Ophthalmic Surgery, Lasers and Imaging Retina

Case Report 

Resolution of Unilateral Dome-Shaped Macula With Serous Detachment After Treatment of Topical Carbonic Anhydrase Inhibitors

Nan-Ni Chen, MD; Ching-Lung Chen, MD; Chien-Hsiung Lai, MD

Abstract

Dome-shaped macula (DSM) is a morphological feature outlined through the use of optical coherence tomography and characterized by an inward or anterior deviation of the macula with respect to chorioretinal concavity. Currently, there is no validated therapy for serous retinal detachment secondary to DSM. The authors report a case of childhood unilateral DSM in a hyperopic eye with normal axial length that was complicated with subretinal fluid (SRF). Topical carbonic anhydrase inhibitors may be an acceptable and safer option for patients presented with nonresolving SRF associated with DSM. Further randomized controlled trials are necessary to confirm the efficacy of this therapeutic approach.

[Ophthalmic Surg Lasers Imaging Retina. 2019;50:e218–e221.]

Abstract

Dome-shaped macula (DSM) is a morphological feature outlined through the use of optical coherence tomography and characterized by an inward or anterior deviation of the macula with respect to chorioretinal concavity. Currently, there is no validated therapy for serous retinal detachment secondary to DSM. The authors report a case of childhood unilateral DSM in a hyperopic eye with normal axial length that was complicated with subretinal fluid (SRF). Topical carbonic anhydrase inhibitors may be an acceptable and safer option for patients presented with nonresolving SRF associated with DSM. Further randomized controlled trials are necessary to confirm the efficacy of this therapeutic approach.

[Ophthalmic Surg Lasers Imaging Retina. 2019;50:e218–e221.]

Introduction

Dome-shaped macula (DSM) is a morphological feature outlined through the use of optical coherence tomography (OCT) and characterized by an inward or anterior deviation of the macula with respect to chorioretinal concavity. This feature was first reported exclusively in myopic patients with or without posterior staphyloma. Recently this abnormality has been reported in both emmetropic and hypermetropic eyes or in the presence of other macular and retinal disease.1 Reported complications were serous retinal detachment, choroidal neovascularizations (CNVs), lamellar macular holes (MHs), full-thickness MHs, and retinoschisis.2

We present a rare case of a 7-year-old child with unilateral DSM in a nonmyopic eye complicated by serous detachment.

Case Report

A 7-year-old Asian girl experienced progressive blurred vision in her left eye (OS) during a period of 4 months. Visual acuity (VA) was 20/25 in the right eye (OD) and 20/100 OS, with refractive error of +0.00 and +1.00, respectively. Fundus examination OS revealed an oval area of subretinal fluid (SRF) in the macula (Figure 1).

Fundus photograph of the left eye showing an oval area of subretinal fluid in the macula.

Figure 1.

Fundus photograph of the left eye showing an oval area of subretinal fluid in the macula.

A spectral-domain optical coherence tomography (SD-OCT) displayed SRF under the fovea OS. The patient's central macular thickness was 337 μm. A horizontally oriented DSM was noted on the vertical sections of the OCT, with an inward bulge of 500 μm (Figure 2). The choroid showed normal thickness OD (232 μm) but a marked decreased thickness OS (87 μm).

An enhanced depth imaging optical coherence tomography (OCT) of the macula in the left eye (OS) shows a dome-shaped elevation with choroidal thinning, subretinal fluid (SRF), and hyperreflective material at the outer retina. (A) SRF under the fovea with intraretinal and subretinal hyperreflective foci OS. (B) Deposits on the retinal pigment epithelium layer corresponding with the areas of hyperpigmentation, as seen on a fundus examination. (C) Vertical spectral-domain OCT scan shows a dome-shaped macula with a bulge height of 500 μm.

Figure 2.

An enhanced depth imaging optical coherence tomography (OCT) of the macula in the left eye (OS) shows a dome-shaped elevation with choroidal thinning, subretinal fluid (SRF), and hyperreflective material at the outer retina. (A) SRF under the fovea with intraretinal and subretinal hyperreflective foci OS. (B) Deposits on the retinal pigment epithelium layer corresponding with the areas of hyperpigmentation, as seen on a fundus examination. (C) Vertical spectral-domain OCT scan shows a dome-shaped macula with a bulge height of 500 μm.

Upon an ultrasound examination, axial length (AL) was 23.58 mm OD and 23.35 mm OS. Scleral thickness was measured as both peripheral scleral thickness and parafoveal scleral thicknesses, which were 0.42 mm and 0.89 mm, respectively, OD and 0.49 mm and 1.10 mm, respectively, OS. Localized sclera thickening was noted OS (Figure 3). Additionally, a fluorescein angiography demonstrated abnormal hyperfluorescence without leakage OS (Figure 4).

B-scan ultrasonography of the left eye shows a dome shaped elevation in the macula, with localized scleral thickening.

Figure 3.

B-scan ultrasonography of the left eye shows a dome shaped elevation in the macula, with localized scleral thickening.

