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Year : 2019  |  Volume : 26  |  Issue : 1  |  Page : 49-51  

Combined parafoveal telangiectasia, multifocal central serous chorioretinopathy, and choroidal hemangioma

1 Department of Vitreo-Retina, Aravind Eye Care System, Puducherry, India
2 Department of Ophthalmology, All India Institute of Medical Sciences, Mangalagiri, Guntur, Andhra Pradesh, India

Date of Web Publication24-Apr-2019

Correspondence Address:
Dr. C K Nagesha
Department of Vitreo-Retina, B. W. Lions Superspeciality Eye Hospital, Bangalore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/meajo.MEAJO_92_18

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The exact pathogenesis of parafoveal telangiectasia (PFT) is unknown, but various associations have been reported in literature such as diabetes and hypertension. Coexisting pathologies like diabetic retinopathy with PFT are not uncommon. We encountered a unique combination of PFT with multifocal central serous chorioretinopathy and choroidal hemangioma in a patient with diabetes mellitus. Multimodal imaging confirmed the findings and focal treatment to central serous chorioretinopathy leaks led to resolution of the subretinal fluid. This unique combination has been rarely reported so far. The long-term follow-up of such cases can help us to understand if there exists common pathogenesis of these varied diseases.

Keywords: Central serous chorioretinopathy, choroidal hemangioma, pachychoroid, parafoveal telangiectasia

How to cite this article:
Nagesha C K, Ganne P. Combined parafoveal telangiectasia, multifocal central serous chorioretinopathy, and choroidal hemangioma. Middle East Afr J Ophthalmol 2019;26:49-51

How to cite this URL:
Nagesha C K, Ganne P. Combined parafoveal telangiectasia, multifocal central serous chorioretinopathy, and choroidal hemangioma. Middle East Afr J Ophthalmol [serial online] 2019 [cited 2020 May 27];26:49-51. Available from: http://www.meajo.org/text.asp?2019/26/1/49/256973

   Introduction Top

Parafoveal telangiectasia (PFT) is a macular neurodegenerative disease manifesting with vascular changes like subtle leakage from perifoveal telangiectatic capillaries.[1] It is frequently associated with diabetes, hypertension, and cardiovascular diseases. Co-presentation of PFT with soft drusen, central serous chorioretinopathy (CSR), foveal detachment, and Stargardt's disease has been reported in the literature.[2],[3] We present a case of previously unreported combination of choroidal hemangioma, bilateral PFT, and multifocal CSR. The presence of pachychoroid features in our case hints at the possible association among these three entities.

   Case Report Top

A 66-year-old male presented with a history of diminished vision in the left eye (LE) for 20 days. He had well-controlled hypertension for 10 years and had a history of undergoing photodynamic therapy (PDT) for circumscribed choroidal hemangioma (CCH) in the right eye (RE). On examination, the best-corrected visual acuity in the RE was 6/6, N6 and LE was 6/9, N8. Intraocular pressures were within the normal limits in both eyes.

Fundus examination of the RE showed a pink circumscribed choroidal mass lesion inferotemporal to disc without clinically evident subretinal fluid (SRF). The macula showed multiple pigmentary clumps in the parafoveal area along with gray reflex suggestive of PFT [Figure 1]a. The LE [Figure 1]b showed similar pigmentary clumps at the macula and large SRF pockets (arrows) hyperautoflourescence was seen in blue fundus autofluorescence imaging [Figure 1]c and [Figure 1]d.
Figure 1: (a) Color fundus photo of the right eye showing pigmentary clumps at macula with orange-colored circumscribed choroidal lesions s/o hemangioma (star). (b) The left eye showed similar pigmentary clumps at macula and large subretinal fluid pockets (arrows) which shows hyperautoflourescence in blue fundus autofluorescence (c and d)

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Enhanced-depth imaging optical coherence tomography (EDI-OCT) of the RE showed dome-shaped choroidal elevation corresponding to mass lesion without overlying SRF [Figure 2]a and [Figure 2]c. Both eyes showed loss of layered architecture of the central macula and serous detachment in the LE [Figure 2]b and [Figure 2]d. LE subfoveal choroidal thickness was approximately 350 μ. The largest diameter of the pachyvessel nasal to fovea measures around 248 μ and choroidal thickness at that site was 352 μ [Figure 2]e and [Figure 2]f. Fundus fluorescein angiography (FFA) revealed granular hyperfluorescence over hemangioma in the RE and multifocal ink blot leaks in the LE, and late phases showed pooling of dye into the subretinal spaces [Figure 3]a,[Figure 3]b,[Figure 3]c,[Figure 3]d.
Figure 2: Enhanced-depth imaging optical coherence tomography of the right eye shows foveal intraretinal hyperreflectivity and choroidal elevation nasally. (a and c). The left eye showed similar intraretinal hyperreflectivity and serous detachment (b and d). The choroid appears thick with evident of pachyvessels, especially in the left eye. (e and f) Enhanced-depth imaging optical coherence tomography of the left eye showing dimensions of pachyvessel and choridal thickness nasal to the fovea

