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OPHTHALMIC IMAGE
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 635

White and dark without pressure with congenital hypertrophy of retinal pigment epithelium


Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, USA

Date of Web Publication28-Apr-2023

Correspondence Address:
Carol L Shields
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut Street, Suite 1440, Philadelphia, PA 19107
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1403_22

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How to cite this article:
Schiller ER, Singla K, Shields CL. White and dark without pressure with congenital hypertrophy of retinal pigment epithelium. Indian J Ophthalmol Case Rep 2023;3:635

How to cite this URL:
Schiller ER, Singla K, Shields CL. White and dark without pressure with congenital hypertrophy of retinal pigment epithelium. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:635. Available from: https://www.ijoreports.in/text.asp?2023/3/2/635/374896



A 12-year-old boy was referred for congenital hypertrophy of the retinal pigment epithelium (CHRPE) in the right eye. Funduscopy revealed concomitant white without pressure (WWOP) and dark without pressure (DWOP) [Figure 1]a and [Figure 1]b. Optical coherence tomography (OCT) through the lesion showed slight retinal pigment epithelium (RPE) hypertrophy with overlying outer retinal loss. WWOP demonstrated hyperreflective ellipsoid layer [Figure 1]c.
Figure 1: (a) Funduscopically, there was CHRPE in the temporal periphery, measuring 3 mm in diameter and surrounded by WWOP (white arrows). (b) There was DWOP superiorly (black arrows). (c) OCT (vertical cut, part A) through the lesion showed slight RPE hypertrophy with overlying outer retinal loss (yellow arrows). WWOP demonstrated hyperreflective ellipsoid layer (blue arrows). CHRPE = congenital hypertrophy of the retinal pigment epithelium, DWOP = dark without pressure, OCT = optical coherence tomography, RPE = retinal pigment epithelium, WWOP = white without pressure

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Shields et al.[1] found WWOP in 1% of patients with CHRPE. WWOP and DWOP are asymptomatic and have no known functional defects.[2] This case is notable, given the infrequent coexisting presence due to the tendency of WWOP to expand[3] and DWOP to regress with age.[4]

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.
Shields CL, Mashayekhi A, Ho T, Cater J, Shields JA. Solitary congenital hypertrophy of the retinal pigment epithelium: Clinical features and frequency of enlargement in 330 patients. Ophthalmology 2003;110:1968-76.  Back to cited text no. 1
    
2.
Li MM, Dalvin LA, Shields CL. Coexisting white and dark without pressure abnormalities surrounding congenital hypertrophy of the retinal pigment epithelium. J Pediatr Ophthalmol Strabismus 2019;56:e5-7.  Back to cited text no. 2
    
3.
Diaz RI, Sigler EJ, Randolph JC, Rafieetary MR, Calzada JI. Spectral domain optical coherence tomography characteristics of white-without-pressure. Retina 2014;34:1020-1.  Back to cited text no. 3
    
4.
Chang MY, McBeath JB, McCannel CA, McCannel TB. “Shadow sign” in congenital hypertrophy of the retinal pigment epithelium of young myopic pigmented patients. Eye (Lond) 2016;30:160-3.  Back to cited text no. 4
    


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