A fluorescein angiography shows mild hyperfluorescence without leakage inferonasal to the fovea in the left eye (arrow), a blocking defect corresponding with the areas of hyper-pigmentation as seen on a fundus examination (arrow head).

Figure 4.

A fluorescein angiography shows mild hyperfluorescence without leakage inferonasal to the fovea in the left eye (arrow), a blocking defect corresponding with the areas of hyper-pigmentation as seen on a fundus examination (arrow head).

The diagnosis of DSM with SRF OS was made on the basis of characteristic findings. No treatment was initiated for this patient at first. Two months later, a follow-up examination showed persistent SRF. Carbonic anhydrase inhibitors (CAIs) (Trusopt 2%; Merck, Kenilworth, NJ) were then started. We followed up on the patient for 6 months, and a sequential SD-OCT image displayed resolving macula edema (Figure 5), with a decreased central macular thickness (275 μm). VA OS had improved from 20/100 to 20/40, as well. There were no side effects during the topical administration of CAI.

Macular optical coherence tomography after treatment with topical carbonic anhydrase inhibitors. Horizontal section (left) and vertical section (right). Central macular thickness (μm). (A) After 1 month of treatment. (B) After 2 months of treatment. (C) After 4 months of treatment (6 months).

Figure 5.

Macular optical coherence tomography after treatment with topical carbonic anhydrase inhibitors. Horizontal section (left) and vertical section (right). Central macular thickness (μm). (A) After 1 month of treatment. (B) After 2 months of treatment. (C) After 4 months of treatment (6 months).

Discussion

The cause of DSM is not known; however, several studies have postulated a range of theories, including localized choroidal thickening, scleral in-folding, and vitreomacular traction.1

The pathophysiology of SRF in DSM without CNV remains uncertain to date. Byeon and Chu proposed that compressive changes of the choroid and choriocapillaris may be involved, as described by the term scleral compression maculopathy,3 similar to the work by Imamura et al. in nanophthalmos.4

Different from the finding of localized macular scleral thickening and a thickened choroid in highly myopic patients with DSM and SRF,4 in nonmyopic eyes, no evidence of increased macular scleral thickness was found, nor was there any choroid thickening.1 In our patient, the scleral thickness outside of the macular region was similar in both eyes, whereas parafoveal scleral thickness in the lesion eye increased, highlighting the relatively localized region of scleral thickening. This different result, as compared to previous studies, may be the result of our more precise measurement for localized disparity in scleral thickness in the area of the DSM, rather than comparing it between individuals. We presumed this to be the cause of the SRF seen in our patient, which has a positive correlation with the macular bulge height in previous studies, as well.5,6

Except for one case report on two patients that outlined a complete resolution of SFD after the use of oral spironolactone,7 there is no validated therapy for serous RD secondary to DSM. Although the use of anti-vascular endothelial growth factor therapy, photodynamic therapy, and focal laser treatment seems ineffective,5 CAIs have been described as having a positive effect on the resolution of macular edema from various etiologies.8,9 The true mechanisms of how CAIs reduce SRF still remain unclear, whereas CAIs have been proposed to restore a relatively normal distribution of carbonic anhydrase activity and polarity of RPE cells, or have a direct role on the retinal vasculature.10, 11 We proposed that the topical CAI may facilitate SRF resorption, through vascular dilation and increased blood flow, thus achieving partial remission of the scleral compression maculopathy resulting from the DSM.

The DSM presents a fluctuating change in serous RD, with a spontaneous resolution of foveal detachment having been reported.6,12 The possibility of SRF alleviation occurring on its own during a long follow-up period cannot be fully excluded. However, different from other reported cases of spontaneously resolved SRF with DSM, our case presented a persistent DSM, which indicates that the resolution of SRF may be caused by medication, rather than by the spontaneously decreasing DSM.

In summary, there are few case reports of DSM in a nonmyopic eye.1,13,14 We reported a childhood unilateral DSM in hyperopic eye with normal AL, along with SRF. Topical CAIs may be an acceptable and safer option for patients presented with non-resolving SRF associated with DSM. Further randomized, controlled trials are still necessary in order to confirm the efficacy of the therapeutic approach of SFD associated with DSM.

References

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Authors

From the Department of Ophthalmology, Chang Gung Memorial Hospital, Chiayi, Taiwan (NNC, CLC, CHL); the College of Medicine, Chang Gung University, Taoyuan, Taiwan (CLC, CHL); the Department of Optometry, Chung Hwa University of Medical Technology, Taiwan (CLC); the Department of Nursing, Chang Gung University of Science and Technology, Chiayi, Taiwan (CHL); and the School of Traditional Chinese Medicine, College of Medicine, Chang Gung University, Taoyuan, Taiwan (CHL).

The authors report no relevant financial disclosures.

Address correspondence to Chien-Hsiung Lai, MD, Department of Ophthalmology, Chang Gung Memorial Hospital, No. 6, West Section, Chia-Pu Road, Pu-Zih City, Chia-Yi County, Taiwan 61363; email: oph4557@gmail.com.

Received: October 12, 2018
Accepted: February 13, 2019

10.3928/23258160-20190806-16

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