SIGNS

Persistent hyperplastic primary vitreous - The martini glass sign

A. Fourie Bezuidenhout

Department of Radiodiagnosis, Stellenbosch University, South Africa

Correspondence

Persistent hyperplastic primary vitreous (PHPV) is a congenital lesion due to incomplete regression of the embryonic ocular blood supply (hyaloid vasculature).¹ It represents 28% of childhood presentations of leukocoria and is almost always accompanied by poor vision, micropthalmia and often retinal detachment.¹-²-³ The absence of ocular calcifications helps distinguish PHPV from the more common retinoblastoma.⁴ The appearance of PHPV has been likened to that of a martini glass. The martini glass is represented by triangular retrolental fibrovascular tissue and a central tissue stalk of hyaloid remnant extending to the optic disc in Cloquet's canal (see Figure 1a).⁵ The retrolental fibrovascular tissue and stalk-like hyaloid remnant are hypointense to isointense on T1- and T2-weighted images and show enhancement post contrast administration. The globe may be hyperintense on T1-weighted images; this may represent either subhyaloid or subretinal fluid with blood degradation products (methaemoglobin) or high protein content (see Figure 1b).¹

Acknowledgements

Competing interests

The author declares that he has no financial or personal relationship(s) that may have inappropriately influenced him in writing this article.

References

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Correspondence:
Fourie Bezuidenhout
PO Box 19179
Tygerberg 7505
South Africa
Email: fouriebez@yahoo.com

Received: 07 Jan. 2014
Accepted: 16 Feb. 2014
Published: 20 June 2014