‘shadow sign’ in congenital hypertrophy of the retinal pigment epithelium of young myopic pigmented patients

ABSTRACT PURPOSE Congenital hypertrophy of the retinal pigment epithelium (CHRPE) may simulate choroidal melanoma in certain cases. We report unique clinical features we have observed in

cases of CHRPE in young myopic pigmented patients. METHODS Patients who were referred for evaluation of a suspicious choroidal lesion and found to have a CHRPE lesion with the clinical

appearance of lesion elevation and a subretinal fluid-like ‘shadow sign’ were included. Patient and lesion characteristics were tabulated. Available images, including fundus photography,

ultrasonography, optical coherence tomography (OCT), and fluorescein angiography (FA) were reviewed. RESULTS Six patients were included. The ‘shadow sign’ was anterior to the CHRPE lesion in

all cases. The mean age of the patients was 27.3 years. The ethnicities of the patients were Chinese (_n_=1), Hispanic (_n_=3), or African-American (_n_=2). Five of six patients were

myopic. CONCLUSIONS Although most CHRPE lesions appear flat on ophthalmoscopy, lesions in young myopic patients of pigmented ethnicities may appear elevated with a ‘shadow sign’ due to ‘dark

without pressure.’ This new finding may be related to the vitreoretinal interface in young myopic pigmented patients and must be distinguished from true subretinal fluid and lesion

thickness, which are often observed in choroidal melanoma. SIMILAR CONTENT BEING VIEWED BY OTHERS SMALL DOME-SHAPED PIGMENT EPITHELIUM DETACHMENT IN POLYPOIDAL CHOROIDAL VASCULOPATHY: AN

UNDER-RECOGNIZED SIGN OF POLYPOIDAL LESIONS ON OPTICAL COHERENCE TOMOGRAPHY? Article 08 April 2021 ANALYSIS OF CLINICAL FEATURES AND SS-OCT FINDINGS IN PATIENTS WITH FOCAL CHOROIDAL

EXCAVATION Article Open access 09 April 2025 HISTOLOGY OF NEOVASCULAR MYOPIC MACULAR DEGENERATION Article Open access 09 November 2021 INTRODUCTION Congenital hypertrophy of the retinal

pigment epithelium (CHRPE) is a benign disorder of the retinal pigment epithelium (RPE) classically described as flat, well-delineated, and with depigmented lacunae developing over time.1, 2

We describe six cases in which a ‘shadow sign’ simulated subretinal fluid and lesion elevation, giving the false impression of a malignant process. MATERIALS AND METHODS Patients who were

referred for evaluation of a suspicious choroidal lesion and found to have a CHRPE lesion with the clinical appearance of lesion elevation and a subretinal fluid-like ‘shadow sign’ were

included. Patients’ age, sex, race, refraction, and lesion characteristics were tabulated. Imaging studies, including fundus photography, ultrasonography, optical coherence tomography (OCT),

and fluorescein angiography (FA), were reviewed when available. RESULTS Six patients were identified who were referred for evaluation of a suspicious choroidal lesion and were found to have

CHRPE with an associated ‘shadow sign’ (Table 1). The mean age of the patients was 27.3 years (range 12–41 years). The ethnicities of the patients were Chinese (_n_=1), Hispanic (_n_=3) or

African-American (_n_=2). Five of six patients were myopic (range −1.00 to −5.00 diopters, spherical equivalent). Color fundus photographs of patients one through three are shown in Figures

