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Optic nerve head drusen associated with neovascular membrane in a child: A case report
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Optic nerve head drusen associated with neovascular membrane in a child: A case report夽 P.M. López-Corell a,∗ , I. Pascual-Camps b , C. Martínez-Rubio a , R. Molina-Pallete a , H. Barranco-González a a b
Unidad de Oftalmología Pediátrica, Servicio de Oftalmología, Hospital Universitario y Politécnico La Fe, Valencia, Spain Servicio de Oftalmología, Hospital Provincial de Castellón, Castellón de la Plana, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Purpose: To describe the anatomical and functional results of intravitreal antiangiogenic
Received 5 December 2017
treatment in optic nerve head drusen associated with choroidal neovascularisation.
Accepted 26 March 2018
Case report: An 8-year-old male patient with a diagnosis of neovascular membrane in the
Available online xxx
right eye associated with bilateral optic nerve head drusen. Best-corrected visual acuity (BCVA) was by counting fingers in the right eye and improved to 0.4 after antiangiogenic
Keywords:
treatment. A complete consolidation of the lesion was observed with no associated compli-
Optic nerve head drusen
cations arising from the treatment.
Choroidal neovascularisation
Discussion/Conclusions: Antiangiogenic treatment is an effective option, and no side-effects
Anti-VEGF
have been observed in treatment of neovascular tissue associated with optic disc drusen in
Children
children. ˜ © 2018 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights
Paediatric
reserved.
Membrana neovascular asociada a drusas del nervio óptico en edad pediátrica: a propósito de un caso r e s u m e n Palabras clave:
Objetivo: Describir los resultados anatómicos y funcionales obtenidos en un caso de neo-
Drusas papilares
vascularización coroidea asociada a drusas papilares tras tratamiento antiangiogénico
Neovascularización coroidea
intravítreo.
Anti-VEGF
˜ Caso clínico: Paciente varón de 8 anos con diagnóstico de membrana neovascular en ojo
˜ Ninos
derecho asociada a drusas papilares bilaterales. La agudeza visual al comienzo fue de cuenta
Pediatría
dedos, mejorando hasta 0,4 tras tratamiento antiangiogénico. Se obtuvo una consolidación completa de la lesión sin complicaciones asociadas derivadas del tratamiento.
夽 Please cite this article as: López-Corell PM, Pascual-Camps I, Martínez-Rubio C, Molina-Pallete R, Barranco-González H. Membrana neovascular asociada a drusas del nervio óptico en edad pediátrica: a propósito de un caso. Arch Soc Esp Oftalmol. 2018. https://doi.org/10.1016/j.oftal.2018.03.009 ∗ Corresponding author. E-mail address: [email protected] (P.M. López-Corell). ˜ 2173-5794/© 2018 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
OFTALE-1331; No. of Pages 4
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
Discusión/Conclusiones: El empleo de terapia antiangiogénica ha resultado efectivo y no se han evidenciado efectos secundarios en el tratamiento de membrana neovascular asociada a drusas en edad pediátrica. ˜ de Oftalmología. Publicado por Elsevier España, S.L.U. Todos © 2018 Sociedad Espanola los derechos reservados.
Introduction
Clinical Case Report
Papillary drusen are the most frequent cause of pseudopapiledema in children, expressing as an elevation and irregular dissemination of edges in the optic disc. Generally, central vision is preserved although the visual field is frequently altered. The presence of drusen could be associated to vascular lesions such as peripapillary hemorrhages or neovascular membranes.1
Patient, age 8, without relevant history, who visited the emergency service due to shadow vision in the right eye with 2 weeks evolution. Vision was finger counting in right eye (RE) and 1 in the left eye. Anterior segment examination was normal, while ocular fundus examination evidenced elevation of both optic discs with dissemination of edges. In
Figure 1 – Retinograph of both eyes, showing in the RE a papillomacular bundle hemorrhage with pigmentation and foveal elevation.
