- Email: [email protected]
Focal Choroidal Excavation Complicated by Choroidal Neovascularization
Focal Choroidal Excavation Complicated by Choroidal Neovascularization Haifeng Xu, MD, PhD, Fanxing Zeng, MD, Depeng Shi, MD, Xiaolei Sun, MD, Xiuli Chen, MD, Yao Bai Purpose: To evaluate the clinical findings of focal choroidal excavation (FCE) complicated by choroidal neovascularization (CNV). Design: Retrospective, observational case series. Participants: Twelve patients (15 eyes) with FCE and CNV. Methods: The medical records of the patients were reviewed. Clinical findings including age, sex, refraction, color photography, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and spectraldomain optical coherence tomography (OCT) were analyzed. Main Outcome Measures: Fundus fluorescein angiography, ICGA, and OCT findings. Results: The 12 patients included 6 women and 6 men. The mean age was 46.8±13.4 years (range, 26-64 years). One half of the patients were emmetropic, and the others were myopic (0.5 to 3.0 diopters). All subjects were Chinese. Before CNV development, normal appearance or nonspecific pigment disturbance could be seen around the choroidal excavation. Corresponding to the excavation, window defects were observed by FFA, whereas hypofluorescence was found on ICGA images. Choroidal neovascularization in all eyes was classic as revealed by FFA. The OCT images showed that all eyes had a single choroidal excavation. In 7 of the 15 eyes, the choroidal excavation was located subfoveally, and in the other 8 eyes, it was eccentric. All CNV lesions grew from the bottom or slope of the excavation. Three patients had bilateral involvement. Choroidal neovascularization occurred in both conforming and nonconforming type FCEs, regardless of whether the excavation was shallow or deep, subfoveal or eccentric. All CNV lesions responded well to intravitreal injection of antievascular endothelial growth factor (VEGF) agents. After a single injection, CNV regressed in 13 of 15 eyes. Two eyes received an additional injection. Nonconforming FCE changed to the conforming type after successful treatment of CNV. Conclusions: Focal choroidal excavation is not always stable. Choroidal neovascularization commonly can be seen in patients with FCE and responds well to intravitreal anti-VEGF agents. Ophthalmology 2014;121:246250 ª 2014 by the American Academy of Ophthalmology.
Focal choroidal excavation (FCE) is a newly recognized entity first described by Jampol et al1 in 2006. It is characterized by excavation of the choroid on optical coherence tomography (OCT) images, good visual acuity, and normal or nearly normal appearance of the overlying retina. The lesion can remain stable and show little change over time.2 In 2011, Margolis et al3 expanded the spectrum of this entity by analyzing a series of 12 patients. Focal choroidal excavation was identified as the conforming type and the nonconforming type. The former referred to the outer retinal layers conforming to retinal pigment epithelial alterations within the excavation; the latter referred to a separation between the outer retina and the retinal pigment epithelium (RPE) within the excavation revealed by OCT. Recently, Obata et al4 reported another group of 17 patients (21 eyes) and elaborated the features of this entity further. The cause of this type of lesion is unclear. The affected eyes usually have a good visual acuity and lack a history of trauma, posterior uveitis, retinal or choroidal vascular disease, or prior retinal or choroidal infection. Moreover, the lesions show little change over time, so choroidal excavation once was believed to be only a congenital imperfection and to
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2014 by the American Academy of Ophthalmology Published by Elsevier Inc.
remain stable. Kumano et al5 speculated that the pathogenesis of FCE involved outward traction on the macula caused by choroidal vascular abnormalities because of embryonic developmental failure of the choroid. Choroidal neovascularization (CNV) is one of the complications of FCE. The aim of our study was to describe the clinical and imaging features of FCE complicated with CNV. Herein we report a group of 12 patients who had unilateral (9 patients) or bilateral (3 patients) FCE complicated by CNV.
