• Users Online: 23320
  • Home
  • Print this page
  • Email this page

   Table of Contents      
ONE MINUTE OPHTHALMOLOGY
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
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1310_17

Rights and Permissions

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 2024 Mar 28];66:359. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2018/66/3/359/226100




  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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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
[PUBMED]    
2.
Pollard ZF. Persistent hyperplastic primary vitreous: Diagnosis, treatment and results. Trans Am Ophthalmol Soc 1997;95:487-549.  Back to cited text no. 2
[PUBMED]    
3.
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
    




 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Case Report
What Is Your Nex...
Findings
Discussion
References

 Article Access Statistics
    Viewed1440    
    Printed30    
    Emailed0    
    PDF Downloaded364    
    Comments [Add]    

Recommend this journal