Ziv-aflibercept combined with intravitreal steroid for management of peripheral exudative haemorrhagic chorioretinopathy

Abstract

Peripheral exudative haemorrhagic chorioretinopathy (PEHCR) is considered a variant of polypoidal choroidal vasculopathy. It may have varried presentations and systemic associations. We present a case of PEHCR which dramatically responded to intravitreal steroid and ziv-aflibercept injection. This case not only highlights the promising role of combination therapy with intravitreal steroids and ziv-aflibercept but also the need to look for any associated systemic comorbidity.

Background

Peripheral exudative haemorrhagic chorioretinopathy (PEHCR) is a rare disorder with varied clinical presentations. They typically present as peripheral mass lesion secondary to subretinal or subretinal pigment epithelial haemorrhage and exudation. Though it is considered as a variant of polypoidal choroidal vasculopathy, some believe that it is a peripheral type of age-related macular degeneration.¹ In addition, it was noted to have certain systemic associations like hypertension. PEHCR can mimic choroidal melanoma and multimodal imaging helps in correct diagnosis. It is not a very common clinical entity and so limited data exist regarding its treatment protocols. Various treatment modalities like observation alone, laser and or anti-vascular endothelial growth factor (anti-VEGFs) were reported.²

Case presentation

A 71-year-old woman presented with reduced vision in both her eyes of 2 months duration. Her visual acuity was counting finger close to face (CFCF) in oculus dexter (OD) and hand motion in oculus sinister (OS). Anterior segment examination was normal except for PCIOL in both eyes. Intraocular pressure (IOP) was 18 mm Hg OU. Fundus evaluation showed large peripheral exudative detachment and subretinal haemorrhage in both her eyes (figure 1A,B). The macula OU showed subretinal fluid (SRF) and scarring. In addition, she had epiretinal membrane in OD and pigment epithelium detachment in OS. A clinical diagnosis of OU exudative retinal detachment was made and ocular imaging was done. She was also on treatment for hypertension and coronary artery disease and was systemically investigated. The review of older documents available confirmed the peripheral choroidal vascular abnormalities in angiogram. She had received intravitreal bevacizumab injection earlier, but with not much improvement. She was planned for intravitreal ziv-aflibercept (IVA) along with steroid injection in her right eye. At 1 month, her vision in OD had improved to 20/250 with further reduction of exudative retinal detachment (RD). She had persisting exudative RD in OS and was advised for intra vitreal triamcinolone acetonide (IVTA) in OS. At 6 months of follow-up, she maintained her vision 20/320 OU and was able to independently do all he activities.

Figure 1.

(A,B) Optos widefield fundus photography showed exudative detachment with scarring in OD and exudative detachment in OS involving temporal and nasal retina.

Investigations

A widefield fundus photography showed exudative detachment with scarring in OD and exudative detachment in OS involving temporal and nasal retina. Optical coherence tomography showed shallow intraretinal and SRF with scarring and epiretinal membrane (ERM) in OD and OS revealed multiple pigment epithelial detachment (PEDs) involving the fovea (figure 2A,B). B scan confirmed the exudative detachment OU (figure 3A,B). Fundus fluorescein angiography (FFA) (figure 4A,B) and ICG showed multiple polyps in OS and leakage in late phase in OU (figure 5A,B).

Figure 2.

(A,B) Optical coherece tomography (OCT) OD showed shallow SRF with scarring and epiretinal membrane and OS revealed multiple PEDs. PEDs, pigment epithelial detachment; SRF, subretinal fluid.

Figure 3.

(A,B) B scan confirmed the exudative detachment with subretinal precipitates OU.

Figure 4.

(A,B) FFA showed multiple polyps in OS and leakage in late phase in OU.

Figure 5.

(A,B) ICG showed multiple polyps in OS and leakage in late phase in OU.

The haematological tests showed hypochromic microcytic anaemia with haemoglobin (Hb) of 60 g/L. At final follow-up, the exudation reduced and was documented on fundus photo (figure 6A,B) and optical coherence tomography (figure 7A,B).

Figure 6.

(A,B) At final follow-up, the exudation resolved on fundus photo.

Figure 7.

(A,B) At final follow-up, the exudation resolving on OCT.

Differential diagnosis

Choroidal detachment was considered based on B scan but the typical M spike was absent as seen in choroidal detachment (CDs). Mass lesion of choroid was also kept as differential diagnosis, but the investigations ruled out the same.

Treatment

She was planned for IVTA 1 mg/0.05 mL along with IVA 2 mg/0.05 mL in OD. After 2 weeks, the height of exudative RD reduced and she was advised for physician review for anaemia. Subsequently, she received 2 units of whole blood and her post-transfusion Hb was 10.1 g/dL. At 1 month of follow-up, OD showed improvement but she had persisting exudative RD in OS and was advised for IVTA in OS.

Best corrected visual acuity (BCVA) improved to 20/200 in OD and CFCF in OS at 2-month follow-up. She was sent to low vision clinic and was advised low vision aids. On follow-up at 5 months, her BCVA was 20/320 in OD and CF at 1 m in OS. The exudation had slightly increased in OD and she was advised repeat IVTA 1 mg/0.05 mL and ziv-aflibercept 2 mg/0.05 mL in OD. At 6 months after initial injection, her BCVA was 20/320 in OD and 20/320 in OS. She required injection of ziv-aflibercept 2 mg/0.05 mL in OS at this follow-up as the intraretinal fluid had reappeared. The IOP was 16 mm Hg in both her eyes.

Outcome and follow-up

She was followed up monthly for next 6 months and her visual acuity and clinical features remained stable.

Discussion

PEHCR is considered a disease of peripheral retina noticed commonly in elderly patients in eighties.² The etiopathogenesis is not very clear but it is postulated that multiple PEDs due to choroidal vascular abnormalities coalesce and lead to exudative detachment.³ A few of the case series reported the polypoidal lesions in the periphery that were detected on indocyanine green angiography.⁴ Ultra-widefield angiography helps in the diagnosis and management of PEHCR. Even though, most of the cases with only peripheral involvement were noted to resolve spontaneously with scarring, treatment was recommended when the lesions were extending to the macular area or when present with vitreous haemorrhage. There are reports of cases being treated with anti-VEGFs, systemic steroids and or laser.^{5 6}

Anti-VEGFs have been used successfully to reduce the exudation but this is the first report of use of combination therapy with ziv-aflibercept for successful outcome. Surgical options are reserved for specific situations like vitreous haemorrhage or massive subretinal haemorrhage. Systemic comorbidities like hypertension and use of blood thinners should be closely monitored.

Patient’s perspective.

I was diagnosed with a serious retinal condition in both the eyes and had a fear of going blind. My vision was so bad that I became dependent on my children for day-to-day activities. I was told elsewhere that I need repeated injections in my eyes due to water accumulation but each injection was costly and vision did not improve. I visited this hospital and I was told about my eye condition in detail after a series of tests and the remedial measures. I was reviewed by physician and my systemic aliment was also cured. My vision has improved now and I do all my activities independently with good effort tolerance.

Learning points.

• Silent disease—caution in patients with hypertension (HTN) and anticoagulant use.

• Ziv-aflibercept in combination with steroids is promising therapeutic option.

• Involve your physician—control of hypertension, anticoagulants at minimum dosage required for systemic disease.