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Choroidal neovascularisation secondary to serpiginous choroiditis: The value of OCT-angiography in diagnosis and response to therapy with aflibercept
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Choroidal neovascularisation secondary to serpiginous choroiditis: The value of OCT-angiography in diagnosis and response to therapy with aflibercept夽 R. Campos Polo a,b,∗ , C. Rubio Sánchez a , A. Sánchez Trancón b a b
Hospital Virgen del Puerto, Plasencia, Cáceres, Spain Clínica Vista Sánchez Trancón, Badajoz, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
A case is presented of a 61-year-old man with a personal history of serpiginous choroiditis
Received 30 August 2018
(SC), who presented with metamorphopsia and decreased visual acuity (VA) in his right eye
Accepted 26 December 2018
(RE). In the examination, using Swept Source OCT-angiography (SS OCT-A), peripapillary
Available online 14 March 2019
hemorrhage secondary to a choroidal neovascularisation (CNV) was observed. The patient
Keywords:
and in the SS OCT-A findings.
was treated with intravitreal aflibercept, having a favorable outcome on his symptomatology Serpiginous choroiditis
CNV is a complication that can appear in up to 25% of cases after SC. An accurate diagnosis
Choroidal neovascularisation
using SS OCT-A, with early treatment with aflibercept, led us to obtain optimal clinical and
OCT-angiography
anatomical results. ˜ ˜ S.L.U. All rights © 2019 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
Swept-Source
reserved.
Aflibercept
Neovascularización coroidea secundaria a coroiditis serpiginosa: valor de la OCT-angiografía en el diagnóstico y en la respuesta al tratamiento con aflibercept r e s u m e n Palabras clave:
˜ Varón de 61 anos, con antecedentes personales de coroiditis serpiginosa (CS), que pre-
Coroiditis serpiginosa
sentó metamorfopsia y disminución de agudeza visual (AV) en su ojo derecho (OD). En
Neovascularización coroidea
la exploración, mediante Swept-Source OCT-angiografía (SS OCT-A), se pudo observar una
OCT-angiografía
hemorragia peripapilar secundaria a una neovascularización coroidea (NVC). El paciente
Swept-Source
fue tratado mediante aflibercept intravítreo, presentando una evolución favorable en su
Aflibercept
sintomatología y en los hallazgos de la SS OCT-A.
夽 Please cite this article as: Campos Polo R, Rubio Sánchez C, Sánchez Trancón A. Neovascularización coroidea secundaria a coroiditis serpiginosa: valor de la OCT-angiografía en el diagnóstico y en la respuesta al tratamiento con aflibercept. Arch Soc Esp Oftalmol. 2019;94:460–464. ∗ Corresponding author. E-mail address: [email protected] (R. Campos Polo). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2019 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
461
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
La NVC es una complicación que puede aparecer hasta en el 25% de los casos tras CS. Un diagnóstico preciso, mediante SS OCT-A, unido al tratamiento precoz con aflibercept, nos llevó a obtener unos óptimos resultados, tanto clínicos como anatómicos. ˜ ˜ S.L.U. Todos de Oftalmolog´ıa. Publicado por Elsevier Espana, © 2019 Sociedad Espanola los derechos reservados.
Introduction Serpiginous choroiditis (SC) is a rare entity characterized by inflammation of the choroids, choriocapillary and retina pigment epithelium. The majority of cases are idiopathic although in some occasions it associates to infectious disorders, mainly tuberculosis. It impairs the peripapillary zone and exhibits a geographic pattern with centrifugal progression.1 The case of a patient who exhibited one of the multiple complications described for this disease is presented.
Clinic case report Male, 61, who consulted due to diminished visual acuity (VA) and metamorphopsia in the right eye (RE) with 48 h evolution. The patient had been diagnosed one year earlier with
SC, treated with systemic corticoid therapy during 6 months, subsequently withdrawing treatment due to remission of the disease (Fig. 1). Patient VA was 0.4 in the RE and 0.05 in the left eye (LE). Anterior segment was normal and tonometry was 16 mmHg in both eyes. In the LE, the patient exhibited chorioretinal atrophy plaque in the macular and peripapillary areas without changes when compared to baseline (Fig. 1). In addition to the chorioretinal atrophy plaques typical of SC, RE fundus presented an intraretinal hemorrhage in the papillomacular bundle close to the optic nerve (Fig. 2a). Autofluorescence did not show changes vis-à-vis the previous examination (Fig. 2b). In addition, optical coherence tomography (OCT) and OCT-angiograph (OCT-A) model DRI OCT Triton SS-OCT Angio (Topcon, Medical Systems, Inc., Oakland, NJ, USA) revealed the presence of cysts in middle layers in the B-scan (Fig. 3a), secondary to the existence of active choroidal neovascularization (CNV), visible in OCT-A (Figs. 2c, d and 3b).