1, 2, 3. Ultra wide-field fundus photography was used in Patient 1, but standard color photography was used on the other five patients. In all six cases, the ‘shadow’ was located anteriorly,

simulating the appearance of subretinal fluid. In three cases, the shadow created the appearance of lesion elevation. However, ultrasonography and OCT in all cases confirmed that the

lesions were flat with no associated subretinal fluid. In addition, OCT demonstrated outer retinal atrophy with loss of the ellipsoid and interdigitation zones in the area of the CHRPE

lesion (Figure 2b). In the area of the ‘shadow,’ OCT showed hyporeflectivity of these same layers. Fluorescein angiography demonstrated blocking of the CHRPE lesion, whereas the ‘shadow’ was

indistinguishable from the surrounding retina (Figure 2c). In three patients, follow-up over 3 years demonstrated reduction or disappearance of the ‘shadow sign.’ DISCUSSION We describe for

the first time a ‘shadow sign’ associated with CHRPE lesions in young, myopic patients with hyperpigmentation of the fundus associated with race. Although the shadow may simulate the

presence of subretinal fluid or lesion elevation on clinical examination, ultrasonography, and optical coherence tomography (OCT) confirm that the lesion is flat and there is no fluid

present. The appearance of the ‘shadow’ matches the description for ‘dark without pressure’ first published by Nagpal _et al_,3 as ‘homogeneous, geographical, flat, brown areas.’ Fawzi _et

al_4 reported that the OCT characteristics of these ‘dark without pressure’ lesions consist of hyporeflectivity of photoreceptor zones, consistent with the OCT findings in our cases. As in

our study, no vitreoretinal alterations were found on OCT, although the authors acknowledge the possibility that the changes may have been precipitated by vitreous traction. The cause of the

related phenomenon of ‘white without pressure’ has been traditionally attributed to vitreoretinal alteration based on histopathological studies.5 The reason for the association of CHRPE

lesions with ‘dark without pressure’ in our patients is unclear. CHRPE lesions have been found to be associated with ‘white without pressure’ in 1% of cases,1 and it is possible that they

may cause vitreoretinal interface alterations leading to the either ‘dark without pressure’ or ‘white without pressure’ based on fundus pigmentation. In a series of 10 patients with ‘white’

or ‘dark without pressure,’ 6 of 7 patients with ‘dark without pressure’ were of pigmented race, whereas 2 of 3 patients with ‘white without pressure’ were Caucasian.4 In our series, all

patients were of pigmented race with hyperpigmented fundi, and this may have led to the appearance of ‘dark without pressure’ rather than ‘white without pressure.’ In Cases 4–6, longer

follow-up revealed a decrease in the shadow sign, which may be related to the theoretical breakdown of the vitreous with age reducing the prominence of the vitreoretinal interface. The fact

that we do not see this shadow in older patients may attest to this. Finally, myopia was also a common denominator in five of the six patients presented, and vitreoretinal interface

alterations influenced by myopia are well described.6, 7, 8 In summary, we report the ‘shadow sign’ with CHRPE lesions in young, myopic, pigmented patients, which may simulate the clinical

appearance of subretinal fluid and/or lesion thickness. Clinicians must be aware that this sign may cause a benign process to appear malignant on ophthalmoscopy, and we recommend the use of

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to Prevent Blindness and the George E. and Ruth Moss Trust. AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Ophthalmology, Stein Eye Institute, University of California, Los

Angeles, CA, USA M Y Chang, C A McCannel & T A McCannel * South Coast Retina Center, Long Beach, CA, USA J B McBeath Authors * M Y Chang View author publications You can also search for

this author inPubMed Google Scholar * J B McBeath View author publications You can also search for this author inPubMed Google Scholar * C A McCannel View author publications You can also

search for this author inPubMed Google Scholar * T A McCannel View author publications You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to T A

McCannel. ETHICS DECLARATIONS COMPETING INTERESTS The authors declare no conflict of interest. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Chang, M.,

McBeath, J., McCannel, C. _et al._ ‘Shadow sign’ in congenital hypertrophy of the retinal pigment epithelium of young myopic pigmented patients. _Eye_ 30, 160–163 (2016).

https://doi.org/10.1038/eye.2015.187 Download citation * Received: 12 April 2015 * Accepted: 25 August 2015 * Published: 16 October 2015 * Issue Date: January 2016 * DOI:

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