Polypoidal choroidal vasculopathy 20 years after resolution of tubercular choroiditis

Summary

We report the case of a 72-year-old white woman with blurring of vision in both eyes of 15 days’ duration. She had a history of treatment for bilateral tuberculosis choroiditis 20 years before. She was diagnosed with polypoidal choroidal vasculopathy in the right eye and inflammatory choroidal neovascular membrane in the left eye, based on multimodal imaging, including optical coherence tomography, fundus fluorescein angiography, and indocyanine green angiography. The right eye received 3 intravitreal injections of aflibercept and showed complete resolution. The left eye was treated with a single intravitreal injection of aflibercept.

Introduction

Inflammatory choroidal neovascular membrane (CNVM) is a known complication of choroiditis. Multifocal choroiditis, punctate inner choroiditis, and serpiginous choroiditis present with CNVM in 32%–46%, 17%–40%, and 10%–25% of all cases, respectively.1,2 In serpiginous choroiditis specifically, CNVM is the most common complication. It originates from the edge of the choroidal lesions causing ischemia in the outer retinal and inner choroidal layers. CNVM in these cases is caused by inflammation-induced angiogenesis, mediated by vascular endothelial growth factor (VEGF).3 Additionally, chronic inflammatory damage to the retinal pigment epithelium (RPE) and Bruch’s membrane allows new capillaries to pass from the choroid to the sub-RPE and subretinal space with development of CNVM. Anti-VEGF medications are the first-line treatment, leading to regression of the CNVM.4

Polypoidal choroidal vasculopathy (PCV) development in healed choroiditis is extremely rare.3 We describe a rare diagnosis of PCV in a patient with healed tubercular choroiditis in one eye and inflammatory CNVM with healed tuberculous serpiginous-like choroiditis in the other eye. Both eyes responded well to intravitreal anti-VEGF injection.

Case Report

A 72-year-old woman presented at Rajan Eye Care Hospital, Chennai, with blurring of vision in both eyes of 15 days’ duration. Twenty years earlier she had been treated for bilateral tuberculous choroiditis with oral steroids and anti-tubercular therapy for 6 months.

On examination, best-corrected visual acuity was 6/9 in the right eye and 6/60 in the left eye. Anterior segment examination showed early cataracts. Intraocular pressure in each eye was normal. Fundus examination of the right eye revealed peripapillary chorioretinal atrophy (PPCRA), with multiple chorioretinal atrophic patches (CRA) in the posterior pole along the vessels. There was subretinal hemorrhage in the peripapillary region and two orange-yellow nodules along the superior arcades (Figure 1A). Fundus examination of the left eye showed PPCRA with CRA involving the fovea in a distinct serpiginous pattern (Figure 2A). There was no active inflammation in either eye.

Figure 1.

Right eye. A, Fundus photograph showing peripapillary chorioretinal atrophy with multiple healed choroiditis patches. Hemorrhagic pigment epithelial detachment (PED) with subretinal heme is seen at the fovea (black arrow) with orangish-red polyps seen along the superotemporal arcade (blue arrows). B-C, Fundus fluorescein angiography and indocyanine green angiography images (red arrows indicate extrafoveal polyps). D, Optical coherence tomography (OCT) showing hemorrhagic PED with subretinal fluid. E, OCT showing resolution of fluid post-treatment.

Figure 2.

Left eye. A, Fundus photograph showing healed choroiditis patch contiguous with disc with choroidal neovascular membrane (CNVM) at the fovea. B-D, Fundus fluorescein angiography and indocyanine green angiography showing window defect and hypofluorescence, respectively, with no evidence of active inflammation. D, OCT showing inflammatory CNVM with intraretinal fluid. E, OCT showing resolution of intraretinal fluid after anti–vascular endothelial growth factor injection.

Fundus fluorescein angiography showed window defects and staining corresponding to CRA, blocked fluorescence at the area of subretinal heme, and pooling at the macula in the right eye (Figure 1B), and staining of CRA with minimal leakage at the fovea corresponding to the choroidal neovascular membrane (CNVM) in the left eye (Figure 2B). Indocyanine green angiography (ICG) showed a hot spot corresponding to the orange-yellow lesions in in the right eye (Figure 1C). Optical coherence tomography (OCT) of the right eye showed hemorrhagic pigment epithelial detachment (PED) with subretinal fluid (SRF) and double layer sign (Figure 1D). OCT of the left eye showed shallow, irregular retinal pigment epithelium elevation, subretinal hyperreflective material, and intraretinal fluid (Figure 2D).

The patient was diagnosed with PCV in the right eye and inflammatory CNVM in the left eye. She underwent intravitreal injection (IVI) of aflibercept, an anti–vascular endothelial growth factor therapeutic, in each eye monthly. One-month post-injection, the right eye showed significant flattening of the PED with reduction in SRF; the left eye showed resolution of fluid (Figure 2E). The right eye received a total of 3 aflibercept injections, monthly, after which there was complete flattening of the serous PED with the resolution of SRF (Figure 1E). At 3 months’ follow-up, best-corrected visual acuity was 6/6 in the right eye, and 6/60 in the left eye, with no recurrence.

Discussion

We present a rare case of PCV in a patient with healed tubercular choroiditis. Our patient also had inflammatory CNV in healed serpiginous choroiditis in the left eye, a well-documented complication. PCV is a disease of the inner choroidal vasculature characterized by polypoidal vascular dilatations at the border of branching vascular networks of the choroidal vasculature. It is associated with RPE detachments, either serous or hemorrhagic, and neurosensory detachment of the retina secondary to leakage from the choroidal vascular lesions.5 The link between PCV and choroiditis is not well-established; only one previous case series by Yannuzzi et al exists.6 In their series, a single patient had multifocal choroiditis with secondary CNV. ICG angiography showed polypoidal changes at the margin of the CNV consistent with PCV. The other 2 patients showed pigment changes contiguous and within the PCV lesions. Since there were no signs of active intraocular inflammation in these 2 eyes, the authors were unable to establish whether it was due to previous inflammatory disease or degeneration secondary to serosanguineous detachments of the macula. They suggested that inflammation stimulates embryonic rests in a susceptible individual, leading to the development of vascular proliferations, which is seen in PCV.6 Choroidal osteoma is an example of this type of choroidal entity. Although rare, PCV has been reported in cases of choroidal osteoma.7,8 The mechanism is not clear, but Shields et al9 suggested that disruption of RPE and Bruch’s membrane in the course of decalcification of the tumor can allow the underlying new choroidal vessels to grow into the sub-RPE space. Kim et al7 reported a case of PCV in a patient with choroidal osteoma. They suggested that type 1 CNV can develop in such cases and can progress to PCV depending on the course of the disease. Decalcification involves choroidal atrophy, RPE changes, and loss of photoreceptors. It is possible that a similar mechanism could lead to the development of PCV in cases of healed choroiditis where choroidal and retinal atrophy also develops; however, there is not enough evidence to link PCV to chorioretinal inflammatory diseases, and the occurrence could be coincidental.5

In conclusion, whether PCV occurs concurrent to the chorioretinal inflammatory process or as a separate entity is yet to be established. More detailed analysis of CNV that develops in cases of choroiditis must be performed to look at the possibility of missed PCV in these cases. Aflibercept can aid in the regression of polypoidal lesions with improvement in visual and anatomical outcomes, especially in cases of peripapillary CNV where photodynamic therapy is precluded, as in our case.10 Our case also responded well to aflibercept with good anatomical and visual outcomes.