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Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 418-419

Combined hamartoma of the retina and retinal pigment epithelium: Multimodal imaging to the rescue


Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India

Date of Submission01-Feb-2021
Date of Acceptance21-Feb-2021
Date of Web Publication02-Jul-2021

Correspondence Address:
Dr. Pooja Bansal
Vitreoretina Services, Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_279_21

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  Abstract 


Keywords: Combined hamartoma of the retina and retinal pigment epithelium, epiretinal membrane, optical coherence tomography angiography, optical coherence tomography


How to cite this article:
Bansal P, Dipu T, Thakar M. Combined hamartoma of the retina and retinal pigment epithelium: Multimodal imaging to the rescue. Indian J Ophthalmol Case Rep 2021;1:418-9

How to cite this URL:
Bansal P, Dipu T, Thakar M. Combined hamartoma of the retina and retinal pigment epithelium: Multimodal imaging to the rescue. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Jul 26];1:418-9. Available from: https://www.ijoreports.in/text.asp?2021/1/3/418/320013



A 22-year-old boy presented with decreased vision in his right eye. Visual acuity OD was 20/200. Fundus imaging revealed a slightly elevated, grayish macular lesion with tortuous vessels, epiretinal membrane (ERM), and distorted foveal avascular zone [Figure 1]a, [Figure 1]b, [Figure 1]c. Optical coherence tomography (OCT) revealed a tightly adherent ERM, omega-shaped disorganization of the inner retinal layers (IRLs) and retinoschisis, suggestive of combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). Cystic spaces were seen in the retinal nerve fiber layer (RNFL). Outer retinal layers (ORLs) appeared partially disrupted at few places [Figure 1]d, [Figure 1]e, [Figure 1]f. OCTA revealed tortuous, stretched vessels in the superficial and deep capillary plexus (SCP and DCP) [Figure 2]a and [Figure 2]b.
Figure 1: (a) A grey lesion with tortuous vessels at macula. (b) ERM and ILM striae better appreciated in red free image. (c) Distorted FAZ with mild hyperfluorescence on fluorescein angiography. (d-f) OCT scans showing ERM (blue arrow), disorganized inner retinal layers; omega sign (white arrow), retinoschisis (red star), cystic spaces in RNFL draped by internal limiting membrane (blue triangle), intact outer retinal layers (red arrow) and pseudo disrupted ORL (white triangle)

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Figure 2: (a and b) OCTA 3 × 3 mm scans showed distorted FAZ with tortuous vessels in SCP and DCP

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A 17-year-old boy presented with decreased vision in his left eye. Visual acuity (OS) was finger counting 1 m with exotropia. Fundus imaging revealed a small pigmented macular lesion with a disciform epieretinal glial tissue and RPE mottling in periphery [Figure 3]a and [Figure 3]b. OCT showed disorganized IRL (saw tooth appearance), suggestive of macular CHRRPE. A hyper reflective ERM with intraretinal extension was observed, giving impression of a “bird sitting on the retina.” Clumps of hyperreflective foci (HRF) were seen in the IRL, with back shadowing, suggestive of intraretinal RPE cell migration [Figure 3]c and [Figure 3]d. OCTA showed disorganized vasculature in SCP and a round hyperintense lesion in DCP and outer retina slab, with back shadowing [Figure 4].
Figure 3: (a) A grey pigmented lesion with focal ERM at macula. (b) RPE mottling seen as transmission hyperfluorescence. (c and d) OCT showed disorganization of inner retinal layers with saw tooth appearance (white arrow), ERM interwoven with inner retinal layers (blue arrow), subfoveal RPE migration (yellow arrow) and intact ORL in periphery (red arrow)

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Figure 4: OCTA showed (a) distorted perifoveal capillary network in SCP. (b and c) hyperintense lesion in DCP and outer retina. (d) shadow artifact in choroid slab

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  Discussion Top


CHRRPE are rare benign tumors that are often misdiagnosed.[1] Comprehensive retinal examination and multimodal imaging can help in their accurate diagnosis. Both our patients had characteristic OCT features of CHRRPE (omega sign, saw tooth appearance, ERM, and retinoschisis).[2],[3],[4],[5] In addition, case 1 showed cystic spaces in RNFL (moth eaten appearance) and case 2 showed intraretinal RPE cell migration, which have not been described earlier for macular CHRRPE.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gass JD. An unusual hamartoma of the pigment epithelium and retina simulating choroidal melanoma and retinoblastoma. Trans Am Ophthalmol Soc 1973;71:171-83; discussions 184-185.  Back to cited text no. 1
    
2.
Arepalli S, Pellegrini M, Ferenczy S, Shields C. Combined hamartoma of the retina and retinal pigment epithelium: Findings on enhanced depth imaging optical coherence tomography in eight eyes. Retina 2014;34:2202-7.  Back to cited text no. 2
    
3.
Kumar V, Chawla R, Tripathy K. Omega sign: A distinct optical coherence tomography finding in macular combined hamartoma of the retina and retinal pigment epithelium. Opthalmic Surg Lasers Imaging Retina 2017;48:122-5.  Back to cited text no. 3
    
4.
Chawla R, Kumar V, Tripathy K, Kumar A, Venkatesh P, Shaikh FR, et al. Combined hamartoma of the retina and retinal pigment epithelium: An optical coherence tomography-based reappraisal. Am J Ophthalmol 2017;181:88-96.  Back to cited text no. 4
    
5.
Gupta R, Fung AT, Lupidi M, Nayak S, Sahoo NP, Kaliki S, et al. Peripapillary versus macular combined hamartoma of the retina and retinal pigment epithelium: Imaging characteristics . Am J Ophthalmol 2019;200:263-9.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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