Chorioretinal folds: Associated disorders and a related maculopathy

Timothy W. Olsen; Neal V. Palejwala; Lyndon B. Lee; Chris S. Bergstrom; Steven Yeh

doi:10.1016/j.ajo.2014.02.021

Chorioretinal folds: Associated disorders and a related maculopathy

Timothy W. Olsen, Neal V. Palejwala, Lyndon B. Lee, Chris S. Bergstrom, Steven Yeh

Ophthalmology

Research output: Contribution to journal › Article › peer-review

29 Scopus citations

Abstract

Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.

Original language	English (US)
Pages (from-to)	1038-1047.e1
Journal	American journal of ophthalmology
Volume	157
Issue number	5
DOIs	https://doi.org/10.1016/j.ajo.2014.02.021
State	Published - May 2014

ASJC Scopus subject areas

Ophthalmology

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10.1016/j.ajo.2014.02.021

Cite this

@article{6a0a4eafcc76440bb3c78fe7d88c1caf,

title = "Chorioretinal folds: Associated disorders and a related maculopathy",

abstract = "Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.",

author = "Olsen, {Timothy W.} and Palejwala, {Neal V.} and Lee, {Lyndon B.} and Bergstrom, {Chris S.} and Steven Yeh",

note = "Funding Information: We present 57 eyes of 40 patients with the uncommon clinical sign of CRFs. Similar to other series, we found that most CRFs were secondary to structural changes of the globe. 6,10,12,17 CRFs represent a unique clinical sign that warrants a careful search for systemic or associated disease states. Table 1 lists the more common associations in our case series and strongly suggests that a careful clinical history along with systemic testing is necessary and may lead to the recognition and diagnosis of an autoimmune, inflammatory disorder or neoplastic disorder. Chronic CRFs involving the macula may result in a unique CRF-related maculopathy. In this condition, the tissues and visual function have a specific angiographic appearance that leads to an insidious progression and gradual decline in visual acuity over time. We have described CRF-related maculopathy in 3 stages, with degenerative macular features seen primarily in later stage 3 eyes. The angiographic features resemble the occult CNV described in AMD, referred to as late leakage of undetermined source . 16 However, the CRF-related maculopathy is progressive and the occasional CNV that may result seems to respond to very infrequent anti-VEGF injections. Local orbital pathologic features always should be considered carefully. Specifically, a flattened or thickened posterior sclera that may be detected on MRI imaging or B-scan ultrasonography also is associated with CRFs. 12 Causes for this finding may range from hyperopia (most benign), to posterior scleritis, 6 to orbital tumors. 3,4,8,12,15,18 However, secondary CRFs form from fibrosis or scarring within the chorioretinal interface, such as in involutional exudative AMD. Although secondary CRFs usually are the result of scarring or fibrosis, clearly, there may be an associated significant decline in visual acuity. The suspicion for systemic disease is lower in these cases. In the literature, 2 large case series of CRFs have been published. 19,20 Cangemi and associates published a series of 59 eyes with CRFs and found that the most common associated condition was hyperopia, whereas 17% were idiopathic. 19 Leahey and associates published a series of 78 eyes of 54 patients with the clinical sign of CRFs. 20 In this series, the most common causes of folds were AMD and hyperopia or the cause was idiopathic. We propose that with improved diagnostic testing, the patients formerly referred to as idiopathic continue to represent a smaller portion of the total. In our study of 40 patients, 6 (15%) were considered to be idiopathic. Friberg proposed that such eyes may have had prior episodes of posterior scleritis, thus leading to permanent thickening of the posterior sclera and flattening of the posterior globe. 12 Of our 6 patients with idiopathic folds, 5 had a history of autoimmune disease, including rheumatoid arthritis, systemic lupus erythematosus, and Crohn disease. Therefore, it is plausible that a previous episode of undiagnosed ocular inflammation may have occurred that then led to the formation of CRFs. CRFs have been proposed to develop as the result of tractional forces emanating from the optic nerve. 21 More recently, folds are believed to result from “any intra- or extraocular process that induces sufficient compressive stress within the choroid, Bruch's membrane, and retina to force these tissues to buckle.” 