Indian Journal of Ophthalmology - Case Reports

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

Association of Straatsma syndrome with persistent hyaloid vasculature

Janani Sreenivasan1, Akila Ramkumar2, Aditya Verma1,  
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

Correspondence Address:
Dr. Aditya Verma
Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, 18, College Road, Chennai . 600 006, Tamil Nadu


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;1:58-59

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 10 ];1:58-59
Available from:

Full Text

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}{Figure 2}


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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1Straatsma 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.
2Nogueira 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.
3Jalil N. Triad of myelinated retinal nerve fibers, axial myopia and amblyopia. J Ophtalmic Vis Res 2010;5:284-5.
4Ellis 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.
5Osaguona VB, Uhumwangho OM. Syndrome of myelinated retinal nerve fibres, myopia, amblyopia and strabismus in a Nigerian. Niger Med J 2014;55:517-8.