop subretinal hemorrhage, and 5% to 20% develop breakthrough vitreous hemorrhage (VH). Vitreous hemorrhage can follow subretinal hemorrhage due to retinal pigment epithelium (RPE) and outer retinal degeneration. In our patient, the mechanism of VH is unclear. Several studies have shown VH in PCV can occur on presentation or after intervention. Risk factors for VH after intravitreal injection include large subretinal hemorrhage at time of injection and concurrent anticoagulation, both of which our patient had. VH has been reported after photodynamic therapy (PDT) and pneumatic displacement of submacular hemorrhage as well. Patients can also have recurrent serosanguinous retinal or RPE detachments that are difficult to treat.
The diagnosis of PCV relies on both biomicroscopy and imaging. On fundus exam, the type 1 CNV appears as an orange subretinal lesion. Polyps are visible on ICG as focal areas of hypercyanescence within a branching vascular network on late frames. OCT may demonstrate multiple adjacent or M-shaped PEDs, a PED notch, and thumb- or dome-shaped polyps. The double-layer sign represents a type 1 CNV. The choroid is often greater than 300 µm in thickness. OCT angiography can help identify CNV as well.
Polyps in PCV are treated with either anti-VEGF monotherapy or anti-VEGF in combination with PDT. The EVEREST-II and PLANET randomized controlled trials compared intravitreal Lucentis (ranibizumab, Genentech) vs. Lucentis/PDT combination therapy and intravitreal Eylea (aflibercept, Regeneron) vs. Eylea with rescue PDT, respectively. The groups that received anti-VEGF monotherapy had significant gains in visual acuity. The visual acuity gains were greater in the groups that received combination therapy. Most providers will therefore treat with anti-VEGF with or without PDT depending on the patient’s response.
Vitreous hemorrhage is treated surgically with pars plana vitrectomy (PPV). Patients with extrafoveal polyps have better visual acuity outcomes than those with subfoveal or juxtafoveal polyps. Unfortunately, only 60% of patients in one study had improvement of visual acuity by two lines or more after PPV for VH. In our patient, multiple factors including anticoagulation and large subretinal hemorrhage on initial presentation likely contributed to severe breakthrough vitreous hemorrhage.
Clinical course continued
On the first postoperative day, the patient’s vision was no light perception and IOP was 34 mm Hg. On subsequent visits over the next 2 months, he developed persistent eye pain. He required repeat CPC due to uncontrolled elevated IOP despite medical therapy. He remains with no light perception in the left eye and now has no eye pain. He is being observed for the development of PCV in the right eye.
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- For more information:
- Christine L. Shen, MD, and Shilpa Desai, MD, can be reached at New England Eye Center, Tufts University School of Medicine. 800 Washington Street, Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Alison J. Lauter, MD, and Sarah E. Thornton, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.