12 Folds may occur secondary to any process causing choroidal swelling, such as an effusion or infiltration, shrinking of the inner sclera (posterior scleritis), or a specific mechanical deformation. The difference in surface area between the 2 layers creates tension and stress on the Bruch membrane with subsequent CRF formation. As Friberg and Lace have demonstrated, the greater elasticity of the choroid compared with the sclera explains why the choroid folds and the sclera flattens. 22 Secondary folds may form as a result of abnormal adhesions within the chorioretinal interface. Chorioretinal scars and subretinal neovascularization may lead to fibrosis, contraction, and secondary tractional influences on the surrounding retina and choroid, producing radial CRFs ( Figure 1 ). 7,13,23 For each cause of CRFs, the anatomic and pathophysiologic response has a final common pathway that either may resolve acutely or may develop into any of the 3 stages of CRF-related maculopathy. Diagnostic imaging methods such as color fundus photography, enhanced-depth OCT, FA, or indocyanine green angiography may help to characterize the pathologic and anatomic changes occurring in the choroid, the Bruch membrane, and RPE layers. Haruyama and associates previously described the appearance of indocyanine green angiography results in patients with CRFs. 7 The authors show delayed filling in choroidal vessels during the early transit phase followed by choroidal venous dilation ( Figure 7 ), suggesting choroidal vascular congestion. Vascular congestion of the choroid may result from resistance of choroidal outflow that may result from extravascular interstitial edema that accompanies many inflammatory conditions. Additionally, there may be structural alterations within the scleral wall that may impair inflow through the short posterior ciliary arteries or outflow through the vortex ampullae. Poor venous outflow, delayed choroidal filling, and interstitial edema may result in an ischemic choroidal environment. Logically, similar abnormalities are not seen in patients with secondary radial folds. 7 Gass suggests that there are regions of high tensile stress that form over time along the convex surface and valleys of the CRFs. 14 Clearly, there is a linear relationship between aging and the loss of elasticity in the Bruch membrane–choroid complex. As the Bruch membrane ages, the structure becomes thickened and calcified. 22 The accompanying increase in fragility of the Bruch membrane along with the mechanical stress created by the folds will predispose the Bruch membrane to fracture in the macular region, especially in an aged eye. A fractured Bruch membrane in a relatively ischemic environment creates an ideal situation for formation of CNV. Interestingly, in this series, we found relatively few cases of CNV associated with CRFs. Four (10%) of our 40 patients demonstrated CNV. Of the stage 3 CRF-related maculopathy eyes, we found that 4 (40%) of 10 demonstrated evidence of CNV. Therefore, later stages should be monitored more closely for signs and symptoms related to the new onset of CNV. Although some of the CNV cases occurred before the anti-VEGF era, our limited experience suggests that fewer injections are required than one would suspect in the management of CNV associated with AMD. Of those who were treated, none showed improvement in visual acuity, whereas 2 had a decrease in acuity. One patient was treated with intravitreal bevacizumab for an extrafoveal CNV and had an improvement in angiographic leakage, yet no improvement in visual acuity. Another received a single intravitreal ranibizumab injection with resolution of both subretinal hemorrhage and fluid. Given that the acuity loss was more severe in the treatment group as compared with the observation group, CRF-related maculopathy does not seem to warrant aggressive treatment. FA has been extremely helpful in highlighting and identifying CRFs as well as characterizing CRF-related maculopathy. Norton highlighted the alternating pattern of hyperfluorescence and hypofluorescence that corresponds to the crests and valleys of the CRFs, respectively. 24 Gass later attributed this pattern to a greater volume of fluorescein in the folded, thicker choroid, a shorter course traveled by the reflected light at the apex with thinner RPE, and the clustered or redundant RPE within the valleys that blocks fluorescence. 13 Macular pathologic features secondary to chronic folds were reported first by Newell, who noted cases of RPE pigment clumping and atrophy as well as linear streaks of fluorescein staining along the folds. 