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.
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.
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.
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.
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).
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.
- Errera MH, Michaelides M, Keane PA, et al. The extended clinical phenotype of dome-shaped macula. Graefes Arch Clin Exp Ophthalmol. 2014;252(3):499–508. doi:10.1007/s00417-013-2561-7 [CrossRef]
- Liang IC, Shimada N, Tanaka Y, et al. Comparison of clinical features in highly myopic eyes with and without a dome-shaped macula. Ophthalmology. 2015;122(8):1591–600. doi:10.1016/j.ophtha.2015.04.012 [CrossRef]
- Byeon SH, Chu YK. Dome-shaped macula. Am J Ophthalmol. 2011;151(6):1101; author reply 1101–1102. doi:10.1016/j.ajo.2011.01.054 [CrossRef]
- Imamura Y, Iida T, Maruko I, Zweifel SA, Spaide RF. Enhanced depth imaging optical coherence tomography of the sclera in dome-shaped macula. Am J Ophthalmol. 2011;151(2):297–302. doi:10.1016/j.ajo.2010.08.014 [CrossRef]
- Caillaux V, Gaucher D, Gualino V, Massin P, Tadayoni R, Gaudric A. Morphologic characterization of dome-shaped macula in myopic eyes with serous macular detachment. Am J Ophthalmol. 2013;156(5):958–967.e1. doi:10.1016/j.ajo.2013.06.032 [CrossRef]
- Viola F, Dell'Arti L, Benatti E, et al. Choroidal findings in dome-shaped macula in highly myopic eyes: A longitudinal study. Am J Ophthalmol. 2015;159(1):44–52. doi:10.1016/j.ajo.2014.09.026 [CrossRef]
- Dirani A, Matet A, Beydoun T, Mantel I, Behar-Cohen F. Resolution of foveal detachment in dome-shaped macula after treatment by spironolactone: Report of two cases and mini-review of the literature. Clin Ophthalmol. 2014;8:999–1002. doi:10.2147/OPTH.S62267 [CrossRef]
- Suzuki T, Hayakawa K, Nakagawa Y, Onouchi H, Ogata M, Kawai K. Topical dorzolamide for macular edema in the early phase after vitrectomy and epiretinal membrane removal. Clin Ophthalmol. 2013;7:549–553. doi:10.2147/OPTH.S42188 [CrossRef]
- Ikeda Y, Yoshida N, Notomi S, et al. Therapeutic effect of prolonged treatment with topical dorzolamide for cystoid macular oedema in patients with retinitis pigmentosa. Br J Ophthalmol. 2013;97(9):1187–1191. doi:10.1136/bjophthalmol-2012-303005 [CrossRef]
- Huang Q, Chen R, Lin X, Xiang Z. Efficacy of carbonic anhydrase inhibitors in management of cystoid macular edema in retinitis pigmentosa: A meta-analysis. PLoS One. 2017;12(10):e0186180. doi:10.1371/journal.pone.0186180 [CrossRef]
- Siesky B, Harris A, Brizendine E, et al. Literature review and meta-analysis of topical carbonic anhydrase inhibitors and ocular blood flow. Surv Ophthalmol. 2009;54(1):33–46. doi:10.1016/j.survophthal.2008.06.002 [CrossRef]
- Tamura N, Sakai T, Tsuneoka H. Spontaneous resolution of foveal detachment in dome-shaped macula observed by spectral domain optical coherence tomography. Clin Ophthalmol. 2014;8:83–86.
- Kedkovid N, Afshar AR, Damato BE, Stewart JM. Dome-shaped macula with thickened choroid in an emmetropic patient. Retin Cases Brief Rep. 2015;9(4):307–310. doi:10.1097/ICB.0000000000000198 [CrossRef]
- Battaglia Parodi M, Zucchiatti I, Fasce F, Cascavilla ML, Cicinelli MV, Bandello F. Dome-shaped macula associated with Best vitelliform macular dystrophy. Eur J Ophthalmol. 2015;25(2):180–181. doi:10.5301/ejo.5000531 [CrossRef]