Multimodal imaging of reactive retinal astrocytic vasoproliferative tumour in a case of systemic tuberculosis

Abstract

A man in his 20s presented with diminished vision in the left eye. He had a history of Pott’s spine and had been diagnosed elsewhere as having left eye tubercular granuloma in the retina. He was started on anti-tubercular therapy and high-dose oral steroids. He presented to us 1 month later. Presently fundus examination revealed a yellow to whitish mass temporal to disc with diffuse hard exudates throughout the retina. Swept-source optical coherence tomography (SSOCT) revealed a hyper-reflective mass involving the retina. SSOCT angiography and fundus fluorescein angiography revealed vascularity within the lesion. We made a diagnosis of a secondary reactive retinal astrocytic vasoproliferative tumour (VPT) and hence tapered the steroids and given intravitreal bevacizumab injection. At 6 weeks follow-up after intravitreal bevacizumab, there was some amount of resolution of hard exudates along with reduction of the vascular pattern of the lesion. The peripapillary location and development of a VPT following resolution of a presumed tubercular granuloma is rare.

Background

A vasoproliferative tumour (VPT) (reactive retinal astrocytic tumour) is a rare tumour characterised by a retinal mass-like lesion commonly in the inferotemporal pre-equatorial retina associated with extensive retinal exudates.^{1 2} It can be primary or secondary—secondary due to retinal inflammation, ischemia, neovascularisation and exudation.³ Here, we report a case of a secondary VPT in a patient with systemic tuberculosis who had been diagnosed elsewhere as having left eye tubercular granuloma. Secondary VPT in association with ocular tuberculosis is rare.^4–6 Most common location of secondary VPT is pre-equatorial.⁶ In our case, the tumour was located peripapillary and temporal to the macula.

Case presentation

A man in his 20s presented with diminution of vision in the left eye for the past 3 months. The patient was diagnosed as having Pott’s spine with right acute otitis media with mastoiditis 3 months back and was started on antitubercular therapy (ATT). The patient was diagnosed elsewhere 1 month back as having left eye tubercular granuloma with active vasculitis with exudative retinal detachment. He was started on oral steroids and was on high dose (60 mg/day) along with the ATT when the patient presented to us. An ultrasound done previously at another centre revealed a hyper-reflective mass at the optic disc with exudative retinal detachment (figure 1A). On presentation to us, the best-corrected visual acuity in the right eye was 20/20, and counting finger close to face with accurate projection of rays in the left eye. Intraocular pressure (IOP) was 28 mm Hg in both eyes. Anterior segment examination and fundus examination of the right eye was normal. Left eye examination revealed a normal anterior segment with few retrolental cells. Fundus examination revealed a yellow to whitish mass temporal to disc involving the fovea with diffuse hard exudates surrounding the optic disc and extending to the retinal periphery in all quadrants, distributed predominantly along the retinal vessels (figure 1B). The previous exudative retinal detachment had resolved when the patient presented the first time in our clinic (figure 1C).

Figure 1.

Left eye of the patient (A) ultrasound image of the posterior segment taken at another centre 1 month earlier before starting oral steroids shows a hyperechoic mass lesion in front of optic nerve head along with exudative retinal detachment and echoes in the vitreous cavity. (B) Ultrawide field imaging of the fundus shows yellow to whitish mass over the disc extending temporally and involving the fovea. diffuse hard exudates are seen in all quadrants. (C) Ultrasound image following 3 months of ATT and 1 month of steroids shows regression in the size of mass and resolution of the exudative retinal detachment and vitreous echoes. (D) Optical coherence tomography image through the whitish mass over the disc shows focal hyper reflective mass involving the retina with back shadowing. (E) OCT-A through the lesion shows internal vascularity in the mass. (F) Arteriovenous phase fundus fluorescein angiography shows vascularity within the mass and multiple dot block fluorescence seen surrounding the mass corresponds to hard exudates. ATT, anti-tubercular therapy; OCT-A, optical coherence tomography angiography.