15 Friberg and Grove presented 2 cases of subretinal neovascularization in the setting of CRFs. 25 In their cases, FA demonstrated late hyperfluorescence corresponding specifically to a small choroidal neovascular complex. They also proposed that the loss of elasticity in the Bruch membrane combined with local stresses from CRFs could precipitate breaks and subsequent subretinal neovascularization. 25 The similarities of CRF-related maculopathy to occult forms of CNV detected in AMD 16 is remarkable. Late leakage of undetermined source as defined in the Macular Photocoagulation Study has a unique fluorescence pattern with early hypofluorescence or delayed filling followed by late, poorly demarcated leaking hyperfluorescence (usually around 2 minutes) in the later phases of the angiogram. 16 We have demonstrated a very similar FA pattern in cases of CRFs that may result from a mechanical injury and relative ischemia at the Bruch membrane ( Figures 4 and 5 ). Clearly, not all CRFs result in this form of maculopathy, yet stage 3 CRF-related maculopathy may be found more commonly in an aged Bruch membrane with choroidal ischemia. Although leakage may represent the vascular incompetence (thus leakage) of a damaged Bruch membrane, one should be careful to assess for subretinal fluid and suspect CNV. We suggest OCT as a recommended complimentary imaging technique to standard angiography. In the patients identified to have angiographic leakage not associated with CNV, we noted a trend toward declining central visual acuity. Most patients in our series were not treated; however, many sought treatment before the anti-VEGF era. Although the risks associated with the use of intravitreal anti-VEGF therapy are low, treatment may not be necessary, because most acuity loss in our series seems to result from degenerative changes to the RPE–Bruchs complex. Although the clinical and angiographic patterns ( ) resemble occult CNV, we suggest that a period of careful observation, imaging, and monitoring is warranted. Table 2 There are several limitations to the current study. First, our study is retrospective. Current technology and therapeutic options were not available over period for collection of these less common cases. There were inconsistent follow-up visits, so a careful comparison or outcome analysis was extremely difficult. Nevertheless, there may also be some significant advantages to examining these patients before the current aggressive use of anti-VEGF agents. Because our patients were studied before current pharmacotherapies, we were better able to study the natural course of CFR-related maculopathy. In summary, we describe a large series of patients with CRFs who were evaluated carefully to detect potential systemic or orbital disease processes. The clinician needs to be acutely aware of the many systemic associations with this important clinical sign. The patient requires a careful medical history and examination for inflammatory, neoplastic, infectious, and infiltrative disorders. A high percentage of our cases had associated inflammatory systemic conditions. Furthermore, we also described the 3 stages of CRF-related maculopathy. This unique maculopathy has been confused with occult CNV. The angiographic pattern of this maculopathy resembles late leakage of undetermined source as defined by the Macular Photocoagulation Study 16 and is seen more commonly in stage 3 CRF-related maculopathy, especially in older individuals. This unique maculopathy has a slow and indolent course with gradual macular dysfunction that evolves over many years. The judicious use of anti-VEGF therapy should be considered when there is OCT evidence of subretinal fluid. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. Supported in part by an unrestricted departmental grant from Research to Prevent Blindness . Involved in Design of study (N.V.P., T.W.O.); Conduct of study (N.V.P., S.Y., C.S.B., T.W.O.); Collection of data (N.V.P., L.B.L., S.Y., C.S.B., T.W.O.; management (S.Y., C.S.B., T.W.O.), analysis (N.V.P., L.B.L., T.W.O.), and interpretation (N.V.P., S.Y., C.S.B., T.W.O.) of data; and preparation (N.V.P., T.W.O.), review (N.V.P., S.Y., C.S.B., T.W.O.), and approval (T.W.O.) of manuscript. Timothy W. Olsen MD is serves as the F. Phinizy Calhoun Sr. Professor of Ophthalmology, Chairman of the Department of Ophthalmology at Emory. He completed both undergraduate and medical school at the University of Kansas, residency training at the University of Minnesota, and Fellowship in Vitreoretinal Diseases and Surgery at Emory. He began his academic career at the University of Wisconsin, founded the Minnesota Lions Macular Degeneration Center and then returned as chairman at Emory. ",

