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Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 244

Posterior vortex vein with foveal hypoplasia in oculocutaneous albinism

Department of Vitreo Retina, Netralaya Superspeciality Eye Hospital, Ahmedabad, Gujarat, India

Date of Web Publication20-Jan-2023

Correspondence Address:
Kushal Delhiwala
Department of Vitreo Retina, Netralaya Superspeciality Eye Hospital, KD House, 1st Floor, Above Union Bank of India, Parimal Cross Roads, Ellisbridge, Ahmedabad - 380 006, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1528_22

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How to cite this article:
Delhiwala K, Shah A, Khamar B, Patel R, Rana P. Posterior vortex vein with foveal hypoplasia in oculocutaneous albinism. Indian J Ophthalmol Case Rep 2023;3:244

How to cite this URL:
Delhiwala K, Shah A, Khamar B, Patel R, Rana P. Posterior vortex vein with foveal hypoplasia in oculocutaneous albinism. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jan 26];3:244. Available from: https://www.ijoreports.in/text.asp?2023/3/1/244/368147

Fundus evaluation in the left eye (OS) of a 9-year-old high-myopic male having oculocutaneous-albinism (OCA) and best-corrected visual-acuity 6/24, N18 revealed tilted optic disc, peripapillary atrophy, absent foveal-reflex, and dilated submacular vasculature (SMV) having radial branching, with attached retina and absent posterior staphyloma [Figure 1]a and [Figure 1]b. Macular swept-source optical coherence tomography showed grade-3 foveal hypoplasia (FH),[1] thin choroid, except localized dilated choroidal vessels corresponding to SMV [Figure 1]c and [Figure 1]d. The patient was diagnosed with OS posterior vortex vein (PVV).
Figure 1: (a) Pseudocolor fundus image of left eye (OS) posterior pole showing tilted optic disc, peripapillary atrophy, absent foveal reflex, and posterior vortex vein (PVV, black arrow). (b) Green channel image showing better visibility of PVV (black arrowhead) due to hypopigmented retinal pigment epithelium (RPE) secondary to oculocutaneous albinism. (c) Radial swept-source optical coherence tomography (SS-OCT) scans of OS macula showing an absence of the foveal pit, persistent inner retinal layers (white arrow) suggestive of foveal hypoplasia, and thin choroid. (d) Radial SS-OCT scan through PVV showing localized dilated choroidal vessels (yellow arrows)

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PVVs can be congenital or acquired.[2],[3] Acquired PVV may be seen in one-fourth of high-myopic eyes, related to venous flow disturbance from the posterior choroid to equatorial vortex veins following axial elongation.[4] The presence of PVV with FH has not been reported in OCA and may suggest developmental or genetic posterior pole abnormality.[5]

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

Kondo H. Foveal hypoplasia and optical coherence tomographic imaging. Taiwan J Ophthalmol 2018;8:181-8.  Back to cited text no. 1
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Higham A, Hildebrand GD, Graham-Evans KA, Gilbert RD, Horton R, Hunt D, et al. Ectopic vortex veins and varices in Donnai Barrow syndrome. Ophthalmic Genet 2022;43:248-52.  Back to cited text no. 2
Moriyama M, Cao K, Ogata S, Ohno-Matsui K. Detection of posterior vortex veins in eyes with pathologic myopia by ultra-widefield indocyanine green angiography. Br J Ophthalmol 2017;101:1179-84.  Back to cited text no. 3
Ohno-Matsui K, Morishima N, Ito M, Yamashita S, Tokoro T. Posterior routes of choroidal blood outflow in high myopia. Retina 1996;16:419-25.  Back to cited text no. 4
Karabas L, Esen F, Celiker H, Elcioglu N, Cerman E, Eraslan M, et al. Decreased subfoveal choroidal thickness and failure of emmetropisation in patients with oculocutaneous albinism. Br J Ophthalmol 2014;98:1087-90.  Back to cited text no. 5


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