With the advent of optical coherence tomography (OCT), cases of choroidal concavity in the absence of staphyloma or scleral ectasia have been characterized by several investigators, beginning with Jampol et al. in 2006.1 Later, Margolis et al. named this entity “focal choroidal excavation” (FCE) in a report of 12 cases, describing both conforming and nonconforming subtypes.2 The etiology of FCE is still subject to debate, as it has been associated with aberrant choroidal vasculature, pachychoroid disease and inflammatory disorders. An increased prevalence in adulthood suggests that the condition may be acquired.3–6
Well-known retinal complications of blunt trauma include choroidal rupture, Purtscher's retinopathy, Berlin's edema, and choroidal neovascularization. We demonstrate a case of nonconforming FCE detected in a young patient that appeared to result from blunt ocular trauma. Multimodal imaging, including enhanced depth imaging optical coherence tomography (EDI OCT), fundus autofluorescence (FAF), and fluorescein angiography (FA), was used to characterize the lesion.
A 32-year-old male was examined for decreased vision in his left eye. He had sustained blunt trauma to the globe without rupture during a physical altercation several months prior to presentation. His past ocular history was noncontributory. He had no significant medical problems. Slit-lamp examination of the anterior segments was unremarkable. Visual acuity was 20/20 in the right eye and hand motion at 3 feet in the left eye. Intraocular pressure was 12 mm Hg in both eyes. Funduscopic examination of the right eye revealed normal findings. In the left eye, there was a lamellar macular hole (MH) with a gray chorioretinal scar temporal to the fovea (Figure 1A). A second gray chorioretinal scar was seen in the nasal macula. FA demonstrated a crescent-shaped area of leakage in the temporal perifoveal area corresponding to the gray scar (Figure 2). FAF demonstrated central hyperautofluorescence corresponding to the lamellar macular hole, focal hyperautofluorescent spots in the perifoveal region, and hypoautofluorescence corresponding to the nasal area of choroidal rupture (Figure 3). Enhanced depth imaging OCT (Figures 4A and 4B) demonstrated a lamellar MH overlying a nonconforming central focal choroidal excavation. The RPE / Bruch's membrane complex appeared attenuated within the area of FCE. Although thinned, the choroidal structure within the FCE appeared normal. A second area of choroidal thinning in the nasal macula corresponded to a second area of choroidal rupture.
Color fundus photographs of a 32-year-old male 5 months (A) and 8 months after blunt ocular trauma. There is an inner lamellar macular hole centrally and grey crescentic scars in the parafoveal and peripapillary regions.
Late arteriovenous (A: 30 seconds) and recirculation (B: 1 minute and 38 seconds) phase fluorescein angiography of the left eye at presentation show crescentic areas of hyperfluorescent staining corresponding to choroidal rupture. There is minimal dye leakage into the subretinal space overlying the nonconforming focal choroidal excavation.
Fundus autofluorescence of the left eye at presentation shows areas of central hyperautofluorescence corresponding to the lamellar macular hole and focal areas of elongated photoreceptor outer segments overlying a nonconforming focal choroidal excavation. There is a crescentic area of hypoautofluorescence nasally corresponding to an area of choroidal rupture.
Horizontal and vertical enhanced depth imaging optical coherence tomography (EDI OCT) images of the left eye (A, B) 1 month post-trauma shows a nonconforming central focal choroidal excavation (FCE) with an overlying lamellar macular hole and schisis within Henle's fiber layer. Although choroidal thickness within the lesion is diminished, the choroidal structure does not appear to manifest cicatricial changes. The retinal pigment epithelium / Bruch's membrane complex is attenuated within the area of FCE. Three months after blunt trauma, EDI OCT shows reduced subretinal fluid and resolution of the schisis (C). The green arrows indicate the location and direction of the EDI OCT B-scans.
Three months later, the patient's vision had improved to hand motion at 9 feet in the left eye. The funduscopic findings appeared similar those seen at presentation (Figure 1B). Follow-up EDI OCT showed a persistent nonconforming FCE with a small reduction in subretinal fluid and resolution of overlying retinoschisis (Figure 4C).
We present a case of nonconforming focal choroidal excavation with visual loss attributable to blunt nonpenetrating ocular trauma in a young emmetropic patient. The nonconforming FCE appeared to result from a choroidal rupture that was not associated with subretinal hemorrhage or secondary choroidal neovascularization.