Figure 2 – CT image taken at the emergency department showing papilla calcification, more intensely in the RE.
addition, the RE exhibited papillomacular bundle hemorrhage with pigmentation and foveal elevation (Fig. 1). Computerized tomography (CT) was the imaging technique carried out at the emergency department after finding bilateral papiledema. The presence of papillary calcification enabled the confirmation of papillary drusen diagnostic (Fig. 2) and served to discard suspected neurological pathology. Optical coherence tomography (OCT) image showed the presence of neovascular tissue and sub-and intra-retinal fluid in RE (Fig. 3A). Campimetry revealed inferior nasal quadrantanopsia in the compromised eye while the Farnsworth test was altered with predominance of deuteranopia. Intravitreal treatment with bevacizumab at a dose of 1.25 mg/0.05 mL was established. Said treatment was indicated on the basis of neovascular activity in the tomographic image. Overall, four injections in consecutive months were administered in the RE. Treatment was halted after verifying the absence of activity of the neovascular lesion and regression of the sub-and intra-retinal fluid in OCT images (Fig. 3B–E). Visual acuity improved during the treatment months and was 0.4 at the end thereof. The patient
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
3
Figure 3 – Images of tomographic sections at the foveal level. (A) Diagnostic image, showing subretinal fibrosis associated to sub-and intra-retinal fluid compatible with active neovascular lesion. (B–E) Images corresponding to checkups after treatment. (F) Image of the last checkup after over 6 months without treatment, showing resolution of the sub-and intra-retinal low density fluid with consolidation of subretinal fibrosis.
remained in monthly examinations during 6 months with visual acuity and tomographic image stability and at present is in bi-monthly follow-up on outpatient basis.
Discussion The diagnostic and treatment of an infrequent complication of papillary drusen is described. Typically said drusen are diagnosed in female Caucasians in both eyes.1 Prevalence in children is low due to the under-diagnosing of drusen that remain in deep areas during the first years of life.1,2 Finding of drusen are generally casual, without patients are referring specific symptoms. Visual field alterations debut at approximately 14 years of age when the drusen become superficial.1 Inferior nasal scotoma, present in the visual field of the present patient, is the most frequent campimetric alteration.1,3 In the present case, the diagnostic was early probably due to diminished vision caused by the associated neovascular membrane. Funduscopy is characterized by presenting swollen and elevated papilla, without showing evident cupping due to the accumulation of calcified material in the deep layers. The present case also evidenced the predominantly nasal shiny and irregular dissemination of the papilla which is typical of superficial papillary drusen.1 In some cases, diagnostic is difficult and requires imaging techniques. Mode-B echography is the gold standard.1 The image produced by calcium deposits is hyperechoic with
posterior shadow and can be detected even in deep drusen.1,2,4 CT can also establish the diagnostic although it is not the first choice test.1,2 In the present case, an urgent CT was taken in order to discard neurological disease that could account for the condition. An additional diagnostic option is OCT, with drusen displayed as small hypo-reflective areas surrounded by hypo-reflective strips at right angles of the light beam.3 The nature of papillary drusen is benign although they are frequently associated to vascular complications. Neovascular tissue development is a rare complication that typically occurs between 8 and 24 years of age.4 Duncan et al.5 described a higher prevalence of said complications even though a small percentage thereof are clinically significant and can be detected in daily clinic practice. The most frequent location is peripapillary without macular involvement and preservation of visual acuity.4,6 In the present case the lesion extended to compromise the macula, causing severe vision impairment. Before the development of antiangiogenics, treatment was based on laser photocoagulation, macular surgery or photodynamic therapy.6 The use of antiangiogenics in this pathology is not protocolized but its use is described in references [6–8]. Alkin et al.6 , Delas et al. and Baillif et al.8 carried out treatment with ranibizumab, which required between one and two doses without finding recurrence in follow-up. As in the present case, Knape et al.7 applied bevacizumab treatment but required two doses in combination with laser treatment. In the present case, four doses of antiangiogenics without adjuvant treatment during consecutive months
ARTICLE IN PRESS 4
a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
was established, requesting prior approval for compassionate use of the medicament and obtaining the informed consent of the patient’s parents. The therapeutic regime was sufficient to stabilize the lesion without evidencing subsequent recurrence thereof. The procedure was carried out under sterile conditions and no complications were found. Antiangiogenics treatment is a new therapeutical option for resolving neovascular membranes associated to optic nerve drusen. Due to the importance of the successful resolution of these lesions in pediatric age, it is relatively prioritary to establish an agreed therapeutic protocol.