Methods This study was approved by the institutional review board of Shandong Eye Institute. Medical records of 12 patients (15 eyes) who were diagnosed with FCE accompanied by CNV at Qingdao Eye Hospital, Shandong Eye Institute, between March 2009 and February 2013 were reviewed retrospectively. All patients sought treatment because of visual disturbance, either blurred vision or metamorphopsia. All patients underwent a complete ophthalmic examination, including measurement of best-corrected visual acuity and intraocular pressure and assessment of the anterior segment by slitlamp biomicroscopy. Fundus examination included indirect ISSN 0161-6420/14/$ - see front matter http://dx.doi.org/10.1016/j.ophtha.2013.08.014
Xu et al
FCE Complicated by CNV
Table 1. Characteristics of 12 Patients with Focal Choroidal Excavation Sex
Age (yrs)
Eye
Choroidal Excavation Position
Pretreatment Visual Acuity
Posttreatment Visual Acuity
Follow-up Period (mos)
Refractory Status
1
F
32
2
M
46
3
M
44
4 5 6 7 8 9 10 11 12
F F M M M F M F F
33 47 52 26 59 63 34 62 64
Left Right Left Right Right Left Left Right Right Right Right Left Left Left Right
Eccentric Subfoveal Subfoveal Eccentric Subfoveal Eccentric Subfoveal Subfoveal Subfoveal Eccentric Subfoveal Eccentric Eccentric Eccentric Eccentric
0.3 0.1 0.4 0.4 0.1 0.4 0.05 0.4 0.2 0.2 0.4 0.15 0.25 0.15 0.5
1.0 0.4 0.6 0.6 0.6 0.8 0.2 0.6 0.4 0.6 0.6 0.5 0.8 0.4 0.8
4 45 42 3 6 20 23 12 11 9 7 5 4 3 1
Emmetropic Emmetropic Emmetropic Emmetropic 3.00 D 2.50 D 1.25 De1.2590 Emmetropic 2.0 D Emmetropic þ1.2560 þ0.25 D 0.5 D 0.50 De1.7575 1.25 Dþ0.7590
Patient
D ¼ diopter; F ¼ female; M ¼ male.
ophthalmoscopy, fundus photography (Viscam; Zeiss, Jena, Germany), fundus fluorescein angiography (FFA), and indocyanine green angiography (ICGA; HRA2, Heidelberg, Germany). The OCT images were obtained using the RTVue-100 OCT (Optovue, Fremont, CA) or Spectralis OCT (Spectralis HRAþOCT; Heidelberg Engineering, Heidelberg, Germany). Changes during the follow-up period were revealed using the comparison mode in RTVue-100 OCT or the follow-up function in Spectralis OCT. All patients received intravitreal injection of antievascular endothelial growth factor (VEGF) agents (ranibizumab 0.5 mg or bevacizumab 1.25 mg). The inclusion criteria were (1) OCT images revealing choroidal excavation at the macular area, (2) FFA and OCT images revealing CNV arising from the choroidal excavation, and (3) no medical history affecting visual acuity.
Results Of the 12 patients, 6 were women and 6 were men. Their mean age was 46.813.4 years (range, 26-64 years). All subjects were Chinese. A half of the patients were emmetropic, and another half were myopic (0.5 to 3.0 diopters). Of the FCEs in the fellow eye of 3 bilaterally involved patients, CNV developed 14, 39, and 41 months after their first presentation, respectively (Table 1). In eyes with FCE but no CNV, normal appearance or nonspecific pigment disturbance could be seen around the choroidal excavation on fundus examination. Corresponding to the excavation, FFA revealed a window defect, whereas hypofluorescence could be found on ICGA images. In eyes with CNV, hyperfluorescence representing classic CNV could be seen on FFA images (Figs 1 and 2). The OCT scans revealed that all eyes had a single FCE. In 7 of the 15 eyes, the FCEs were located subfoveally, and the others were eccentric (defined as the distance between the posterior border of the FCE and the fovea being larger than 200 mm). The development of CNV was not related to the depth of the excavation. Choroidal neovascularization occurred either in shallow or deep FCEs, either in conforming or nonconforming FCEs. Moreover, it presented in subfoveal FCE cases, as well as in eccentric cases (Figs 1e4). The shape of the excavation changed with the growing and shrinking of the CNV (Fig 3).