year = "2014",

month = may,

doi = "10.1016/j.ajo.2014.02.021",

language = "English (US)",

volume = "157",

pages = "1038--1047.e1",

journal = "American Journal of Ophthalmology",

issn = "0002-9394",

publisher = "Elsevier USA",

number = "5",

}

TY - JOUR

T1 - Chorioretinal folds

T2 - Associated disorders and a related maculopathy

AU - Olsen, Timothy W.

AU - Palejwala, Neal V.

AU - Lee, Lyndon B.

AU - Bergstrom, Chris S.

AU - Yeh, Steven

N1 - Funding Information: We present 57 eyes of 40 patients with the uncommon clinical sign of CRFs. Similar to other series, we found that most CRFs were secondary to structural changes of the globe. 6,10,12,17 CRFs represent a unique clinical sign that warrants a careful search for systemic or associated disease states. Table 1 lists the more common associations in our case series and strongly suggests that a careful clinical history along with systemic testing is necessary and may lead to the recognition and diagnosis of an autoimmune, inflammatory disorder or neoplastic disorder. Chronic CRFs involving the macula may result in a unique CRF-related maculopathy. In this condition, the tissues and visual function have a specific angiographic appearance that leads to an insidious progression and gradual decline in visual acuity over time. We have described CRF-related maculopathy in 3 stages, with degenerative macular features seen primarily in later stage 3 eyes. The angiographic features resemble the occult CNV described in AMD, referred to as late leakage of undetermined source . 16 However, the CRF-related maculopathy is progressive and the occasional CNV that may result seems to respond to very infrequent anti-VEGF injections. Local orbital pathologic features always should be considered carefully. Specifically, a flattened or thickened posterior sclera that may be detected on MRI imaging or B-scan ultrasonography also is associated with CRFs. 12 Causes for this finding may range from hyperopia (most benign), to posterior scleritis, 6 to orbital tumors. 3,4,8,12,15,18 However, secondary CRFs form from fibrosis or scarring within the chorioretinal interface, such as in involutional exudative AMD. Although secondary CRFs usually are the result of scarring or fibrosis, clearly, there may be an associated significant decline in visual acuity. The suspicion for systemic disease is lower in these cases. In the literature, 2 large case series of CRFs have been published. 19,20 Cangemi and associates published a series of 59 eyes with CRFs and found that the most common associated condition was hyperopia, whereas 17% were idiopathic. 19 Leahey and associates published a series of 78 eyes of 54 patients with the clinical sign of CRFs. 20 In this series, the most common causes of folds were AMD and hyperopia or the cause was idiopathic. We propose that with improved diagnostic testing, the patients formerly referred to as idiopathic continue to represent a smaller portion of the total. In our study of 40 patients, 6 (15%) were considered to be idiopathic. Friberg proposed that such eyes may have had prior episodes of posterior scleritis, thus leading to permanent thickening of the posterior sclera and flattening of the posterior globe. 12 Of our 6 patients with idiopathic folds, 5 had a history of autoimmune disease, including rheumatoid arthritis, systemic lupus erythematosus, and Crohn disease. Therefore, it is plausible that a previous episode of undiagnosed ocular inflammation may have occurred that then led to the formation of CRFs. CRFs have been proposed to develop as the result of tractional forces emanating from the optic nerve. 21 More recently, folds are believed to result from “any intra- or extraocular process that induces sufficient compressive stress within the choroid, Bruch's membrane, and retina to force these tissues to buckle.” 12 Folds may occur secondary to any process causing choroidal swelling, such as an effusion or infiltration, shrinking of the inner sclera (posterior scleritis), or a specific mechanical deformation. The difference in surface area between the 2 layers creates tension and stress on the Bruch membrane with subsequent CRF formation. As Friberg and Lace have demonstrated, the greater elasticity of the choroid compared with the sclera explains why the choroid folds and the sclera flattens. 22 Secondary folds may form as a result of abnormal adhesions within the chorioretinal interface. Chorioretinal scars and subretinal neovascularization may lead to fibrosis, contraction, and secondary tractional influences on the surrounding retina and choroid, producing radial CRFs ( Figure 1 ). 7,13,23 For each cause of CRFs, the anatomic and pathophysiologic response has a final common pathway that either may resolve acutely or may develop into any of the 3 stages of CRF-related maculopathy. Diagnostic imaging methods such as color fundus photography, enhanced-depth OCT, FA, or indocyanine green angiography may help to characterize the pathologic and anatomic changes occurring in the choroid, the Bruch membrane, and RPE layers. Haruyama and associates previously described the appearance of indocyanine green angiography results in patients with CRFs. 7 The authors show delayed filling in choroidal vessels during the early transit phase followed by choroidal venous dilation ( Figure 7 ), suggesting choroidal vascular congestion. Vascular congestion of the choroid may result from resistance of choroidal outflow that may result from extravascular interstitial edema that accompanies many inflammatory conditions. Additionally, there may be structural alterations within the scleral wall that may impair inflow through the short posterior ciliary arteries or outflow through the vortex ampullae. Poor venous outflow, delayed choroidal filling, and interstitial edema may result in an ischemic choroidal environment. Logically, similar abnormalities are not seen in patients with secondary radial folds. 7 Gass suggests that there are regions of high tensile stress that form over time along the convex surface and valleys of the CRFs. 14 Clearly, there is a linear relationship between aging and the loss of elasticity in the Bruch membrane–choroid complex. As the Bruch membrane ages, the structure becomes thickened and calcified. 