Polypoidal choroidal vasculopathy in a patient with early-onset large colloid drusen





Abstract


Purpose


To report a case of a 46-year-old patient affected by polypoidal choroidal vasculopathy (PCV) in large colloid drusen (LCD) and to show how switching to intravitreal injection of aflibercept could be considered as a useful treatment of PCV not responsive to other anti-vascular endothelial growth factor (VEGF) injections.


Observations


A 46-year-old woman was referred to our department with diagnosis of early-onset retinal drusen. Multimodal imaging confirmed the diagnosis of LCD in both eyes, complicated by suggestive PVC in the left eye. Due to the absence of anatomical improvement after 6 intravitreal injections of ranibizumab, the patient was switched and treated by a single injection of aflibercept, showing a complete anatomical and functional recovery.


Conclusions and Importance


This case suggests progressive development of PCV as a possible late evolution of degenerating LCD. In case of exudative complication, intravitreal aflibercept injection could be considered as a useful treatment, especially in patients who are not responsive to others anti-VEGF injections.


1


Introduction


Drusen are extracellular deposits between the basal lamina of the retinal pigment epithelium (RPE) and the inner collagenous layer of Bruch’s membrane. Although drusen occur more frequently in the patient above 50 years, early onset drusen (EOD) are identified in young patients. Large colloid drusen (LCD) are a subgroup of EOD that are considered a benign condition. , LCD are large (200–300 μm), bilateral and yellowish lesion, located in the macula and peripheral retina. In the intermediate and late phases of indocyanine green angiography (ICGA), LCD core shows a hypofluorescent center surrounded by a hyperfluorescent halo. This halo is bordered with a peripheral thin hypofluorescent ring, harboring a ‘donut shape’. On structural spectral domain optical coherence tomography (SD-OCT), most LCD appear convex with medium and homogeneous internal reflectivity. SD-OCT suggests that the smallest LCD have no effect on the ellipsoid zone, and the larger LCD erode into the photoreceptors.


Here, we report a case of a 46-year-old patient affected by polypoidal choroidal vasculopathy (PCV) in LCD and showed how switching to intravitreal injection of aflibercept could be considered as a useful treatment of polypoidal lesion not responsive to other anti-VEGF injections.


2


Case report


A 46-year old woman was referred to our department with diagnosis of LCD that was done 8 years before based on multimodal imaging examinations ( Figs. 1–3 ). The last fluorescein angiography, performed in 2012, showed hyperfluorescent spots compatible with large colloid drusen ( Fig. 2 ). One year before, the patient reported central vision loss because of PCV in the left eyes (LE) ( Fig. 1 A). The patient was treated with six intravitreal 0.5 mg/0.05 ml ranibizumab injections (Lucentis, Novartis, Basel, Switzerland) in the left eye (the last performed one month before the examination in our department).




Fig. 1


Horizontal structural optical coherence tomography (OCT) scans passing through the polypoidal choroidal vasculopathy (PCV) of the left eye at baseline and at all follow-up examinations. A) Combined infrared reflectance imaging and structural OCT at the baseline with a pigment epithelium detachment nasally to the fovea matching PCV associated with subretinal hyporeflective exudation. B) After 6 intravitreal injections of ranibizumab, structural OCT showed the persistence of subretinal fluid, involving the foveal area. C) One month after the intravitreal injection of aflibercept, structural OCT showed a great improvement of subretinal fluid, that persists only nasally to the PED. D) Two months after the intravitreal injection of aflibercept, structural OCT showed the complete resolution of the subretinal fluid. RPE: retinal pigment epithelium.



Fig. 2


Fluorescein angiography (FA) of the left eye of the patient at the time of large colloid drusen diagnosis. Early phases (A) and late phases of FA showing the presence of hyperfluorescent spots compatible with large colloid drusen.



Fig. 3


Multimodal imaging evaluation of the left eye of the patient. A) Ultra-wide field (UWF) pseudocolor examination showing the presence of large colloid drusen involving the posterior pole (magnification) and the presence of a paving-stone degeneration in the temporal periphery of the retina (magnification). B) UWF fundus autofluorescence showing the presence of hyperautofluorescence areas matching to the large drusen and an inhomogeneous hypoautofluorescence at the posterior pole. C) Combined infrared reflectance imaging and structural optical coherence tomography (OCT) passing through the fovea showing the presence of large colloid drusen (magnification, asterisks). D) OCT-angiography showing a type 1 branching neovascular network (white triangles of en-face image), associated with the polypoidal lesion (white triangle of the B-scan image).


At the presentation, best-corrected visual acuity (BCVA) was 20/40 in the right eye (RE) (amblyopic eye) and 20/25 in the left eye (LE). Intraocular pressure was 16 mmHg in both eyes. Anterior segment examination revealed the result of photorefractive keratectomy in the RE and of radial keratotomy treatment in the LE. Fundus examination revealed the presence of large round drusen in correspondence of the vascular arcades and the mid-periphery in both eyes, with paving-stone degeneration in both eyes ( Figs. 3 and 4 ). Fundus autofluorescence showed hyperautofluorescence from the large drusen and an inhomogeneous hypoautofluorescence at the posterior pole in both eyes ( Figs. 3B and 4 B). Fundus photography and structural SD-OCT (Spectralis HRA + SD-OCT; Heidelberg Engineering, Heidelberg, Germany) revealed a myopic staphyloma in the RE ( Fig. 4 A) and the presence of large colloid and cuticular drusen with various inner reflectivity in both eyes ( Figs. 3C and 4 C). In the LE, structural SD-OCT showed the presence of pigment epithelial detachment (PED) associated with the persistence of subretinal fluid ( Fig. 1 B), despite the last intravitreal injection of ranibizumab was performed one month before. Swept Source OCT angiography (OCT-A; PlexElite 9000 SS-OCT, Carl Zeiss Meditec, Inc., Dublin, USA) revealed a type 1 branching neovascular network associated with polypoidal lesion ( Fig. 3 D). So, based on the multimodal imaging evaluation (including structural OCT and OCT-A), but due to the lack of ICGA, a suggestive diagnosis of PCV was performed.




Fig. 4


Multimodal imaging evaluation of the right eye of the patient. A) Ultra-wide field (UWF) pseudocolor examination showing the presence of myopic staphyloma and some colloid drusen involving the posterior pole (magnification) and the presence of a paving-stone degeneration in the temporal periphery of the retina. B) UWF fundus autofluorescence showing the presence of inhomogeneous hypoautofluorescence and hyperautofluorescence areas matching to the drusen at the posterior pole (magnification on the right) and atrophic areas matching to paving-stone degeneration in the temporal periphery of the retina (magnification on the left). C) Combined infrared reflectance imaging and structural optical coherence tomography (OCT) showing the presence of colloid drusen (magnification).

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Jul 10, 2021 | Posted by in OPHTHALMOLOGY | Comments Off on Polypoidal choroidal vasculopathy in a patient with early-onset large colloid drusen

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