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   Table of Contents      
Year : 2018  |  Volume : 66  |  Issue : 3  |  Page : 359

Possible retinoblastoma?

Wills Eye Hospital, Philadelphia, PA 19107, USA

Date of Web Publication26-Feb-2018

Correspondence Address:
Carol L Shields
Ocular Oncology Services, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1310_17

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How to cite this article:
Shields CL, Elimimian EB. Possible retinoblastoma?. Indian J Ophthalmol 2018;66:359

How to cite this URL:
Shields CL, Elimimian EB. Possible retinoblastoma?. Indian J Ophthalmol [serial online] 2018 [cited 2018 Dec 18];66:359. Available from:

  Case Report Top

A 23-month-old girl with leukocoria in the left eye (OS) at birth was referred for suspicious retinoblastoma. Birth, medical, and family history were unremarkable. Parents denied trauma. On examination, visual acuity was fix and follow in the right eye (OD) and no fixation OS. Intraocular pressures were 15 mmHg OD and 6 mmHg OS. The OD was normal. Anterior segment examination OS revealed persistent tunica vasculosa lentis (TVL) with anterior and posterior synechia and cataract. Posterior segment examination demonstrated dense vitreous membranes and total tractional retinal detachment. Ultrasonography (USG) confirmed total retinal detachment, and there was no evidence of intraocular calcification.

  What Is Your Next Step? Top

  1. Phacoemulsification for cataract removal
  2. Fluorescein angiography (FA) for further investigation
  3. Anterior vitrectomy for removal of vitreous fibrosis
  4. Intravitreal antivascular endothelial growth factor injection.

  Findings Top

Anterior segment FA was performed demonstrating persistent TVL (a). Fundus photograph through hazy media secondary to cataract showed dense retrolental vitreous membranes with total retinal detachment (b). USG (c) and magnetic resonance imaging (d) revealed no evidence of solid intraocular mass or calcification. In addition, the retinal detachment appeared tractional from vitreous fibrosis (b) with centripetal traction from retrolental fibrovascular membranes causing the peripheral retina to be drawn inward in a concave configuration (c and d).

Diagnosis: Combined anterior and posterior persistent fetal vasculature (PFV) OS.

Correct Answer: b.

  Discussion Top

The embryologic hyaloid artery and TVL nourish the primary vitreous. Incomplete regression of this tissue leads to various manifestations of PFV.[1],[2] PFV is typically unilateral, with leukocoria, and is the second leading simulator of retinoblastoma.[3] Any child with leukocoria requires exclusion of retinoblastoma. In our case, USG and MRI revealed the absence of mass or calcification. FA demonstrated normal vasculature in the fellow eye excluding retinopathy of prematurity and familial exudative vitreoretinopathy. Shorter axial length, vitreoretinal fibrosis and persistent TVL were suggestive of PFV. Suggested management was observation, but milder forms of PFV can be surgically managed.

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.

  References Top

Goldberg MF. Persistent fetal vasculature (PFV): An integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson memorial lecture. Am J Ophthalmol 1997;124:587-626.  Back to cited text no. 1
Pollard ZF. Persistent hyperplastic primary vitreous: Diagnosis, treatment and results. Trans Am Ophthalmol Soc 1997;95:487-549.  Back to cited text no. 2
Shields CL, Schoenberg E, Kocher K, Shukla SY, Kaliki S, Shields JA, et al. Lesions simulating retinoblastoma (pseudoretinoblastoma) in 604 cases: Results based on age at presentation. Ophthalmology 2013;120:311-6.  Back to cited text no. 3


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