• Users Online: 92
  • Print this page
  • Email this page

 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 680-682

Tubercular retinal vasculitis in a patient with benign familial fleck retina mimicking frosted branch angiitis

1 Department of Vitreo-Retina, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India
2 Department of Uvea, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India

Date of Submission29-Dec-2020
Date of Acceptance10-Apr-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Chitaranjan Mishra
Department of Vitreo-Retinal services, Aravind Eye Hospital, Anna Nagar, Madurai - 625 020, Tamilnadu
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3742_20

Rights and Permissions

Patients with tubercular uveitis can present in a myriad of clinical findings. Tubercular retinal vasculitis (TRV) mimicking frosted branch angiitis (FBA) is sparsely reported in literature. However, TRV in the background of benign familial fleck retina simulating FBA is never reported. We report a patient of TRV from a tubercular endemic region, supported by ancillary investigations like fundus fluorescein angiography and fundus autofluorescence. The patient was diagnosed as a case of “possible intraocular tuberculosis.” She was managed with systemic and topical steroids along with antitubercular therapy and had a favorable outcome.

Keywords: Antitubercular therapy, intraocular tuberculosis, tubercular uveitis, vasculitis, wide-angle fundus autofluorescence

How to cite this article:
Mishra C, Upadhyay A, Thundikandy R, Rajesh V, Baliga G, Rathinam S. Tubercular retinal vasculitis in a patient with benign familial fleck retina mimicking frosted branch angiitis. Indian J Ophthalmol Case Rep 2021;1:680-2

How to cite this URL:
Mishra C, Upadhyay A, Thundikandy R, Rajesh V, Baliga G, Rathinam S. Tubercular retinal vasculitis in a patient with benign familial fleck retina mimicking frosted branch angiitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:680-2. Available from: https://www.ijoreports.in/text.asp?2021/1/4/680/327634

Intraocular tuberculosis (IOTB) is a form of extrapulmonary tuberculosis presenting most commonly as tubercular uveitis. The clinical features of tubercular posterior uveitis have been described to be choroidal granuloma, retinal vasculitis, optic disc edema, and macular edema.[1],[2]

Frosted branch angiitis (FBA) is a form of vasculitis with preferential involvement of veins over arteries. Usually, there is a lack of associated infectious or autoimmune disease.[3] Tubercular retinal vasculitis (TRV) presenting as FBA is rare but reported in the literature.[4],[5],[6]

Benign familial fleck retina (BFFR) is an autosomal recessive disorder characterized by hypopigmented flecks present all over the retina sparing the macula. These lesions are localized to the retinal pigment epithelial layer.[7],[8]

We herein report this case to highlight the unusual clinical presentation of TRV in a patient of BFFR resembling FBA. Also, we describe the management of macular edema in this report.

  Case Report Top

A 23-year-old woman presented with a bilateral blurring of vision, greater in the right eye (RE) for 3 days. Her best-corrected visual acuity (BCVA) was 20/80 and N24 in the RE and 20/32 and N9 in the LE. Ishihara test for color vision was normal in both the eyes. Intraocular pressure was 10 mm Hg in RE and 12 mm Hg in LE.