22 The accompanying increase in fragility of the Bruch membrane along with the mechanical stress created by the folds will predispose the Bruch membrane to fracture in the macular region, especially in an aged eye. A fractured Bruch membrane in a relatively ischemic environment creates an ideal situation for formation of CNV. Interestingly, in this series, we found relatively few cases of CNV associated with CRFs. Four (10%) of our 40 patients demonstrated CNV. Of the stage 3 CRF-related maculopathy eyes, we found that 4 (40%) of 10 demonstrated evidence of CNV. Therefore, later stages should be monitored more closely for signs and symptoms related to the new onset of CNV. Although some of the CNV cases occurred before the anti-VEGF era, our limited experience suggests that fewer injections are required than one would suspect in the management of CNV associated with AMD. Of those who were treated, none showed improvement in visual acuity, whereas 2 had a decrease in acuity. One patient was treated with intravitreal bevacizumab for an extrafoveal CNV and had an improvement in angiographic leakage, yet no improvement in visual acuity. Another received a single intravitreal ranibizumab injection with resolution of both subretinal hemorrhage and fluid. Given that the acuity loss was more severe in the treatment group as compared with the observation group, CRF-related maculopathy does not seem to warrant aggressive treatment. FA has been extremely helpful in highlighting and identifying CRFs as well as characterizing CRF-related maculopathy. Norton highlighted the alternating pattern of hyperfluorescence and hypofluorescence that corresponds to the crests and valleys of the CRFs, respectively. 24 Gass later attributed this pattern to a greater volume of fluorescein in the folded, thicker choroid, a shorter course traveled by the reflected light at the apex with thinner RPE, and the clustered or redundant RPE within the valleys that blocks fluorescence. 13 Macular pathologic features secondary to chronic folds were reported first by Newell, who noted cases of RPE pigment clumping and atrophy as well as linear streaks of fluorescein staining along the folds. 15 Friberg and Grove presented 2 cases of subretinal neovascularization in the setting of CRFs. 25 In their cases, FA demonstrated late hyperfluorescence corresponding specifically to a small choroidal neovascular complex. They also proposed that the loss of elasticity in the Bruch membrane combined with local stresses from CRFs could precipitate breaks and subsequent subretinal neovascularization. 25 The similarities of CRF-related maculopathy to occult forms of CNV detected in AMD 16 is remarkable. Late leakage of undetermined source as defined in the Macular Photocoagulation Study has a unique fluorescence pattern with early hypofluorescence or delayed filling followed by late, poorly demarcated leaking hyperfluorescence (usually around 2 minutes) in the later phases of the angiogram. 16 We have demonstrated a very similar FA pattern in cases of CRFs that may result from a mechanical injury and relative ischemia at the Bruch membrane ( Figures 4 and 5 ). Clearly, not all CRFs result in this form of maculopathy, yet stage 3 CRF-related maculopathy may be found more commonly in an aged Bruch membrane with choroidal ischemia. Although leakage may represent the vascular incompetence (thus leakage) of a damaged Bruch membrane, one should be careful to assess for subretinal fluid and suspect CNV. We suggest OCT as a recommended complimentary imaging technique to standard angiography. In the patients identified to have angiographic leakage not associated with CNV, we noted a trend toward declining central visual acuity. Most patients in our series were not treated; however, many sought treatment before the anti-VEGF era. Although the risks associated with the use of intravitreal anti-VEGF therapy are low, treatment may not be necessary, because most acuity loss in our series seems to result from degenerative changes to the RPE–Bruchs complex. Although the clinical and angiographic patterns ( ) resemble occult CNV, we suggest that a period of careful observation, imaging, and monitoring is warranted. Table 2 There are several limitations to the current study. First, our study is retrospective. Current technology and therapeutic options were not available over period for collection of these less common cases. There were inconsistent follow-up visits, so a careful comparison or outcome analysis was extremely difficult. Nevertheless, there may also be some significant advantages to examining these patients before the current aggressive use of anti-VEGF agents. Because our patients were studied before current pharmacotherapies, we were better able to study the natural course of CFR-related maculopathy. In summary, we describe a large series of patients with CRFs who were evaluated carefully to detect potential systemic or orbital disease processes. The clinician needs to be acutely aware of the many systemic associations with this important clinical sign. The patient requires a careful medical history and examination for inflammatory, neoplastic, infectious, and infiltrative disorders. A high percentage of our cases had associated inflammatory systemic conditions. Furthermore, we also described the 3 stages of CRF-related maculopathy. This unique maculopathy has been confused with occult CNV. The angiographic pattern of this maculopathy resembles late leakage of undetermined source as defined by the Macular Photocoagulation Study 16 and is seen more commonly in stage 3 CRF-related maculopathy, especially in older individuals. This unique maculopathy has a slow and indolent course with gradual macular dysfunction that evolves over many years. The judicious use of anti-VEGF therapy should be considered when there is OCT evidence of subretinal fluid. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. Supported in part by an unrestricted departmental grant from Research to Prevent Blindness . Involved in Design of study (N.V.P., T.W.O.); Conduct of study (N.V.P., S.Y., C.S.B., T.W.O.); Collection of data (N.V.P., L.B.L., S.Y., C.S.B., T.W.O.; management (S.Y., C.S.B., T.W.O.), analysis (N.V.P., L.B.L., T.W.O.), and interpretation (N.V.P., S.Y., C.S.B., T.W.O.) of data; and preparation (N.V.P., T.W.O.), review (N.V.P., S.Y., C.S.B., T.W.O.), and approval (T.W.O.) of manuscript. Timothy W. Olsen MD is serves as the F. Phinizy Calhoun Sr. Professor of Ophthalmology, Chairman of the Department of Ophthalmology at Emory. He completed both undergraduate and medical school at the University of Kansas, residency training at the University of Minnesota, and Fellowship in Vitreoretinal Diseases and Surgery at Emory. He began his academic career at the University of Wisconsin, founded the Minnesota Lions Macular Degeneration Center and then returned as chairman at Emory.