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Figure 3: Fundus fluorescein angiography early and late pictures showing granular hyperfluorescence in the right eye (a and c) and multifocal ink-blot leaks (b and d) in the left eye. (e and f) Postlaser optical coherence tomography of the left eye showing complete resolution of the subretinal fluid

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A diagnosis of RE inactive CCH, bilateral nonproliferative-type PFT, and LE multifocal acute CSR was made, and CSR leaks were treated with focal green laser. Six-week follow-up showed complete resolution of SRF [Figure 3]e and [Figure 3]f and the vision in the LE improved to 6/6. The patient was symptom-free for the next 6 months.

   Discussion Top

This unusual case shows copresentation of CCH, multifocal CSR, and bilateral PFT confirmed on multimodal imaging. The presence of CSR along with PFT has been observed in two-isolated case reports,[2],[3] and both reports have shown extramacular ink-blot leaks. They assumed it to be a coincidental finding along with PFT. Recently, Matet et al.[4] observed a very interesting finding in cases of PFT having CSR with multimodal imaging. Of nine eyes, eight eyes had subfoveal serous detachment and one eye had extramacular serous detachment. All these cases had pachychoroid features on EDI-OCT, but none had classical leaks on FFA except for some speckled hyperfluorescence in one. The authors assumed that pachychoroid could be the cause for the subretinal fluid in these cases and that the condition was probably exacerbated by underlying diabetes.

The RE of our patient had CCH. Previously, this eye had a serous detachment which resolved after one session of PDT. Four years later, the LE developed serous detachment and ultrasound showed no focal or diffuse choroidal mass lesions [Figure 4]a and [Figure 4]b. Dave et al.[5] reported a case of Sturge– Weber syndrome More Details presenting with diffuse choroidal hemangioma masquerading as CSR. FFA, in that case, did not show ink-blot leaks but showed speckled hyperfluorescence. The EDI-OCT of both eyes showed the pachychoroid features with thickened choriocapillary zone and pachy vessels. Apart from CSR, that case hinted at probable association of pachychoroid with choroidal hemangioma and PFT. A few authors have noted variable choroidal thickness in patients with PFT, but its true association is yet to be studied by large scale studies.[6],[7]
Figure 4: Ultrasound B scan of the right eye showed choroidal mass lesion with homogenous internal reflectivity (a) and the left eye was essentially normal (b)

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Focal laser therapy is the standard treatment for the leaks away from the fovea. In our case, the SRF completely resolved over 6 weeks and remained stable over the next 6 months. This case highlights the utility of multimodal imaging in diagnosing and managing such unusual cases and also additional findings, such as pachychoroid, that need more understanding. Long-term follow-up is needed to understand the course of the disease and its treatment outcomes.

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. Ophthalmology 1993;100:1536-46.  Back to cited text no. 1
Atmani K, Querques G, Zourdani A, Benhamou N, Souied EH. Unusual presentations of type 2 idiopathic macular telangiectasia. Ophthalmologica 2013;230:126-30.  Back to cited text no. 2
Thomas NR, Roy R, Saurabh K, Das K. A rare case of idiopathic parafoveal telangiectasia associated with central serous chorioretinopathy. Indian J Ophthalmol 2017;65:516-7.  Back to cited text no. 3
[PUBMED]  [Full text]  
Matet A, Yzer S, Chew EY, Daruich A, Behar-Cohen F, Spaide RF. Concurrent idiopathic macular telangiectasia type 2 and central serous chorioretinopathy. Retina 2018;38 Suppl 1:S67-78.  Back to cited text no. 4
Dave T, Dave VP, Shah G, Pappuru RR. Diffuse choroidal hemangioma masquerading as central serous chorioretinopathy treated with oral propranolol. Retin Cases Brief Rep 2016;10:11-4.  Back to cited text no. 5
Chhablani J, Kozak I, Jonnadula GB, Venkata A, Narayanan R, Pappuru RR, et al. Choroidal thickness in macular telangiectasia type 2. Retina 2014;34:1819-23.  Back to cited text no. 6
Nunes RP, Goldhardt R, de Amorim Garcia Filho CA, Thorell MR, Abbey AM, Kuriyan AE, et al. Spectral-domain optical coherence tomography measurements of choroidal thickness and outer retinal disruption in macular telangiectasia type 2. Ophthalmic Surg Lasers Imaging Retina 2015;46:162-70.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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