All CNV lesions responded well to intravitreal anti-VEGF therapy. After a single injection, CNV regressed in 13 of 15 eyes. Two eyes needed an additional injection. Visual acuity was improved in all eyes. Nonconforming FCE changed to conforming FCE after successful treatment of CNV (Figs 2 and 3).
Discussion Focal choroidal excavation, an unusual structural change in the eye, recently was discovered thanks to advances in ocular imaging technology and the development of OCT. Until now, altogether 44 eyes (38 patients) with FCE were documented, among which 6 eyes were complicated by CNV and 1 by polypoidal choroidal vasculopathy (PCV).6 In this study, we reported 15 eyes (12 patients) with FCE, 12 of which had combined CNV at their presentation to our institution. During follow-up, CNV occurred in the other 3 eyes. Similar to previous reports, there was no gender predilection, and the mean age was approximately 40 years in this series of patients. As for the refraction, the presence of emmetropia in half of the patients was different from that in other reports. So, moderate myopia may not be a specific character of FCE. Focal choroidal excavation has been regarded simply as a result of structural changes over a patient’s lifespan based on the observations that patients with FCE usually have a good visual acuity and that the excavation remains stable for a long period.2 However, with the increase in reported FCE cases and profound understanding of the entity, the above view has been challenged. In this study, all eyes with FCE had CNV formation at the site of choroidal excavation. Margolis et al3 suggested that choroidal excavation can affect the structure of the RPE and the underlying choroidal layers, resulting in ischemic changes and CNV formation. Obata et al4 concluded that FCE may lead to aberrant choroidal circulation based on their findings of filling defect, venous dilation, focal hyperfluorescence, and punctuate hyperfluorescence on
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Figure 1. Images from a 44-year-old man with a conforming focal choroidal excavation (FCE) complicated by choroidal neovascularization (CNV). A, Color photograph of the left eye at presentation showing retinal pigment epithelial (RPE) alterations adjacent to the fovea (thick arrow). B, Fundus fluorescein angiography image showing irregular hyperfluorescence corresponding to RPE alterations (thick arrow). C, Indocyanine green angiography image showing hypofluorescence in the area temporal-inferior to the fovea, corresponding to the FCE (thick arrows). D, Spectral-domain optical coherence tomography scan obtained through the fovea at presentation showing a conforming FCE. E, Fourteen months later, the patient reported blurred vision in the eye for 2 weeks. Yellowish CNV (arrow) and hemorrhage could be found on the color photograph. F, Fundus fluorescein angiography image obtained 14 months after presentation showing a well-defined CNV (arrow). G, Spectral-domain optical coherence tomography scan obtained 14 months after presentation showing CNV growing from the FCE with overlying retinal thickening.
Figure 2. Images from a 64-year-old woman with a nonconforming focal choroidal excavation (FCE) combined with choroidal neovascularization (CNV). A, Color photograph of the right eye showing a grey nodule in the area nasoinferior to the fovea (CNV, arrow) and depigmentation adjacent to the branch artery (thick arrow). B, C, Early- and late-phase fundus fluorescein angiographic (FFA) images showing well-defined CNV (arrows) and a patch of weak hyperfluorescence corresponding to the FCE (thick arrows). D, E, Early- and middle-phase indocyanine green angiographic images showing well-defined CNV (arrows) and hypofluorescence nasoinferior to the fovea, corresponding to the FCE. F, Spectral-domain optical coherence tomography scan obtained through the fovea on presentation showing a nonconforming FCE (thick arrow) and CNV (arrow). G, Spectral-domain optical coherence tomography scan obtained through the fovea 4 weeks after intravitreal injection of ranibizumab showing the regression of CNV (arrow) and the nonconforming FCE turning to the conforming type (thick arrow).