PY - 2014/5

Y1 - 2014/5

N2 - Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.

AB - Purpose To describe a series of chorioretinal folds (CRFs) representing a clinical sign that may be associated with multiple systemic, orbital, and ophthalmologic disorders. We report the associations with systemic disease and describe 3 stages of a CRF-related maculopathy. Design Observational, retrospective case series. Methods We reviewed 57 affected eyes from 40 patients with the clinical sign of CRF from 1 of 2 academic institutions. A careful review of the medical histories and systemic diagnostic evaluations were conducted. Imaging studies were conducted. Results The mean age at diagnosis was 64 ± 17 years. Most eyes (n = 18) were hyperopic (+2.60 ± +2.90 diopters). There were 20 patients (50%) with some form of autoimmune disorder. Overall, the mean presenting visual acuity was 20/50, declining slightly to 20/60 over 19 ± 30 months. Ten eyes had stage 3 CRF-related maculopathy, more common in older individuals with more chronic CRFs. Four stage 3 eyes had associated choroidal neovascularization, and these eyes had 20/60 presenting visual acuity that decreased to 20/100 over approximately 1.5 years. Stage 3 eyes without choroidal neovascularization had a mean presenting visual acuity of 20/40 that decreased to 20/65 over 2.1 years. Conclusions CRFs are associated with numerous ophthalmic and systemic disorders. A careful medical history and evaluation are essential. We describe 3 stages of a unique CRF-related maculopathy. Stage 3 resembles occult choroidal neovascularization, occurs primarily in older individuals with chronic CRFs, and is accompanied by a slow deterioration in central acuity.

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UR - http://www.scopus.com/inward/citedby.url?scp=84898846762&partnerID=8YFLogxK

U2 - 10.1016/j.ajo.2014.02.021

DO - 10.1016/j.ajo.2014.02.021

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AN - SCOPUS:84898846762

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SP - 1038-1047.e1

JO - American Journal of Ophthalmology

JF - American Journal of Ophthalmology

SN - 0002-9394

IS - 5

ER -

Chorioretinal folds: Associated disorders and a related maculopathy

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