Fig. 1 – Retinograph (upper row) and autofluorescence (lower row) of both eyes in quiescent state of SC.
462
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
Fig. 2 – (a) Retinograph showing peripapillary intraretinal hemorrhage. (b) Autofluorescence that does not show significant changes with respect to previous examinations. (c) OCT-A image showing the full CNV extension. (d) OCT-A image showing an enlarged view of the CMB in its section close to the macula.
Considering the clinic, the patient was diagnosed with CNV type 2 secondary to SC, and it was decided to prescribe a single injection of aflibercept (Eylea; Regeneron, Tarrytown, NY, USA, and Bayer, Leverkusen, Germany) in the RE, as compassionate use due to diminished VA and the metamorphopsia exhibited by the patient. One month later, VA improved to 0.7 and cysts disappeared in structural OCT (Fig. 3c). However, the patient continued to refer metamorphopsia, and some vascular fronds could be observed in OCT-A (Fig. 3d). Accordingly, it was decided to administer a new dose of aflibercept, and in the checkup one month later the CNV has completely disappeared both in the B-scan (Fig. 3e) and in the OCT-A image (Fig. 3f). The patient reached a VA of 0.8 without metamorphopsia, and during the follow-up in the 12 subsequent months he did not present any reactivation of SC or CNV.
Discussion OCT-A is a new, noninvasive tool that enables the study of all the retinal vascular layers as well as the detection of CNV without the need to use contrast. This is due to the OCTA technology that provides information about blood flow by detecting the movement of red blood cells within the vessels.2
VA reduction in SC is usually related to the extension of the initial lesion which produces retinal pigment epithelium hyperplasia and fibrosis,3 but it also relates to the appearance of CNV in up to 25% of cases.4 Due to the low frequency of said entity, there are no large studies to support the search for the most adequate treatment, not even in the case of considering anti-VEGF (vascular endothelial growth factor) treatment to learn about the most adequate dosage.5 Accordingly, the authors decided to follow a PRN pattern based on monthly checkups and inject if clinic activity was observed. Aflibercept exhibits a broader action mechanism than other anti-VEGF because, in addition to VEGF-A, it is able to inhibit VEGF-B and the placenta growth factor (PLGF), which also plays an important role as inflammation mediator.6 VEGF overexpression has been demonstrated in CNV secondary to chorioretinal inflammatory diseases, although the pathogeny of this CNV type is complex. Chronic inflammation plays a crucial role because the activation of macrophages and other inflammatory cells induce Bruch membrane degradation which could induce the growth of CNV.7 Various publications have reported satisfactory results in the management of CNV secondary to inflammatory pathologies based on systemic treatment with corticoids and
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
463
Fig. 3 – (a) Structural OCT prior to treatment with aflibercept. (b) OCT-A prior to treatment with aflibercept. (c) Structural OCT image one month after the first aflibercept dose. (d) OCT-A one month after the first aflibercept dose. (e) Structural OCT one month after the second aflibercept dose. (f) OCT-A one month after the second aflibercept dose.
immunosuppressant therapy. However, the side effects of said treatments increase the attractiveness of intravitreal treatment with anti-VEGF.8 The present patient was on systemic treatment which was withdrawn about 18 months ago due to the quiescent state of the disease. The patient was administered 2 injections, a small number when compared to other CNV causes. This could be due to various factors such as being CNV type 2, the small size thereof and the generally young age of patients.9 It has been described that 2 mechanisms participate in the process of forming a CNV: angiogenesis and arteriogenesis. The former consists in the formation of new capillaries from pre-existing vessels in a VEGF-dependent process. In contrast, arteriogenesis consists in the expansion of previously formed vascular channels, making it a posterior mechanism mainly dependent on inflammatory factors.10 Due to the short evolution period of CNV, its main producing mechanism could be angiogenesis and for this reason treatment with aflibercept could account for its complete disappearance.