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FCE Complicated by CNV
Figure 3. Spectral-domain optical coherence tomography (SDOCT) scans from a 33-year-old woman with focal choroidal excavation (FCE) in the left eye. A, An SD OCT scan obtained through the fovea at presentation with a report of metamorphopsia for 1 week showing a nonconforming choroidal excavation and hyperreflective mass (choroidal neovascularization [CNV]) at the superior slope of the excavation (arrow). Best-corrected visual acuity (BCVA) was 1.0. B, An SD OCT scan obtained 25 days later showing that the CNV is much larger, that cystoid changes and neuroretinal detachment are present, and that the FCE becomes flat. The BCVA decreased to 0.05. C, An SD OCT scan obtained 4 weeks after intravitreal injection of Avastin (1.25 mg) showing that the CNV regressed, but there is still residual subretinal fluid. The FCE reappeared. D, An SD OCT scan obtained 7 weeks after intravitreal injection of Avastin showing that the subretinal fluid resolved and the FCE was filled with scar tissue left by the regressed CNV. The BCVA was 0.1.
ICGA images. Kobayashi et al6 determined that CNV and PCV in the FCE arose not only from bad circulation, but also from a collapse of Bruch’s membrane caused by a choroidal excavation. In our series, hypofluorescence also was observed by ICGA. We agreed that both bad circulation and an intact Bruch’s membrane may contribute to the formation of CNV. Focal choroidal excavation can be classified into 2 types: conforming and nonconforming. Margolis et al3 hypothesized that FCE may be a congenital posterior segment malformation. Initially, the elasticity of the retina
Figure 4. Spectral-domain optical coherence tomography (SD OCT) findings from a 32-year-old woman with subfoveal conforming focal choroidal excavation (FCE). A, An SD OCT scan obtained through the fovea at presentation showing a shallow subfoveal FCE. B, An SD OCT scan obtained 41 months after presentation showing CNV growing from the FCE with overlying retinal thickening. C, An SD OCT scan obtained 4 weeks after intravitreal injection of ranibizumab (0.5 mg) showing the regression of CNV.
allowed the photoreceptors to remain attached to the RPE. With time, eyes with conforming FCE progressed to nonconforming lesions as stress on the outer retina resulted in separation of the photoreceptor tips from the apical surface of the RPE. The nonconforming type seemed to be more advanced than the conforming type. In this study, although the FCE type could not be identified exactly because of CNV formation, we found that CNV can be associated with both conforming and nonconforming types. Moreover, after successful treatment of CNV, the nonconforming type changed into the conforming type. Neither the cause and pathologic mechanisms of choroidal excavations nor whether they are congenital or acquired are clear. In the report by Kobayashi et al,6 the lesion did not react to 2 monthly intravitreal injections of ranibizumab, but the pigment epithelial detachment disappeared after the third injection. They suggested that FCE can be followed by devastating changes such as CNV or PCV, so long-term care should be given to patients with FCE. In the current study, all CNV lesions grew from choroidal excavation and
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responded well to anti-VEGF, and most regressed after 1 injection. Moreover, no case had recurrence after successful treatment in this group of patients. According to Kobayashi et al,6 pigment epithelial detachment was not continuous with excavation, so the shape of the excavation did not change after treatment. It may be that FCE and PCV coexisted incidentally but not causatively. The bad response to antiVEGF may be the result of the PCV itself, but not the result of FCE. In summary, FCE seems not always to be stable. Although we have encountered a few FCE cases without CNV (data not shown), we cannot conclude that CNV is more inclined to grow in the site of choroidal excavation because of a lack of strong epidemiologic data, but caution should be taken with these patients. Anti-VEGF treatment for CNV complicated by FCE can achieve favorable effects. Comprehensive understanding of this entity needs accumulation of more cases in the future investigations.