Anterior segment was normal in BE. On posterior segment evaluation, mild vitritis was present in BE and snowballs were present in the RE. Fleck-like pisciform lesions were present in the background retina in BE. Retinal hemorrhages and vasculitis were evident in BE [Figure 1]a, [Figure 1]b. Fundus fluorescein angiography (FFA) revealed vessel leakage and peripheral capillary nonperfusion areas in BE [Figure 1]c, [Figure 1]d. Wide-angle fundus autofluorescence (FAF) was suggestive of typical pisciform hyperfluorescence lesions throughout the retina sparing the macula in BE [Figure 1]e, [Figure 1]f. On optical coherence tomography (OCT) imaging, the RE showed cystoid macular edema (CME) with central macular thickness (CMT) of 577 microns [Figure 2]a. There was no macular edema on the OCT of the LE.
Figure 1: a, b: Wide-angle fundus photo showing fleck-like pisciform lesions present in the retina in both the eyes (BE). Sheathing of vessels and infiltrates along the retinal veins associated with retinal hemorrhages were present in BE. The RE shows evidence of inferotemporal branched retinal vein occlusion (BRVO). c, d: Wide-angle fundus fluorescein angiography showing vessel leak in all the quadrants in BE. Blocked fluorescence due to hemorrhages was noted in the inferotemporal quadrant in the RE. e, f: Wide-angle fundus autofluorescence showing hyperfluorescent fleck lesions throughout the retina sparing the macula in BE. Imaging device: a, b, e, f – Optos Daytona Plus (Optos plc, Dunfermline, Scotland, UK). c, d – ClarusTM 500 (Carl Zeiss Meditech Inc., Dublin, USA)

Click here to view
Figure 2: OCT of the right macula at a: presentation showing macular edema and subretinal fluid. b: 1-month follow-up image, showing resolving macular edema with minimal subretinal fluid. c: 6-month follow-up image showing almost resolved macular edema with minimal intraretinal fluid. Imaging device: Spectralis HRA (Heidelberg Engineering, Heidelberg, Germany)

Click here to view

Laboratory evaluation revealed an ESR of 26 mm in the first hour. Tuberculin skin test demonstrated an induration of size 26 × 26 mm. Chest X-ray was normal. Laboratory tests for HIV and VDRL were nonreactive. Serum ACE levels were normal. CECT chest and abdomen ruled out any active or healed lesions suggestive of TB or sarcoidosis.

The patient was treated with topical prednisolone acetate (1%) eye drops in BE and posterior subtenon (PST) injection of triamcinolone acetonide (TA) 40 mg/1 mL in the RE.

The patient was referred to a pulmonologist and was started on antitubercular treatment (ATT) with isoniazid 300 mg/day, rifampicin 600 mg/day, pyrazinamide 1600 mg/day, and ethambutol 1100 mg/day. She also received oral prednisolone at 1 mg/kg body weight. ATT was given for 6 months. Oral prednisolone was tapered over a period of 2 months and then maintained at a low dose, i.e., 10 mg/day for 1 month.

At 1 month follow-up, BCVA was 20/32, N9 in RE and 20/20, N6 in LE. Fundus examination showed resolution of vitritis, retinal exudation, and retinal hemorrhages. OCT of RE showed resolving CME with CMT of 269 microns and minimal subretinal fluid [Figure 2]b.

At 6-month follow-up visit, BCVA was 20/32, N9 in RE and 20/20, N6 in LE. There were snowball remnants inferiorly in the RE. Mild exudation and minimal hemorrhages were present in the inferior retina in both the eyes with the RE showing collaterals and sclerosed vessels along the inferotemporal arcade [Figure 3]a, [Figure 3]b. The benign fleck retinal lesions were evident in the FAF [Figure 3]c, [Figure 3]d. OCT of RE showed resolved CME with CMT of 250 μ and minimal intraretinal fluid [Figure 3]c. There was hyperreflectivity at the vitreoretinal interface on the OCT, which was suggestive of the epiretinal membrane. The presence of subfoveal hyperreflectivity at the IS–OS layer was suggestive of inflammatory cells. These findings explain the 20/32, N9 vision in the RE at final follow-up.
Figure 3: a, b: Wide-angle fundus photo showing fleck-like pisciform lesions present in the retina in both the eyes. There is marked resolution of sheathing and infiltrates along the vessels. Also, note the snowball exudates inferiorly in the RE. c, d: Wide-angle fundus auto-fluorescence showing hyperfluorescent fleck lesions throughout the retina sparing the macula in both the eyes. Imaging device: Optos Daytona Plus (Optos plc, Dunfermline, Scotland, UK)