Focal choroidal excavation has been described in the context of pachychoroid disease such as central serous chorioretinopathy, pachychoroid neovascularization, and polypoidal choroidal vasculopathy (also known as aneurysmal type 1 neovascularization).3,7 The mechanism for FCE formation remains uncertain, and controversy persists regarding whether FCEs are congenital or acquired. Studies by Park and Oh8 demonstrate that choroidal excavations are not found in young patients without a history of inflammation or trauma, suggesting that they are an acquired condition. In our patient, the detection of FCE following choroidal rupture supports a hypothesis previously proposed by other investigators, who suggested that an intact Bruch's membrane is needed to maintain retinal layer morphology in the horizontal plane.9,10 Thus, FCE may occur following disruption of Bruch's membrane, resulting in prolapse of overlying retina posteriorly. Similar findings have been observed following resolution of inflammatory pigment epithelial detachments in multifocal choroiditis10 and other white dot syndromes.11 We have also observed smaller but similar changes following lacquer crack formation in pathological myopia and associated with defects in Bruch's membrane breaks occurring in pseudoxanthoma elasticum.
In summary, we present a case of nonconforming FCE detected shortly following blunt, nonpenetrating trauma associated with choroidal rupture. We believe that focal disruption of Bruch's membrane — in particular, its elastic layer — may represent a common pathway for FCE formation in a range of clinical settings.12 If FCE are acquired lesions, longitudinal studies describing the evolution of these lesions will aid in the understanding of their pathophysiology.
- Jampol LM, Shankle R, Tornambe P, Tornambe P, Spaide RF, Hee MR. Diagnostic and therapeutic challenges. Retina. 2006;26(9):1072–1076. doi:10.1097/01.iae.0000248819.86737.a5 [CrossRef]
- Margolis R, Mukkamala SK, Jampol LM, et al. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol. 2011;129(10):1320–1325. doi:10.1001/archophthalmol.2011.148 [CrossRef]
- Chung H, Byeon SH, Freund KB. Focal choroidal excavation and its association with pachychoroid spectrum disorders: A review of the literature and multimodal imaging findings. Retina. 2017;37(2):199–221. doi:10.1097/IAE.0000000000001345 [CrossRef]
- Lee CS, Woo SJ, Kim YK, et al. Clinical and spectral-domain optical coherence tomography findings in patients with focal choroidal excavation. Ophthalmology. 2014;121(5):1029–1035. doi:10.1016/j.ophtha.2013.11.043 [CrossRef]
- Obata R, Takahashi H, Ueta T, Yuda K, Kure K, Yanagi Y. Tomographic and angiographic characteristics of eyes with macular focal choroidal excavation. Retina. 2013;33(6):1201–1210. doi:10.1097/IAE.0b013e31827b6452 [CrossRef]
- Lee JH, Lee WK. Choroidal neovascularization associated with focal choroidal excavation. Am J Ophthalmol. 2014;157(3):710–718.e1. doi:10.1016/j.ajo.2013.12.011 [CrossRef]
- Kobayashi W, Abe T, Tamai H, Nakazawa T. Choroidal excavation with polypoidal choroidal vasculopathy: A case report. Clin Ophthalmol. 2012;6:1373–1376. doi:10.2147/OPTH.S33879 [CrossRef]
- Park KA, Oh SY. The absence of focal choroidal excavation in children and adolescents without retinal or choroidal disorders or ocular trauma. Eye (Lond). 2015;29(6):841–842. doi:10.1038/eye.2015.6 [CrossRef]
- Hashimoto Y, Saito W, Noda K, Ishida S. Acquired focal choroidal excavation associated with multiple evanescent white dot syndrome: Observations at onset and a pathogenic hypothesis. BMC Ophthalmology. 2014;14:135–139. doi:10.1186/1471-2415-14-135 [CrossRef]
- Kim H, Woo SJ, Kim YK, Lee SC, Lee CS. Focal choroidal excavation in multifocal choroiditis and punctate inner choroidopathy. Ophthalmology. 2015;122(7):1534–1535. doi:10.1016/j.ophtha.2015.01.012 [CrossRef]
- Nishikawa Y, Fujinami K, Watanabe K, Noda T, Tsunoda K, Akiyama K. Clinical course of focal choroidal excavation in Vogt-Koyanagi-Harada disease. Clin Ophthalmol. 2014;8:2461–2465.
- Soma R, Moriyama M, Ohno-Matsui K. Hemodynamics of focal choroidal excavations. Int Ophthalmol. 2015;35(2):261–268. doi:10.1007/s10792-015-0042-1 [CrossRef]