Posterior vortex vein with foveal hypoplasia in oculocutaneous albinism

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 1a and 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 1c and 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)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 The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.