Click here to view

  Discussion Top

Our patient presented with retinal vasculitis of tubercular etiology in BE. Additionally, there was BRVO and CME in RE. The presence of fleck lesions in the retina made the clinical picture resemble FBA. Though TRV resembling FBA has been described previously, our case was different from the prior reports due to the association of BFFR.[4],[5],[6]

Mantoux tests larger than 15 mm are unlikely to be due to environmental mycobacteria exposure or previous BCG vaccination.[9] Our patient was categorized as “possible IOTB” as per the proposed intraocular tuberculosis classification by A. Gupta et al., as there was the presence of clinical evidence, nonconfirmatory chest X-ray findings with immunological evidence of tuberculosis infection.[3]

TRV traditionally involves the peripheral retinal vessels. Agarwal M et al. mentioned about the posterior pole vascular involvement in their case simulating FBA.[6] In our patient, retinal vasculitis was evident both at the periphery and at the posterior pole in BE. The patient described by Zhao et al. presented with tubercular meningitis.[4] The patient described by Agarwal M et al. presented with abdominal tuberculosis.[5] However, our patient did not have any signs suggestive of meningitis or abdominal tuberculosis.

Though vasculitis in the background of BFFR resembled FBA, the typical hyperfluorescent lesions on wide-angle FAF helped in the diagnosis of BFFR.[5]

In the absence of neovascularization of the retina, laser photocoagulation is not needed, as in our case. Since the macular edema was unilateral, local treatment with PST injection of TA was preferred. The outcomes of the management of vasculitis and macular edema were favorable in our patient.

  Conclusion Top

This case highlights the unusual clinical presentation of TRV and BRVO in a patient of BFFR who presented with a decrease in visual acuity due to CME. There was an improvement in the clinical picture after treatment with ATT, systemic, and local steroids. A high index of clinical suspicion, ancillary investigations like FFA and FAF helped in the diagnosis of vasculitis and background retinal lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Testi I, Agrawal R, Mahajan S, Agarwal A, Gunasekeran DV, Raje D, et al. Tubercular uveitis: Nuggets from Collaborative Ocular Tuberculosis Study (COTS)-1. Ocul Immunol Inflamm 2019:1-9. doi: 10.1080/09273948.2019.1646774.  Back to cited text no. 1
Gupta A, Sharma A, Bansal R, Sharma K. Classification of intraocular tuberculosis. Ocul Immunol Inflamm 2015;23:7-13.  Back to cited text no. 2
Geier SA, Nasemann J, Klauss V, Kronawitter U, Goebel FD. Frosted branch angiitis in a patient with the acquired immunodeficiency syndrome. Am J Ophthalmol 1992;113:203-5.  Back to cited text no. 3
Zhao H, Guan J, Zhou L, Zhang Q, Si Y. Frosted branch angiitis in a woman with Mycobacterium tuberculosis infection. Ocul Immunol Inflamm 2012;20:227-9.  Back to cited text no. 4
Agarwal M, Biswas J. Unilateral frosted branch angiitis in a patient with abdominal tuberculosis. Retin Cases Brief Rep 2008;2:39-40.  Back to cited text no. 5
Agarwal M, Shrivastav A, Waris A. Tubercular retinal vasculitis mimicking frosted branch angiitis: A case report. J Ophthalmic Inflamm Infect 2018;8:3.  Back to cited text no. 6
Neriyanuri S, Rao C, Raman R. Structural and functional characterization of benign fleck retina using multimodal imaging. Retin Cases Brief Rep 2017;11:186-90.  Back to cited text no. 7
Gupta S, Jain S, Kumar V. Benign familial fleck retina. JAMA Ophthalmol 2019;137:e185539.  Back to cited text no. 8
Nayak S, Acharjya B. Mantoux test and its interpretation. Indian Dermatol Online J 2012;3:2-6.  Back to cited text no. 9
[PUBMED]  [Full text]  


  [Figure 1], [Figure 2], [Figure 3]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded18    
    Comments [Add]    

Recommend this journal