Investigations

Swept-source optical coherence tomography through the lesion revealed a hyper-reflective mass involving the retina (figure 1D). There was total disorganisation of the retinal architecture in the area of the mass with some overlying areas of vitreoretinal traction. Most of the underlying choroid was not seen due to shadowing, but overall the lesion appeared to be predominantly retinal in origin. SS OCTangiography revealed mature vessels within the lesion at the posterior pole (figure 1E). Fundus fluorescein angiography also highlighted the fine vasculature within the lesion (figure 1F). No significant leakage was seen from these vessels. Scattered focal hypofluorescent areas were seen throughout the fundus corresponding to the overlying hard exudates.

Treatment

As the imaging revealed a reactive retinal mass, the steroids were tapered and patient was asked to take full course of ATT as per physicians advice. As IOP was high anti glaucoma medication was started in both eyes. Since the imaging of the retinal lesion revealed a vascular component, an intravitreal injection of bevacizumab was given in the hope that reduction in the vascularity of the lesion might also lead to a reduction in the size of the lesion.

Outcome and follow-up

At 6 weeks postinjection follow-up visit, the size of the tumour did not show much change. However, there was some amount of resolution of hard exudates and reduction in the internal vascularity of the lesion on OCTA. The visual acuity did not improve which could be due to the location of the lesion. IOP was 22 mm Hg in both eyes on topical antiglaucoma medications.

Discussion

Rodrigues et al published a case of VPT associated with presumed ocular tuberculosis.⁴ Similar to our case, that patient had systemic tuberculosis and the patient was on steroids and antitubercular treatment. The patient described by Rodrigues et al had a vascularised elevated mass located in the lower peripheral retina along with exudates and serous detachment. The authors reported some resolution of the tumour after cryotherapy of the lesion and intravitreal injection of triamcinolone. An unusual case of bilateral optic nerve head reactive retinal astrocytic tumour refractory to multiple therapies including anti-VEGF (vascular endothelial growth factor) injections has also been reported.⁷ We report the multimodal imaging of a case of a peripapillary reactive retinal astrocytic tumour, which could have developed following the resolution of an optic disc tubercular granuloma in a patient with a history of systemic tuberculosis. We refer to the lesion in our case as reactive retinal vasoproliferative astrocytic tumour as the tumour contained both fibrotic/gliotic tissue and had a vascular component evident on multimodal imaging. Reactive VPTs can be primary or secondary—secondary due to retinal inflammation, ischaemia, neovascularisation and exudation. In our case, we presume it to be secondary. Various treatment modalities have been described for reactive vasoproliferative tumours such as laser photocoagulation or transpupillary thermotherapy, cryotherapy, plaque brachytherapy, intravitreal triamcinolone and intravitreal antivascular endothelial growth factor injections.³ In our case, imaging of the lesion had revealed a vascular component. Thus, an intravitreal injection of bevacizumab was given in the hope that regression of vascularisation might lead to a reduction in the size of the lesion and thus some improvement in vision. Rogers et al have reported regression of one out of six cases of VPTs with intravitreal bevacizumab.⁸ In our study at 6 weeks postinjection, we could observe some amount of resolution of hard exudates (figure 2A) even though the size of the tumour did not show much change (figure 2B). There was also some reduction in the internal vascularity of the lesion on OCTA (figure 2C). As the lesion was in the posterior pole, cryotherapy was not considered. It is important to recognise this entity of reactive retinal tumours different from inflammatory granuloma. This would prevent further unnecessary therapy in the form of oral steroids.

Figure 2.

Images of left eye, 6 weeks post bevacizumab intravitreal injection. (A) Ultrawide field imaging of the fundus showing significant resolution of hard exudates, however, no significant change in size of the tumour was observed. (B) Optical coherence tomography image shows the tumour to be almost similar in size. (C) OCTA shows a reduction in internal vascularity of the lesion. OCTA, optical coherence tomography angiography.

Patient’s perspective

• I came to know that secondary changes following resolution of ocular tuberculosis had damaged the retina. Though an attempt was made to treat it with bevacizumab, there was no significant improvement in vision.

Learning points

• Vasoproliferative tumours (reactive retinal astrocytoma) can be peripapillary in location.

• Vasoproliferative tumours can arise secondary to resolution of presumed intraocular tubercular granulomas.

• Anti-VEGF leads to regression of vascularity and also to some extent reduces the exudation from such lesions.

• It is important to recognise this entity of reactive retinal tumours and not an inflammatory granuloma. This would prevent further unnecessary therapy in the form of oral steroids.

Ethics statements

Patient consent for publication

Consent obtained directly from patient(s).