Conflict of interests No conflict of interests was declared by the authors.
references
1. Buggage RR, Nussemblatt RB. Serpiginous choroiditis. Surv Ophthalmol. 2005;50:231–44. 2. Al-Sheikh M, Tepelus TC, Nazikyan T, Sadda SR. Repeatability of automated vessel density measurements using optical coherence tomography angiography. Br J Ophthalmol. 2017;101:449–52. 3. Weiss H, Annesley WH Jr, Shields JA, Tomer T, Christopherson K. The clinical course of serpiginous choroidopathy. Am J Ophthalmol. 1979;87:133–42. 4. Jampol LM, Orth D, Daily MJ, Rabb MF. Subretinal neovascularization with geographic (serpiginous) choroiditis. Am J Ophthalmol. 1979;88:683–9. 5. Battaglia Parodi M, Iacono P, Verbraak FD, Bandello F. Antivascular endothelial growth factors for inflammatory chorioretinal disorders. Dev Ophthalmol. 2010;46: 84–95. 6. Schatz P, Skarin A, Lavaque AJ, Lima LH, Arevalo JF. Preservation of macular structure and function after intravitreal aflibercept for choroidal neovascularization associated with serpiginous choroiditis. Acta Ophthalmol. 2016;94:839–41. 7. Kuo IC, Cunningham ET Jr. Ocular neovascularization in patients with uveitis. Int Ophthalmol Clin. 2000;40:111–26.
464
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
8. Dees C, Arnold JJ, Forrester JV, Dick AD. Immunosuppressive treatment of choroidal neovascularization associated with endogenous posterior uveitis. Arch Ophthalmol. 1998;116:1456–61. 9. Mansour AM, Arevalo JF, Ziemssen F, Mehio-Sibai A, Mackensen F, Adan A, et al. Long-term visual outcomes of
intravitreal bevacizumab in inflammatory ocular neovascularization. Am J Ophthalmol. 2009;148:310–6.e2. 10. Spaide RF. Optical coherence tomography angiography signs of vascular abnormalization with antiangiogenic therapy for choroidal neovascularization. Am J Ophthalmol. 2015;160:6–16.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Choroidal neovascularisation secondary to serpiginous choroiditis: The value of OCT-angiography in diagnosis and response to therapy with aflibercept夽 R. Campos Polo a,b,∗ , C. Rubio Sánchez a , A. Sánchez Trancón b a b
Hospital Virgen del Puerto, Plasencia, Cáceres, Spain Clínica Vista Sánchez Trancón, Badajoz, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
A case is presented of a 61-year-old man with a personal history of serpiginous choroiditis
Received 30 August 2018
(SC), who presented with metamorphopsia and decreased visual acuity (VA) in his right eye
Accepted 26 December 2018
(RE). In the examination, using Swept Source OCT-angiography (SS OCT-A), peripapillary
Available online 14 March 2019
hemorrhage secondary to a choroidal neovascularisation (CNV) was observed. The patient
Keywords:
and in the SS OCT-A findings.
was treated with intravitreal aflibercept, having a favorable outcome on his symptomatology Serpiginous choroiditis
CNV is a complication that can appear in up to 25% of cases after SC. An accurate diagnosis
Choroidal neovascularisation
using SS OCT-A, with early treatment with aflibercept, led us to obtain optimal clinical and
OCT-angiography
anatomical results. ˜ ˜ S.L.U. All rights © 2019 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
Swept-Source
reserved.
Aflibercept
Neovascularización coroidea secundaria a coroiditis serpiginosa: valor de la OCT-angiografía en el diagnóstico y en la respuesta al tratamiento con aflibercept r e s u m e n Palabras clave:
˜ Varón de 61 anos, con antecedentes personales de coroiditis serpiginosa (CS), que pre-
Coroiditis serpiginosa
sentó metamorfopsia y disminución de agudeza visual (AV) en su ojo derecho (OD). En
Neovascularización coroidea
la exploración, mediante Swept-Source OCT-angiografía (SS OCT-A), se pudo observar una
OCT-angiografía
hemorragia peripapilar secundaria a una neovascularización coroidea (NVC). El paciente
Swept-Source
fue tratado mediante aflibercept intravítreo, presentando una evolución favorable en su
Aflibercept
sintomatología y en los hallazgos de la SS OCT-A.