References 1. Jampol L, Shankle J, Schroeder R, et al. Diagnostic and therapeutic challenges. Retina 2006;26:1072–6. 2. Wakabayashi Y, Nishimura A, Higashide T, et al. Unilateral choroidal excavation in the macula detected by spectral-domain optical coherence tomography [report online]. Acta Ophthalmol 2010;88:e87–91. 3. Margolis R, Mukkamala SK, Jampol LM, et al. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320–5. 4. Obata R, Takahashi H, Ueta T, et al. Tomographic and angiographic characteristics of eyes with macular focal choroidal excavation. Retina 2013;33:1201–10. 5. Kumano Y, Nagai H, Enaida H, et al. Symptomatic and morphologic difference between choroidal excavations [report online]. Optom Vis Sci 2013;90:e110–8. 6. Kobayashi W, Abe H, Tamai H, Nakazawa T. Choroidal excavation with polypoidalchoroidal vasculopathy: a case report. Clin Ophthalmol 2012;6:1373–6.
Footnotes and Financial Disclosures Originally received: April 6, 2013. Final revision: July 9, 2013. Accepted: August 12, 2013. Available online: October 4, 2013.
Manuscript no. 2013-569.
Qingdao Eye Hospital, Shandong Eye Institute, Shandong Academy of Medical Sciences, Qingdao, China.
250
Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. Correspondence: Haifeng Xu, MD, PhD, Shandong Eye Institute, 5 Yanerdao Road, Qingdao 266071, China. E-mail: [email protected].
Focal choroidal excavation (FCE) is a newly recognized entity first described by Jampol et al1 in 2006. It is characterized by excavation of the choroid on optical coherence tomography (OCT) images, good visual acuity, and normal or nearly normal appearance of the overlying retina. The lesion can remain stable and show little change over time.2 In 2011, Margolis et al3 expanded the spectrum of this entity by analyzing a series of 12 patients. Focal choroidal excavation was identified as the conforming type and the nonconforming type. The former referred to the outer retinal layers conforming to retinal pigment epithelial alterations within the excavation; the latter referred to a separation between the outer retina and the retinal pigment epithelium (RPE) within the excavation revealed by OCT. Recently, Obata et al4 reported another group of 17 patients (21 eyes) and elaborated the features of this entity further. The cause of this type of lesion is unclear. The affected eyes usually have a good visual acuity and lack a history of trauma, posterior uveitis, retinal or choroidal vascular disease, or prior retinal or choroidal infection. Moreover, the lesions show little change over time, so choroidal excavation once was believed to be only a congenital imperfection and to
246
2014 by the American Academy of Ophthalmology Published by Elsevier Inc.
remain stable. Kumano et al5 speculated that the pathogenesis of FCE involved outward traction on the macula caused by choroidal vascular abnormalities because of embryonic developmental failure of the choroid. Choroidal neovascularization (CNV) is one of the complications of FCE. The aim of our study was to describe the clinical and imaging features of FCE complicated with CNV. Herein we report a group of 12 patients who had unilateral (9 patients) or bilateral (3 patients) FCE complicated by CNV.
Methods This study was approved by the institutional review board of Shandong Eye Institute. Medical records of 12 patients (15 eyes) who were diagnosed with FCE accompanied by CNV at Qingdao Eye Hospital, Shandong Eye Institute, between March 2009 and February 2013 were reviewed retrospectively. All patients sought treatment because of visual disturbance, either blurred vision or metamorphopsia. All patients underwent a complete ophthalmic examination, including measurement of best-corrected visual acuity and intraocular pressure and assessment of the anterior segment by slitlamp biomicroscopy. Fundus examination included indirect ISSN 0161-6420/14/$ - see front matter http://dx.doi.org/10.1016/j.ophtha.2013.08.014
Xu et al
FCE Complicated by CNV
Table 1. Characteristics of 12 Patients with Focal Choroidal Excavation Sex
Age (yrs)
Eye
Choroidal Excavation Position
Pretreatment Visual Acuity
Posttreatment Visual Acuity
Follow-up Period (mos)
Refractory Status
1
F
32
2
M
46
3
M
44
4 5 6 7 8 9 10 11 12
F F M M M F M F F
33 47 52 26 59 63 34 62 64
Left Right Left Right Right Left Left Right Right Right Right Left Left Left Right
Eccentric Subfoveal Subfoveal Eccentric Subfoveal Eccentric Subfoveal Subfoveal Subfoveal Eccentric Subfoveal Eccentric Eccentric Eccentric Eccentric
0.3 0.1 0.4 0.4 0.1 0.4 0.05 0.4 0.2 0.2 0.4 0.15 0.25 0.15 0.5
1.0 0.4 0.6 0.6 0.6 0.8 0.2 0.6 0.4 0.6 0.6 0.5 0.8 0.4 0.8
4 45 42 3 6 20 23 12 11 9 7 5 4 3 1
Emmetropic Emmetropic Emmetropic Emmetropic 3.00 D 2.50 D 1.25 De1.2590 Emmetropic 2.0 D Emmetropic þ1.2560 þ0.25 D 0.5 D 0.50 De1.7575 1.25 Dþ0.7590
Patient
D ¼ diopter; F ¼ female; M ¼ male.