Conflict of Interest No conflict of interests was declared by the authors.
references
1. Chang MY, Pineles SL. Optic disk drusen in children. Surv Ophthalmol. 2016;61:745–58.
2. Delas B, Almudí L, Carreras A, Asaad M. Bilateral choroidal neovascularization associated with optic nerve head drusen treated by antivascular endotelial growth factor therapy. Clin Ophthalmol. 2012;6:225–30. 3. Flores-Reyes E, Hoskens K, Mansouri K. Optic nerve head drusen: imaging using optical coherence tomography angiography. J Glaucoma. 2017;26:845–9. 4. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol. 2002;47:515–32. 5. Duncan JE, Freedman SF, El-Dairi MA. The incidence of neovascular membranes and visual field defects from optic nerve head drusen in children. J AAPOS. 2016;20: 44–8. 6. Alkin Z, Ozkaya A, Yilmaz I, Yazici AT. A single injection of intravitreal ranibizumab in the treatment of choroidal neovascularization secondary to optic nerve head drusen in a child. BMJ Case Rep. 2014;2:2014. 7. Knape RM, Zavaleta EM, Clark CL 3rd, Khuddus N, Peden MC. Intravitreal.bevacizumab treatment of bilateral peripapillary choroidal neovascularization. from optic nerve head drusen. J AAPOS. 2011;15:87–90. 8. Baillif S, Nguyen E, Colleville-El Hayek A, Bétis F. Long term follow-up after a single intravitreal ranibizumab injection for choroidal neovascularisation secondary to optic nerve head drusen in a 5-year-old child. Graefes Arch Clin Exp Ophthalmol. 2013;251:1657–9.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Optic nerve head drusen associated with neovascular membrane in a child: A case report夽 P.M. López-Corell a,∗ , I. Pascual-Camps b , C. Martínez-Rubio a , R. Molina-Pallete a , H. Barranco-González a a b
Unidad de Oftalmología Pediátrica, Servicio de Oftalmología, Hospital Universitario y Politécnico La Fe, Valencia, Spain Servicio de Oftalmología, Hospital Provincial de Castellón, Castellón de la Plana, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Purpose: To describe the anatomical and functional results of intravitreal antiangiogenic
Received 5 December 2017
treatment in optic nerve head drusen associated with choroidal neovascularisation.
Accepted 26 March 2018
Case report: An 8-year-old male patient with a diagnosis of neovascular membrane in the
Available online xxx
right eye associated with bilateral optic nerve head drusen. Best-corrected visual acuity (BCVA) was by counting fingers in the right eye and improved to 0.4 after antiangiogenic
Keywords:
treatment. A complete consolidation of the lesion was observed with no associated compli-
Optic nerve head drusen
cations arising from the treatment.
Choroidal neovascularisation
Discussion/Conclusions: Antiangiogenic treatment is an effective option, and no side-effects
Anti-VEGF
have been observed in treatment of neovascular tissue associated with optic disc drusen in
Children
children. ˜ © 2018 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights
Paediatric
reserved.
Membrana neovascular asociada a drusas del nervio óptico en edad pediátrica: a propósito de un caso r e s u m e n Palabras clave:
Objetivo: Describir los resultados anatómicos y funcionales obtenidos en un caso de neo-
Drusas papilares
vascularización coroidea asociada a drusas papilares tras tratamiento antiangiogénico
Neovascularización coroidea
intravítreo.
Anti-VEGF
˜ Caso clínico: Paciente varón de 8 anos con diagnóstico de membrana neovascular en ojo
˜ Ninos
derecho asociada a drusas papilares bilaterales. La agudeza visual al comienzo fue de cuenta
Pediatría
dedos, mejorando hasta 0,4 tras tratamiento antiangiogénico. Se obtuvo una consolidación completa de la lesión sin complicaciones asociadas derivadas del tratamiento.
