International Journal of Retina (IJRETINA) 2025.
Volume 8.
Number 1.
P-ISSN.
E-ISSN.
POLYPOIDAL CHOROIDAL VASCULOPATHY MANIFESTATION
IN EXTRA-AXIAL CAVERNOUS TUMOR:
A RARE FINDINGS IN CHRONIC PAPILLEDEMA
Annisa Kinanti Asti1.
Lukisiari Agustini2.
Muhammad Firmansjah2 Resident of ophthalmology.
Faculty of medicine Universitas Airlangga.
Surabaya.
Indonesia Department of ophthalmology.
Dr.
Soetomo General Academic Hospital.
Surabaya.
Indonesia Abstract Introduction: Age-related macular degeneration (AMD) is a leading cause of blindness worldwide.
Polypoidal choroidal vasculopathy (PCV), a subtype of neovascular AMD, is characterized by an abnormal branching network of vessels with aneurysmal dilations .
Choroidal neovascularization can also result from chronic disc edema.
This case presents a rare occurrence of PCV in a patient with an extra-axial cavernous tumor and explores the best management Case Report: A 43-year-old man presented with an 8-month history of blurry vision in his right eye, double vision on left gaze, and left eye protrusion, accompanied by headaches and occasional Visual acuity was 2/60 in the right eye and 5/10 in the left improving with pinhole become 5/6.
RAPD was found in left eye.
Funduscopy revealed peripapillary atrophy in both eyes, with exudates in the right macula.
OCT showed dome-shaped polyps in both eyes, larger in the right.
MRI revealed a left sphenoid meningioma compressing the orbital cavity.
The patient was diagnosed with both eyes PCV and compressive optic neuropathy, left eye multiple cranial nerve palsy and dyslipidemia.
Intravitreal anti-VEGF injection was planned.
Discussion PCV is a subtype of AMD characterized by recurrent serosanguineous detachments.
Chronic papilledema, possibly due to intracranial tumors, may lead to visual loss from retinal nerve fiber damage or neovascularization.
Chronic posterior globe flattening and choroidal vessel abnormalities likely contributed to PCV development.
Conclusion: PCV may result from chronic papilledema due to intracranial tumors.
Anti-VEGF therapy offers a viable treatment option, balancing polyp regression and visual acuity stabilization.
Keywords: Polypoidal Choroidal Vasculopathy.
Choroidal neovascularization.
Papilledema.
Meningioma Cite This Article: ASTI.
Annisa Kinanti.
AGUSTINI.
Lukisiari.
FIRMANSJAH.
Muhammad.
POLYPOIDAL CHOROIDAL
VASCULOPATHY MANIFESTATION IN EXTRA-AXIAL CAVERNOUS TUMOR: A RARE FINDINGS IN CHRONIC
PAPILLEDEMA.
International Journal of Retina, [S.
], v.
8, n.
1, p.
57, mar.
ISSN 2614-8536.
Available at:
<https://w.
com/index.
php/ijretina/article/view/306>.
Date doi: https://doi.
org/10.
35479/ijretina.
Published by: INAVRS https://w.
org/ | International Journal of Retina https://ijretina.
accompanied by double vision when looking to the INTRODUCTION Worldwide.
Over the same period, the left eye had become million people will be blind protruded, occur with headaches, nausea, and due to age-related macular occasional vomiting.
The patient was referred by the neurosurgery department due to the detection of a No history of systemic disease was found, but An irregular had undergone strabismus surgery a year earlier.
The branching network of blood vessels with aneurysmal patient family history included cervical cancer in the dilations called polyps characterises polypoidal patientAos mother.
Correspondence to:
Annisa Kinanti Asti.
Universitas Airlangga.
Surabaya.
Indonesia, drannisakinantiasti@gmail.
instances predicted in Asia alone.
(AMD)
(PCV),
neovascular AMD.
PCV is also known as type 1 In extreme cases, persistent disc oedema might lead to choroidal neovascularisation.
Diagnosing papilledema can be challenging, particularly when it is accompanied by visual loss.
The narrowing of visual fields (VF) caused by the gradual atrophy of retinal nerve fibres is the usual cause of this visual impairment.
Occasionally, it can the latter is more common in longterm instances.
On examination, the patient visual acuity was 2/60 in the right eye and 5/10 in the left, improving to 5/6.
5 with pinhole correction.
Intraocular pressure (IOP) was normal in both eyes.
Color vision was impaired in the right eye .
but normal in the left .