ophthalmoscopy, fundus photography (Viscam; Zeiss, Jena, Germany), fundus fluorescein angiography (FFA), and indocyanine green angiography (ICGA; HRA2, Heidelberg, Germany). The OCT images were obtained using the RTVue-100 OCT (Optovue, Fremont, CA) or Spectralis OCT (Spectralis HRAþOCT; Heidelberg Engineering, Heidelberg, Germany). Changes during the follow-up period were revealed using the comparison mode in RTVue-100 OCT or the follow-up function in Spectralis OCT. All patients received intravitreal injection of antievascular endothelial growth factor (VEGF) agents (ranibizumab 0.5 mg or bevacizumab 1.25 mg). The inclusion criteria were (1) OCT images revealing choroidal excavation at the macular area, (2) FFA and OCT images revealing CNV arising from the choroidal excavation, and (3) no medical history affecting visual acuity.
Results Of the 12 patients, 6 were women and 6 were men. Their mean age was 46.813.4 years (range, 26-64 years). All subjects were Chinese. A half of the patients were emmetropic, and another half were myopic (0.5 to 3.0 diopters). Of the FCEs in the fellow eye of 3 bilaterally involved patients, CNV developed 14, 39, and 41 months after their first presentation, respectively (Table 1). In eyes with FCE but no CNV, normal appearance or nonspecific pigment disturbance could be seen around the choroidal excavation on fundus examination. Corresponding to the excavation, FFA revealed a window defect, whereas hypofluorescence could be found on ICGA images. In eyes with CNV, hyperfluorescence representing classic CNV could be seen on FFA images (Figs 1 and 2). The OCT scans revealed that all eyes had a single FCE. In 7 of the 15 eyes, the FCEs were located subfoveally, and the others were eccentric (defined as the distance between the posterior border of the FCE and the fovea being larger than 200 mm). The development of CNV was not related to the depth of the excavation. Choroidal neovascularization occurred either in shallow or deep FCEs, either in conforming or nonconforming FCEs. Moreover, it presented in subfoveal FCE cases, as well as in eccentric cases (Figs 1e4). The shape of the excavation changed with the growing and shrinking of the CNV (Fig 3).
All CNV lesions responded well to intravitreal anti-VEGF therapy. After a single injection, CNV regressed in 13 of 15 eyes. Two eyes needed an additional injection. Visual acuity was improved in all eyes. Nonconforming FCE changed to conforming FCE after successful treatment of CNV (Figs 2 and 3).
