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Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 58-59

Association of Straatsma syndrome with persistent hyaloid vasculature

1 Shri Bhagwan Mahavir Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India
2 Department of Pediatric Ophthalmology and Strabismus, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Submission01-Apr-2020
Date of Acceptance14-Jul-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Aditya Verma
Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18, College Road, Chennai . 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_779_20

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Keywords: Amblyopia, myelinated nerve fibers, persisted hyaloid vasculature, pseudocolor image, unilateral myopia

How to cite this article:
Sreenivasan J, Ramkumar A, Verma A. Association of Straatsma syndrome with persistent hyaloid vasculature. Indian J Ophthalmol Case Rep 2021;1:58-9

How to cite this URL:
Sreenivasan J, Ramkumar A, Verma A. Association of Straatsma syndrome with persistent hyaloid vasculature. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Apr 14];1:58-9. Available from: https://www.ijoreports.in/text.asp?2021/1/1/58/305539

A 7-year-old girl presented with defective vision in the left eye (OS) since three years of age. Her right eye (OD) was emmetropic, while OS had refraction of –12.50DS/–3.50 Dcyl @ 150 degrees with best-corrected visual acuity (BCVA) of counting fingers at 1 meter. Slit-lamp examination with diffuse illumination showed early posterior capsular opacity in the visual axis in OS [[Figure 1]a, yellow arrow] and retro-illumination revealed persistent hyaloid vasculature [[Figure 1]b, blue arrows]. Fundus examination (pseudocolor fundus picture [Optos 200Tx imaging system, Optos PLC, Dunfermline, Scotland, UK]) of left eye [[Figure 2]a, red arrow] showed extensive myelinated nerve fibers over the posterior pole with a small fibrous stalk was found to emanate from optic nerve head (ONH), possible distal remnant of hyaloid artery [[Figure 2]b, white arrows]. Swept Source optical coherence tomography (SSOCT, DRI-OCT1 Atlantis; Topcon, Tokyo, Japan) of the macula showed gross retinal thinning, with posterior staphyloma, and hyperreflectivity of retinal layers due to myelination with loss of retinal architecture [[Figure 2]c, red arrow] in left eye. SSOCT of the ipsilateral ONH showed elevation with a hyperreflective echo suggestive of hyaloid remnant [[Figure 2]d, blue arrowhead] and shadowing caused by the vascular stalk [[Figure 2]e, yellow arrowhead]. The girl was advised patching with correction. At six months follow up, BCVA in left eye improved to 20/60. Informed consent from parents was obtained.
Figure 1: Slit lamp (diffuse) photograph (a) of the left eye, showing early central posterior subcapsular opacification (yellow arrow). Retro-illumination (b, blue arrows) shows the entire network of persistent anterior hyaloid vasculature

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Figure 2: Pseudocolor fundus picture of the left eye showing extensive myelinated nerve fibres over the posterior pole (a, red arrow) with a small fibrous stalk emanating from the disc (b, white arrows). Corresponding Swept Source optical coherence tomography showed gross retinal thinning at macula (c, red arrow), with posterior staphyloma and hyperreflectivity of inner retinal layers with loss of retinal architecture; and optic disc elevation with an overlying hyperreflective echo suggesting hyaloid remnant (d, blue arrowhead) and shadowing caused by the vascular stalk (e, yellow arrowhead)

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  Discussion Top

Straatsma syndrome consists of ipsilateral extensive myelinated retinal nerve fibers, anisometropic myopia, amblyopia, and strabismus.[1],[2],[3],[4],[5] Early diagnosis is important as it makes possible correction of the anisometropia by amblyopia therapy and appropriate strabismus management.[1] Although many ocular associations (including persistent hyaloid vasculature) have been described with this entity,[3],[4] this is the first photographic documentation of the persistent hyaloid vasculature with straatsma syndrome in the literature.

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

Straatsma BR, Heckenlively JR, Foos RY, Shahinian JK. Myelinated retinal nerve fibers associated with ipsilateral myopia, amblyopia, and strabismus. Am J Ophthalmol 1979;88:506-10.  Back to cited text no. 1
Nogueira G, Heringer J, Filho LP, Raupp N, Morizot E. Straatsma syndrome: Two case reports. Rev Bras Oftalmol 2017;76. doi: 10.5935/0034-7280.20170055.  Back to cited text no. 2
Jalil N. Triad of myelinated retinal nerve fibers, axial myopia and amblyopia. J Ophtalmic Vis Res 2010;5:284-5.  Back to cited text no. 3
Ellis GS Jr, Frey T, Gouterman RZ. Myelinated nerve fibers, axial myopia, and refractory amblyopia: An organic disease. J Pediatr Ophthalmol Strabismus 1987;24:111-9.  Back to cited text no. 4
Osaguona VB, Uhumwangho OM. Syndrome of myelinated retinal nerve fibres, myopia, amblyopia and strabismus in a Nigerian. Niger Med J 2014;55:517-8.  Back to cited text no. 5
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