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PHOTO ESSAY
Year : 2016  |  Volume : 64  |  Issue : 9  |  Page : 672-673

An unusual case of congenital hypertrophy of retinal pigment epithelium with overlying hemorrhages


Department of Ophthalmology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission02-Dec-2015
Date of Acceptance30-Jul-2016
Date of Web Publication17-Nov-2016

Correspondence Address:
Dr. Shreyas Temkar
Room No. 123, RPC Hostel 1, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0301-4738.194333

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  Abstract 

Keywords: Congenital hypertrophy of retinal pigment epithelium, intraocular tumors, pigmented lesions, retinal pigment epithelium, retinal tumors, retinal vein occlusion


How to cite this article:
Chawla R, Temkar S, Sagar P, Venkatesh P. An unusual case of congenital hypertrophy of retinal pigment epithelium with overlying hemorrhages. Indian J Ophthalmol 2016;64:672-3

How to cite this URL:
Chawla R, Temkar S, Sagar P, Venkatesh P. An unusual case of congenital hypertrophy of retinal pigment epithelium with overlying hemorrhages. Indian J Ophthalmol [serial online] 2016 [cited 2019 Oct 16];64:672-3. Available from: http://www.ijo.in/text.asp?2016/64/9/672/194333

A 24-year-old asymptomatic female was referred to the vitreoretina services in view of the presence of a pigmented lesion in the right eye. Fundus examination showed the presence of a solitary, hyperpigmented flat lesion with scalloped margins in the inferotemporal quadrant suggestive of congenital hypertrophy of retinal pigment epithelium (CHRPE). Scattered retinal hemorrhages were evident over the lesion [Figure 1]. Fundus fluorescein angiography (FFA) showed blocked fluorescence due to pigment epithelial hypertrophy. A small retinal vein within the lesion showed segmented filling suggestive of sluggish flow. Areas of capillary dropout with leakage from smaller venules adjacent to the vein described above were seen [Figure 2]. Spectral domain optical coherence tomography (SD-OCT) of the lesion showed thickened hyper-reflective retinal pigment epithelium (RPE) with shadowing, attenuation of the overlying retinal layers, abruptly terminating outer layers at the edge of the lesion and irregular focal hyper-reflective areas in the inner retinal layers corresponding to intraretinal hemorrhages [Figure 3]. The above features suggested CHRPE with small vein occlusion.
Figure 1: Fundus color image showing a solitary congenital hypertrophy of retinal pigment epithelium lesion with overlying retinal hemorrhages (dashed arrow)

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Figure 2: Fundus fluorescein image of the congenital hypertrophy of retinal pigment epithelium lesion showing blocked fluorescence from pigment epithelial hypertrophy, segmented filling within a small retinal vein suggestive of sluggish flow (arrow) and areas of capillary dropout with leakage from smaller venules (arrowheads)

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Figure 3: Spectral domain optical coherence tomography showing thickened hyper-reflective retinal pigment epithelium with shadowing, attenuation of the overlying retinal layers with abruptly terminating outer layers at the edges of the lesion and irregular focal hyper-reflective areas in the inner retinal layers corresponding to the intraretinal hemorrhages (thick arrow)

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  Discussion Top


Retinal vascular abnormalities overlying CHRPE have been documented in literature.[1],[2] The most common vascular abnormality noted in CHRPE is capillary nonperfusion, which was seen in our case. Other vascular abnormalities include microaneurysms, chorioretinal anastomosis, leak from abnormal capillaries, and focal attenuation of vessels crossing the lesion leading to partial vascular blockage.[1],[2] Intraretinal hemorrhages have not been documented as a spectrum of vascular abnormalities in patients with CHRPE but were a predominant feature in our case supported by SD-OCT. Hemorrhages within the area of CHRPE can also be seen as a feature of choroidal neovascular membrane (CNVM) complicating CHRPE or in adenocarcinoma arising from CHRPE.[3],[4] Our case did not show evidence of CNVM on FFA.[3] There was also no evidence of any nodular mass arising within the lesion excluding adenocarcinoma.[4]

SD-OCT showed attenuation of retinal layers with the loss of retinal architecture and thickened RPE similar to the previous observations.[5] However, choroidal structures were not visible in SD-OCT due to shadowing by hypertrophic RPE as the axial scan was captured through the pigmented area and not the lacunae. Focal hyper-reflective intraretinal lesions corresponding to hemorrhages in fundus photograph were visible. Careful clinical examination along with SD-OCT and FFA identified the benign nature of the lesion in our case.

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Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Cleary PE, Gregor Z, Bird AC. Retinal vascular changes in congenital hypertrophy of the retinal pigment epithelium. Br J Ophthalmol 1976;60:499-503.  Back to cited text no. 1
    
2.
Cohen SY, Quentel G, Guiberteau B, Coscas GJ. Retinal vascular changes in congenital hypertrophy of the retinal pigment epithelium. Ophthalmology 1993;100:471-4.  Back to cited text no. 2
    
3.
Youhnovska P, Toffoli D, Gauthier D. Congenital hypertrophy of the retinal pigment epithelium complicated by a choroidal neovascular membrane. Digit J Ophthalmol 2013;19:24-7.  Back to cited text no. 3
    
4.
Shields JA, Eagle RC Jr., Shields CL, Brown GC, Lally SE. Malignant transformation of congenital hypertrophy of the retinal pigment epithelium. Ophthalmology 2009;116:2213-6.  Back to cited text no. 4
    
5.
Fung AT, Pellegrini M, Shields CL. Congenital hypertrophy of the retinal pigment epithelium: Enhanced-depth imaging optical coherence tomography in 18 cases. Ophthalmology 2014;121:251-6.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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