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Multimodal Imaging of Type 1 Aneurysmal Telangiectasia in a Turkish Female With Giant Arteriovenous Aneurysmal Malformation

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Case Reports

Multimodal Imaging of Type 1 Aneurysmal Telangiectasia in a Turkish Female With Giant Arteriovenous Aneurysmal Malformation

Yasin Sakir Goker et al. Ther Adv Ophthalmol.

Abstract

We present a case with giant arteriovenous aneurysmal malformation with different imaging modalities, including fundus photography, fundus fluorescein angiography, spectral domain optical coherence tomography, and optical coherence tomography angiography. A 43-year-old Turkish female presented with gradual blurred vision in her left eye. Her best-corrected visual acuity was 20/200. The fundoscopic examination revealed hard exudates and microaneurysms around the macula. Spectral domain optical coherence tomography showed cystoid macular edema and serous macular detachment. Fundus fluorescein angiography and optical coherence tomography angiography showed bean-like arteriovenous anastomosis between the inferior venous vascular arcade and the superior arterial vascular arcade. An en face angiogram using optical coherence tomography angiography within the deep capillary plexus also showed venous collateral channels across the horizontal raphe. The patient was diagnosed with arteriovenous aneurysmal malformation in association with type 1 aneurysmal telangiectasia. Ophthalmologists should note that giant aneurysmal lesions can occur in type 1 aneurysmal telangiectasia patients.

Keywords: arteriovenous malformation; en face angiogram; optical coherence tomography; optical coherence tomography angiography; type 1 aneurysmal telangiectasia.

Conflict of interest statement

Conflict of interest statement: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Fundus photographs and spectral domain optical coherence tomography (SD-OCT) images of the case: (a) normal examination of the right eye; (b) hard exudates, microaneurysms, and a few intraretinal hemorrhages were seen around the macula in the left eye; and (c) SD-OCT showed cystoid macular edema and serous macular detachment.
Figure 2.
Figure 2.
Fundus fluorescein angiography (FFA) images of the case. (a) Arteriovenous phase of the FFA at 15 s. Upper blue arrow shows the superior temporal artery, and lower blue arrow shows the inferior temporal vein. Laminar flow pattern with a railroad track appearance was seen in the filling venules. (b) Still arteriovenous phase of the FFA at 17 s. Red arrow shows an anastomosis between the inferior venous vascular arcade and the superior arterial vascular arcade. (c) Venous phase of the FFA at 32 s. Red arrow shows giant arteriovenous aneurysmal malformation with a bean-like morphology. Blue stars show telangiectatic capillaries and dilated collateral venous channels across the horizontal raphe between inferior and superior vascular arcades.
Figure 3.
Figure 3.
Optical coherence tomography angiography (OCTA) images of the case: (a) superficial capillary plexus segmentation, (b) deep capillary plexus segmentation, (c) outer retina segmentation of the case, and (d) color overlay images of the lesion. The yellowish part of the image shows the malformation located in the outer retina.
Figure 4.
Figure 4.
En face images of the case via optical coherence tomography angiography: (a) superficial capillary plexus segmentation, (b) deep capillary plexus segmentation, and (c, d) outer retina segmentation of the case. In (b), red arrows show collateral venous vessels across the horizontal raphe between inferior and superior vascular arcades.
Figure 5.
Figure 5.
Optical coherence tomography angiography (OCTA) revealed flow voids within the arteriovenous aneurysmal malformation (red line) in deep capillary plexus and avascular outer retina: (a) manual segmentation of OCTA through the outer retina and (b) en face angiogram of OCTA after the aflibercept therapy.
Figure 6.
Figure 6.
Spectral domain optical coherence tomography (SD-OCT) changes during the follow-up: (a) SD-OCT image at baseline examination; (b–d) SD-OCT images at first-, second-, and third-month examinations, respectively; (e) SD-OCT image at the sixth-month examination after the fifth intravitreal injection of aflibercept; and (f) SD-OCT image at the 12th-month examination.

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