夽 Please cite this article as: Campos Polo R, Rubio Sánchez C, Sánchez Trancón A. Neovascularización coroidea secundaria a coroiditis serpiginosa: valor de la OCT-angiografía en el diagnóstico y en la respuesta al tratamiento con aflibercept. Arch Soc Esp Oftalmol. 2019;94:460–464. ∗ Corresponding author. E-mail address: [email protected] (R. Campos Polo). ˜ ˜ S.L.U. All rights reserved. 2173-5794/© 2019 Sociedad Espanola de Oftalmolog´ıa. Published by Elsevier Espana,
461
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
La NVC es una complicación que puede aparecer hasta en el 25% de los casos tras CS. Un diagnóstico preciso, mediante SS OCT-A, unido al tratamiento precoz con aflibercept, nos llevó a obtener unos óptimos resultados, tanto clínicos como anatómicos. ˜ ˜ S.L.U. Todos de Oftalmolog´ıa. Publicado por Elsevier Espana, © 2019 Sociedad Espanola los derechos reservados.
Introduction Serpiginous choroiditis (SC) is a rare entity characterized by inflammation of the choroids, choriocapillary and retina pigment epithelium. The majority of cases are idiopathic although in some occasions it associates to infectious disorders, mainly tuberculosis. It impairs the peripapillary zone and exhibits a geographic pattern with centrifugal progression.1 The case of a patient who exhibited one of the multiple complications described for this disease is presented.
Clinic case report Male, 61, who consulted due to diminished visual acuity (VA) and metamorphopsia in the right eye (RE) with 48 h evolution. The patient had been diagnosed one year earlier with
SC, treated with systemic corticoid therapy during 6 months, subsequently withdrawing treatment due to remission of the disease (Fig. 1). Patient VA was 0.4 in the RE and 0.05 in the left eye (LE). Anterior segment was normal and tonometry was 16 mmHg in both eyes. In the LE, the patient exhibited chorioretinal atrophy plaque in the macular and peripapillary areas without changes when compared to baseline (Fig. 1). In addition to the chorioretinal atrophy plaques typical of SC, RE fundus presented an intraretinal hemorrhage in the papillomacular bundle close to the optic nerve (Fig. 2a). Autofluorescence did not show changes vis-à-vis the previous examination (Fig. 2b). In addition, optical coherence tomography (OCT) and OCT-angiograph (OCT-A) model DRI OCT Triton SS-OCT Angio (Topcon, Medical Systems, Inc., Oakland, NJ, USA) revealed the presence of cysts in middle layers in the B-scan (Fig. 3a), secondary to the existence of active choroidal neovascularization (CNV), visible in OCT-A (Figs. 2c, d and 3b).
Fig. 1 – Retinograph (upper row) and autofluorescence (lower row) of both eyes in quiescent state of SC.
462
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
Fig. 2 – (a) Retinograph showing peripapillary intraretinal hemorrhage. (b) Autofluorescence that does not show significant changes with respect to previous examinations. (c) OCT-A image showing the full CNV extension. (d) OCT-A image showing an enlarged view of the CMB in its section close to the macula.
Considering the clinic, the patient was diagnosed with CNV type 2 secondary to SC, and it was decided to prescribe a single injection of aflibercept (Eylea; Regeneron, Tarrytown, NY, USA, and Bayer, Leverkusen, Germany) in the RE, as compassionate use due to diminished VA and the metamorphopsia exhibited by the patient. One month later, VA improved to 0.7 and cysts disappeared in structural OCT (Fig. 3c). However, the patient continued to refer metamorphopsia, and some vascular fronds could be observed in OCT-A (Fig. 3d). Accordingly, it was decided to administer a new dose of aflibercept, and in the checkup one month later the CNV has completely disappeared both in the B-scan (Fig. 3e) and in the OCT-A image (Fig. 3f). The patient reached a VA of 0.8 without metamorphopsia, and during the follow-up in the 12 subsequent months he did not present any reactivation of SC or CNV.