Discussion Focal choroidal excavation, an unusual structural change in the eye, recently was discovered thanks to advances in ocular imaging technology and the development of OCT. Until now, altogether 44 eyes (38 patients) with FCE were documented, among which 6 eyes were complicated by CNV and 1 by polypoidal choroidal vasculopathy (PCV).6 In this study, we reported 15 eyes (12 patients) with FCE, 12 of which had combined CNV at their presentation to our institution. During follow-up, CNV occurred in the other 3 eyes. Similar to previous reports, there was no gender predilection, and the mean age was approximately 40 years in this series of patients. As for the refraction, the presence of emmetropia in half of the patients was different from that in other reports. So, moderate myopia may not be a specific character of FCE. Focal choroidal excavation has been regarded simply as a result of structural changes over a patient’s lifespan based on the observations that patients with FCE usually have a good visual acuity and that the excavation remains stable for a long period.2 However, with the increase in reported FCE cases and profound understanding of the entity, the above view has been challenged. In this study, all eyes with FCE had CNV formation at the site of choroidal excavation. Margolis et al3 suggested that choroidal excavation can affect the structure of the RPE and the underlying choroidal layers, resulting in ischemic changes and CNV formation. Obata et al4 concluded that FCE may lead to aberrant choroidal circulation based on their findings of filling defect, venous dilation, focal hyperfluorescence, and punctuate hyperfluorescence on
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Figure 1. Images from a 44-year-old man with a conforming focal choroidal excavation (FCE) complicated by choroidal neovascularization (CNV). A, Color photograph of the left eye at presentation showing retinal pigment epithelial (RPE) alterations adjacent to the fovea (thick arrow). B, Fundus fluorescein angiography image showing irregular hyperfluorescence corresponding to RPE alterations (thick arrow). C, Indocyanine green angiography image showing hypofluorescence in the area temporal-inferior to the fovea, corresponding to the FCE (thick arrows). D, Spectral-domain optical coherence tomography scan obtained through the fovea at presentation showing a conforming FCE. E, Fourteen months later, the patient reported blurred vision in the eye for 2 weeks. Yellowish CNV (arrow) and hemorrhage could be found on the color photograph. F, Fundus fluorescein angiography image obtained 14 months after presentation showing a well-defined CNV (arrow). G, Spectral-domain optical coherence tomography scan obtained 14 months after presentation showing CNV growing from the FCE with overlying retinal thickening.
Figure 2. Images from a 64-year-old woman with a nonconforming focal choroidal excavation (FCE) combined with choroidal neovascularization (CNV). A, Color photograph of the right eye showing a grey nodule in the area nasoinferior to the fovea (CNV, arrow) and depigmentation adjacent to the branch artery (thick arrow). B, C, Early- and late-phase fundus fluorescein angiographic (FFA) images showing well-defined CNV (arrows) and a patch of weak hyperfluorescence corresponding to the FCE (thick arrows). D, E, Early- and middle-phase indocyanine green angiographic images showing well-defined CNV (arrows) and hypofluorescence nasoinferior to the fovea, corresponding to the FCE. F, Spectral-domain optical coherence tomography scan obtained through the fovea on presentation showing a nonconforming FCE (thick arrow) and CNV (arrow). G, Spectral-domain optical coherence tomography scan obtained through the fovea 4 weeks after intravitreal injection of ranibizumab showing the regression of CNV (arrow) and the nonconforming FCE turning to the conforming type (thick arrow).
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FCE Complicated by CNV
Figure 3. Spectral-domain optical coherence tomography (SDOCT) scans from a 33-year-old woman with focal choroidal excavation (FCE) in the left eye. A, An SD OCT scan obtained through the fovea at presentation with a report of metamorphopsia for 1 week showing a nonconforming choroidal excavation and hyperreflective mass (choroidal neovascularization [CNV]) at the superior slope of the excavation (arrow). Best-corrected visual acuity (BCVA) was 1.0. B, An SD OCT scan obtained 25 days later showing that the CNV is much larger, that cystoid changes and neuroretinal detachment are present, and that the FCE becomes flat. The BCVA decreased to 0.05. C, An SD OCT scan obtained 4 weeks after intravitreal injection of Avastin (1.25 mg) showing that the CNV regressed, but there is still residual subretinal fluid. The FCE reappeared. D, An SD OCT scan obtained 7 weeks after intravitreal injection of Avastin showing that the subretinal fluid resolved and the FCE was filled with scar tissue left by the regressed CNV. The BCVA was 0.1.