夽 Please cite this article as: López-Corell PM, Pascual-Camps I, Martínez-Rubio C, Molina-Pallete R, Barranco-González H. Membrana neovascular asociada a drusas del nervio óptico en edad pediátrica: a propósito de un caso. Arch Soc Esp Oftalmol. 2018. https://doi.org/10.1016/j.oftal.2018.03.009 ∗ Corresponding author. E-mail address: [email protected] (P.M. López-Corell). ˜ 2173-5794/© 2018 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
OFTALE-1331; No. of Pages 4
ARTICLE IN PRESS 2
a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
Discusión/Conclusiones: El empleo de terapia antiangiogénica ha resultado efectivo y no se han evidenciado efectos secundarios en el tratamiento de membrana neovascular asociada a drusas en edad pediátrica. ˜ de Oftalmología. Publicado por Elsevier España, S.L.U. Todos © 2018 Sociedad Espanola los derechos reservados.
Introduction
Clinical Case Report
Papillary drusen are the most frequent cause of pseudopapiledema in children, expressing as an elevation and irregular dissemination of edges in the optic disc. Generally, central vision is preserved although the visual field is frequently altered. The presence of drusen could be associated to vascular lesions such as peripapillary hemorrhages or neovascular membranes.1
Patient, age 8, without relevant history, who visited the emergency service due to shadow vision in the right eye with 2 weeks evolution. Vision was finger counting in right eye (RE) and 1 in the left eye. Anterior segment examination was normal, while ocular fundus examination evidenced elevation of both optic discs with dissemination of edges. In
Figure 1 – Retinograph of both eyes, showing in the RE a papillomacular bundle hemorrhage with pigmentation and foveal elevation.
Figure 2 – CT image taken at the emergency department showing papilla calcification, more intensely in the RE.
addition, the RE exhibited papillomacular bundle hemorrhage with pigmentation and foveal elevation (Fig. 1). Computerized tomography (CT) was the imaging technique carried out at the emergency department after finding bilateral papiledema. The presence of papillary calcification enabled the confirmation of papillary drusen diagnostic (Fig. 2) and served to discard suspected neurological pathology. Optical coherence tomography (OCT) image showed the presence of neovascular tissue and sub-and intra-retinal fluid in RE (Fig. 3A). Campimetry revealed inferior nasal quadrantanopsia in the compromised eye while the Farnsworth test was altered with predominance of deuteranopia. Intravitreal treatment with bevacizumab at a dose of 1.25 mg/0.05 mL was established. Said treatment was indicated on the basis of neovascular activity in the tomographic image. Overall, four injections in consecutive months were administered in the RE. Treatment was halted after verifying the absence of activity of the neovascular lesion and regression of the sub-and intra-retinal fluid in OCT images (Fig. 3B–E). Visual acuity improved during the treatment months and was 0.4 at the end thereof. The patient
ARTICLE IN PRESS a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
3
Figure 3 – Images of tomographic sections at the foveal level. (A) Diagnostic image, showing subretinal fibrosis associated to sub-and intra-retinal fluid compatible with active neovascular lesion. (B–E) Images corresponding to checkups after treatment. (F) Image of the last checkup after over 6 months without treatment, showing resolution of the sub-and intra-retinal low density fluid with consolidation of subretinal fibrosis.
remained in monthly examinations during 6 months with visual acuity and tomographic image stability and at present is in bi-monthly follow-up on outpatient basis.