Discussion OCT-A is a new, noninvasive tool that enables the study of all the retinal vascular layers as well as the detection of CNV without the need to use contrast. This is due to the OCTA technology that provides information about blood flow by detecting the movement of red blood cells within the vessels.2
VA reduction in SC is usually related to the extension of the initial lesion which produces retinal pigment epithelium hyperplasia and fibrosis,3 but it also relates to the appearance of CNV in up to 25% of cases.4 Due to the low frequency of said entity, there are no large studies to support the search for the most adequate treatment, not even in the case of considering anti-VEGF (vascular endothelial growth factor) treatment to learn about the most adequate dosage.5 Accordingly, the authors decided to follow a PRN pattern based on monthly checkups and inject if clinic activity was observed. Aflibercept exhibits a broader action mechanism than other anti-VEGF because, in addition to VEGF-A, it is able to inhibit VEGF-B and the placenta growth factor (PLGF), which also plays an important role as inflammation mediator.6 VEGF overexpression has been demonstrated in CNV secondary to chorioretinal inflammatory diseases, although the pathogeny of this CNV type is complex. Chronic inflammation plays a crucial role because the activation of macrophages and other inflammatory cells induce Bruch membrane degradation which could induce the growth of CNV.7 Various publications have reported satisfactory results in the management of CNV secondary to inflammatory pathologies based on systemic treatment with corticoids and
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
463
Fig. 3 – (a) Structural OCT prior to treatment with aflibercept. (b) OCT-A prior to treatment with aflibercept. (c) Structural OCT image one month after the first aflibercept dose. (d) OCT-A one month after the first aflibercept dose. (e) Structural OCT one month after the second aflibercept dose. (f) OCT-A one month after the second aflibercept dose.
immunosuppressant therapy. However, the side effects of said treatments increase the attractiveness of intravitreal treatment with anti-VEGF.8 The present patient was on systemic treatment which was withdrawn about 18 months ago due to the quiescent state of the disease. The patient was administered 2 injections, a small number when compared to other CNV causes. This could be due to various factors such as being CNV type 2, the small size thereof and the generally young age of patients.9 It has been described that 2 mechanisms participate in the process of forming a CNV: angiogenesis and arteriogenesis. The former consists in the formation of new capillaries from pre-existing vessels in a VEGF-dependent process. In contrast, arteriogenesis consists in the expansion of previously formed vascular channels, making it a posterior mechanism mainly dependent on inflammatory factors.10 Due to the short evolution period of CNV, its main producing mechanism could be angiogenesis and for this reason treatment with aflibercept could account for its complete disappearance.
Conflict of interests No conflict of interests was declared by the authors.
references
1. Buggage RR, Nussemblatt RB. Serpiginous choroiditis. Surv Ophthalmol. 2005;50:231–44. 2. Al-Sheikh M, Tepelus TC, Nazikyan T, Sadda SR. Repeatability of automated vessel density measurements using optical coherence tomography angiography. Br J Ophthalmol. 2017;101:449–52. 3. Weiss H, Annesley WH Jr, Shields JA, Tomer T, Christopherson K. The clinical course of serpiginous choroidopathy. Am J Ophthalmol. 1979;87:133–42. 4. Jampol LM, Orth D, Daily MJ, Rabb MF. Subretinal neovascularization with geographic (serpiginous) choroiditis. Am J Ophthalmol. 1979;88:683–9. 5. Battaglia Parodi M, Iacono P, Verbraak FD, Bandello F. Antivascular endothelial growth factors for inflammatory chorioretinal disorders. Dev Ophthalmol. 2010;46: 84–95. 6. Schatz P, Skarin A, Lavaque AJ, Lima LH, Arevalo JF. Preservation of macular structure and function after intravitreal aflibercept for choroidal neovascularization associated with serpiginous choroiditis. Acta Ophthalmol. 2016;94:839–41. 7. Kuo IC, Cunningham ET Jr. Ocular neovascularization in patients with uveitis. Int Ophthalmol Clin. 2000;40:111–26.
464
a r c h s o c e s p o f t a l m o l . 2 0 1 9;9 4(9):460–464
8. Dees C, Arnold JJ, Forrester JV, Dick AD. Immunosuppressive treatment of choroidal neovascularization associated with endogenous posterior uveitis. Arch Ophthalmol. 1998;116:1456–61. 9. Mansour AM, Arevalo JF, Ziemssen F, Mehio-Sibai A, Mackensen F, Adan A, et al. Long-term visual outcomes of
intravitreal bevacizumab in inflammatory ocular neovascularization. Am J Ophthalmol. 2009;148:310–6.e2. 10. Spaide RF. Optical coherence tomography angiography signs of vascular abnormalization with antiangiogenic therapy for choroidal neovascularization. Am J Ophthalmol. 2015;160:6–16.