ICGA images. Kobayashi et al6 determined that CNV and PCV in the FCE arose not only from bad circulation, but also from a collapse of Bruch’s membrane caused by a choroidal excavation. In our series, hypofluorescence also was observed by ICGA. We agreed that both bad circulation and an intact Bruch’s membrane may contribute to the formation of CNV. Focal choroidal excavation can be classified into 2 types: conforming and nonconforming. Margolis et al3 hypothesized that FCE may be a congenital posterior segment malformation. Initially, the elasticity of the retina
Figure 4. Spectral-domain optical coherence tomography (SD OCT) findings from a 32-year-old woman with subfoveal conforming focal choroidal excavation (FCE). A, An SD OCT scan obtained through the fovea at presentation showing a shallow subfoveal FCE. B, An SD OCT scan obtained 41 months after presentation showing CNV growing from the FCE with overlying retinal thickening. C, An SD OCT scan obtained 4 weeks after intravitreal injection of ranibizumab (0.5 mg) showing the regression of CNV.
allowed the photoreceptors to remain attached to the RPE. With time, eyes with conforming FCE progressed to nonconforming lesions as stress on the outer retina resulted in separation of the photoreceptor tips from the apical surface of the RPE. The nonconforming type seemed to be more advanced than the conforming type. In this study, although the FCE type could not be identified exactly because of CNV formation, we found that CNV can be associated with both conforming and nonconforming types. Moreover, after successful treatment of CNV, the nonconforming type changed into the conforming type. Neither the cause and pathologic mechanisms of choroidal excavations nor whether they are congenital or acquired are clear. In the report by Kobayashi et al,6 the lesion did not react to 2 monthly intravitreal injections of ranibizumab, but the pigment epithelial detachment disappeared after the third injection. They suggested that FCE can be followed by devastating changes such as CNV or PCV, so long-term care should be given to patients with FCE. In the current study, all CNV lesions grew from choroidal excavation and
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responded well to anti-VEGF, and most regressed after 1 injection. Moreover, no case had recurrence after successful treatment in this group of patients. According to Kobayashi et al,6 pigment epithelial detachment was not continuous with excavation, so the shape of the excavation did not change after treatment. It may be that FCE and PCV coexisted incidentally but not causatively. The bad response to antiVEGF may be the result of the PCV itself, but not the result of FCE. In summary, FCE seems not always to be stable. Although we have encountered a few FCE cases without CNV (data not shown), we cannot conclude that CNV is more inclined to grow in the site of choroidal excavation because of a lack of strong epidemiologic data, but caution should be taken with these patients. Anti-VEGF treatment for CNV complicated by FCE can achieve favorable effects. Comprehensive understanding of this entity needs accumulation of more cases in the future investigations.
References 1. Jampol L, Shankle J, Schroeder R, et al. Diagnostic and therapeutic challenges. Retina 2006;26:1072–6. 2. Wakabayashi Y, Nishimura A, Higashide T, et al. Unilateral choroidal excavation in the macula detected by spectral-domain optical coherence tomography [report online]. Acta Ophthalmol 2010;88:e87–91. 3. Margolis R, Mukkamala SK, Jampol LM, et al. The expanded spectrum of focal choroidal excavation. Arch Ophthalmol 2011;129:1320–5. 4. Obata R, Takahashi H, Ueta T, et al. Tomographic and angiographic characteristics of eyes with macular focal choroidal excavation. Retina 2013;33:1201–10. 5. Kumano Y, Nagai H, Enaida H, et al. Symptomatic and morphologic difference between choroidal excavations [report online]. Optom Vis Sci 2013;90:e110–8. 6. Kobayashi W, Abe H, Tamai H, Nakazawa T. Choroidal excavation with polypoidalchoroidal vasculopathy: a case report. Clin Ophthalmol 2012;6:1373–6.
Footnotes and Financial Disclosures Originally received: April 6, 2013. Final revision: July 9, 2013. Accepted: August 12, 2013. Available online: October 4, 2013.
Manuscript no. 2013-569.
Qingdao Eye Hospital, Shandong Eye Institute, Shandong Academy of Medical Sciences, Qingdao, China.
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Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. Correspondence: Haifeng Xu, MD, PhD, Shandong Eye Institute, 5 Yanerdao Road, Qingdao 266071, China. E-mail: [email protected].