Discussion The diagnostic and treatment of an infrequent complication of papillary drusen is described. Typically said drusen are diagnosed in female Caucasians in both eyes.1 Prevalence in children is low due to the under-diagnosing of drusen that remain in deep areas during the first years of life.1,2 Finding of drusen are generally casual, without patients are referring specific symptoms. Visual field alterations debut at approximately 14 years of age when the drusen become superficial.1 Inferior nasal scotoma, present in the visual field of the present patient, is the most frequent campimetric alteration.1,3 In the present case, the diagnostic was early probably due to diminished vision caused by the associated neovascular membrane. Funduscopy is characterized by presenting swollen and elevated papilla, without showing evident cupping due to the accumulation of calcified material in the deep layers. The present case also evidenced the predominantly nasal shiny and irregular dissemination of the papilla which is typical of superficial papillary drusen.1 In some cases, diagnostic is difficult and requires imaging techniques. Mode-B echography is the gold standard.1 The image produced by calcium deposits is hyperechoic with
posterior shadow and can be detected even in deep drusen.1,2,4 CT can also establish the diagnostic although it is not the first choice test.1,2 In the present case, an urgent CT was taken in order to discard neurological disease that could account for the condition. An additional diagnostic option is OCT, with drusen displayed as small hypo-reflective areas surrounded by hypo-reflective strips at right angles of the light beam.3 The nature of papillary drusen is benign although they are frequently associated to vascular complications. Neovascular tissue development is a rare complication that typically occurs between 8 and 24 years of age.4 Duncan et al.5 described a higher prevalence of said complications even though a small percentage thereof are clinically significant and can be detected in daily clinic practice. The most frequent location is peripapillary without macular involvement and preservation of visual acuity.4,6 In the present case the lesion extended to compromise the macula, causing severe vision impairment. Before the development of antiangiogenics, treatment was based on laser photocoagulation, macular surgery or photodynamic therapy.6 The use of antiangiogenics in this pathology is not protocolized but its use is described in references [6–8]. Alkin et al.6 , Delas et al. and Baillif et al.8 carried out treatment with ranibizumab, which required between one and two doses without finding recurrence in follow-up. As in the present case, Knape et al.7 applied bevacizumab treatment but required two doses in combination with laser treatment. In the present case, four doses of antiangiogenics without adjuvant treatment during consecutive months
ARTICLE IN PRESS 4
a r c h s o c e s p o f t a l m o l . 2 0 1 8;x x x(x x):xxx–xxx
was established, requesting prior approval for compassionate use of the medicament and obtaining the informed consent of the patient’s parents. The therapeutic regime was sufficient to stabilize the lesion without evidencing subsequent recurrence thereof. The procedure was carried out under sterile conditions and no complications were found. Antiangiogenics treatment is a new therapeutical option for resolving neovascular membranes associated to optic nerve drusen. Due to the importance of the successful resolution of these lesions in pediatric age, it is relatively prioritary to establish an agreed therapeutic protocol.
Conflict of Interest No conflict of interests was declared by the authors.
references
1. Chang MY, Pineles SL. Optic disk drusen in children. Surv Ophthalmol. 2016;61:745–58.
2. Delas B, Almudí L, Carreras A, Asaad M. Bilateral choroidal neovascularization associated with optic nerve head drusen treated by antivascular endotelial growth factor therapy. Clin Ophthalmol. 2012;6:225–30. 3. Flores-Reyes E, Hoskens K, Mansouri K. Optic nerve head drusen: imaging using optical coherence tomography angiography. J Glaucoma. 2017;26:845–9. 4. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol. 2002;47:515–32. 5. Duncan JE, Freedman SF, El-Dairi MA. The incidence of neovascular membranes and visual field defects from optic nerve head drusen in children. J AAPOS. 2016;20: 44–8. 6. Alkin Z, Ozkaya A, Yilmaz I, Yazici AT. A single injection of intravitreal ranibizumab in the treatment of choroidal neovascularization secondary to optic nerve head drusen in a child. BMJ Case Rep. 2014;2:2014. 7. Knape RM, Zavaleta EM, Clark CL 3rd, Khuddus N, Peden MC. Intravitreal.bevacizumab treatment of bilateral peripapillary choroidal neovascularization. from optic nerve head drusen. J AAPOS. 2011;15:87–90. 8. Baillif S, Nguyen E, Colleville-El Hayek A, Bétis F. Long term follow-up after a single intravitreal ranibizumab injection for choroidal neovascularisation secondary to optic nerve head drusen in a 5-year-old child. Graefes Arch Clin Exp Ophthalmol. 2013;251:1657–9.