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In all instances, optical coherence tomography (OCT) has demonstrated 1 or more focal areas of choroidal depression without any evidence of scleral ectasia or staphyloma. The retina is found either to conform to the path of the choroid or to maintain its normal course and remain separated from the choroid by a hyporeflective area. In this article, we report the first instance of an FCE having both a conforming and a nonconforming retina in the same eye, as well as a case with the highest total number of excavations, at 4.
A healthy 27-year-old female of Chinese descent with no significant ocular history presented with metamorphopsia and decreased vision in the right eye. Best corrected visual acuity was 20/25 in the right eye and 20/20 in the left, with respective refractive errors of −2.50 D and −2.00 D. Anterior segment examination was unremarkable. Of note, the OD macula had subtle changes in the retinal pigment epithelium. Corresponding hyperfluorescent window defects were observed on fluorescein angiography. On spectral domain OCT, 4 discrete areas of FCE were evident—2 conforming and 2 nonconforming. The inner segment/outer segment junction appeared to be somewhat attenuated at each FCE. Of interest, on 2 OCT cuts (Fig. 1, G, H) there appeared to be thickening of the outer plexiform layer. At 8-month follow-up, there was increased separation between the inner segment/outer segment line and the retinal pigment epithelium in the 2 nonconforming lesions. No case of conforming FCE converting to nonconforming FCE has been documented, so long-term follow-up is needed to determine whether this is the natural progression of the disease.
A review of patient characteristics from the literature reveals certain trends (Table 1). Including our case, the majority are young (mean 41 years old); myopic (76%); female (76%); and with unilateral involvement (94%). There does seem to be a preponderance of Asian patients (53%). Recognizing an obvious selection bias, we found that most patients are symptomatic (65%), with either decreased vision or metamorphopsia. Pigmentary changes have been observed in all eyes.
Table 1Patient characteristics in 17 cases of focal choroidal excavation
vision may be significantly affected in this condition. One patient detailed by Margolis et al. had a history of central serous retinopathy and developed a choroidal neovascular membrane. The group notes that the choroid in patients with FCE, as in those with central serous retinopathy, appears to be thickened. One would typically expect myopic individuals to have a thinner choroid, so perhaps this relates to the pathophysiology of the disease or, alternatively, may be a consequence of it.
Although the etiology of FCE is still unknown, in all likelihood it may represent a congenital malformation. As more cases of this interesting condition are reported, we will develop a better understanding of its causes and evolution.