AOSIS OpenJournalshttp://www.openjournals.netinfo@openjournals.nethttp://www.sajr.org.za1027-202X2078-6778A. Fourie Bezuidenhout11Department of Radiodiagnosis, Stellenbosch University, South Africa
Fourie Bezuidenhoutfouriebez@yahoo.comPO Box 19179, Tygerberg 7505, South Africa59718110.4102/sajr.v18i1.59707 Jan. 201416 Feb. 2014 20 June 2014Bezuidenhout AF. Persistent hyperplastic primary vitreous – The martini glass sign. S Afr J Rad. 2014;18(1); Art. #597, 1 page. http://dx.doi.org/10.4102/sajr.v18i1.597© 2014. The Authors.
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Attribution License.ContentPersistent 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
AcknowledgementsCompeting interestsThe author declares that he has no financial or personal relationship(s) that may have inappropriately influenced him in writing this article.1. Smirniotopoulos JG, Bargallo N, Mafee MF. Differential diagnosis of leukokoria: Radiologic-pathologic correlation. Radiographics. 1994;14(5):1059–1079. PMID: 7991814. http://dx.doi.org/10.1148/radiographics.14.5.7991814
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5. Kaste SC, Jenkins JJ, Meyer D, Fontanesi J, Pratt CB. Persistent hyperplastic primary vitreous of the eye: Imaging findings with pathologic correlation. Am J Roentgenol. 1994;162(2):437–440. PMID: 8310942. http://dx.doi.org/10.2214/ajr.162.2.8310942