Ocular motility was restricted in the left eye, with -3 limitation in the superotemporal, temporal, and inferotemporal directions, though without pain Confrontation normal, and the Hertel test measured 20-121-22.
The Hirschberg test showed orthophoria.
A relative afferent pupillary deficit (RAPD) measuring 4 mm was detected in the left eye, but the anterior portion The goal of this case study is to discuss the optimal course of treatment for a patient who presented with the unusual symptoms of polypoidal choroidal vasculopathy in addition to their extraaxial cavernous tumour.
of the right eye seemed to be functioning normally.
Peripapillary perimacula exudates were seen during funduscopy in the right eye, whereas the left eye also showed Ocular Coherence Tomography (OCT) scans of the macula in both eyes CASE REPORT A 43-year-old man presented to the outpatient clinic with complaints of blurry vision in his right eye, ongoing for the past 8 months.
The complaint also showed a dome-shaped or thumb-like elevation polyp with hyperreflective rings and a hyporeflective and hyperreflective lumen within.
Figure 1.
(A) Right eye funduscopy show peripapillary atrophy around the optic nerve head and exudate in inferotemporal perimacula.
(B) The peripapillary atrophy findings in left eye.
*Pictures taken with patientAos consent.
Courtesy: Poli Mata RSUD Dr.
Soetomo.
Published by: INAVRS https://w.
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with the polyp being more prominent and elevated The Humphrey Visual Field (HVF) test for in the right eye.
Between the Bruch's membrane and both eyes was deemed unreliable due to excessive another hyperreflective tissue is the double-layer fixation losses and a high rate of negative errors Figure 2.
(A) ONH and RFNL OCT in both eyes are within normal limit.
(B) Macula Thickness OCT represent edema in some quadrants.
(C) Thumb-like elevation polyp is shown in HD-21 OCT, and shown in the left eye but smaller in size (D) *Pictures taken with patientAos consent.
Courtesy: Poli Mata RSUD Dr.
Soetomo .
sign, which is depicted in Figure 2, which is a pigment (Figure .
Laboratory tests revealed elevated lipid levels, and the patient's HbA1C was in the There noticeable issues with the optic nerve head (ONH) or All IgG retinal nerve fibre layer (RNFL) complex in either Toxoplasma.
Rubella, and Cytomegalovirus (CMV), eye's macula.
The layers are staying the same returned positive.
A contrast-enhanced brain MRI Retinal pigment epithelium and internal showed a left sphenoid meningioma causing slight limiting membrane swelling is observed in the nasal, compression of the orbital cavity, along with left lateral, and superior quadrants of the right eye.
In the ethmoidal sinusitis, bilateral mastoiditis .
ore left eye, the upper quadrant is thicker while the lower prominent on the lef.
, nasal septum deviation, and and nasal quadrants are thinner.
chronic rhinitis.
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Figure 3.
Humphrey Visual Field examination in right eye (A) and left eye (B) show unreliable result.
Courtesy: Poli Mata RSUD Dr.
Soetomo.
The patient was found to have symptoms such as compressive optic neuropathy in both eyes, dyslipidaemia, a suspected left-sided extra-axial cavernous meningioma, and left-eye cranial nerve i and VI palsy with pupillary involvement, according to the results of the examination.
The treatment plan includes an intravitreal anti-VEGF injection for the left eye, while conservative management is recommended for the right eye.
Routine follow-up visits are advised prior to tumor excision via craniotomy to monitor for any unexpected ocular Unfortunately, this patient had return to the patient place in Gorontalo and no further follow neovascularisation (CNV).
A vascularised serous or DISCUSSION retina's deep capillary plexus.
these growths, fibrovascular pigment epithelial detachment (PED) surface can consequence of bleeding and leakage caused by this Type 2 macular hole (MNV), also called classic CNV, is marked by the formation of new blood vessels that link the neurosensory retina to the retinal pigment epithelium (RPE).
In such a scenario, an examination can show a gray-green or lacy lesion.
A minimally classic CNV or mixed type 1 and type 2 MNV is a form of neovascularisation that extends beneath the RPE and the neurosensory retina.
The genesis of type 3 MNV is aberrant capillaries in the One kind of age-related macular degeneration formerly called retinal angiomatous proliferations, is polypoidal choroidal vasculopathy, or PCV.
eventually reach the RPE.
Before this subtype fully Recurrent serosanguineous detachments of the develops, intraretinal pigment migration may occur.
retinal pigment epithelium (RPE) characterise this On examination, type 3 MNV often appears as a variety of type 1 macular neovascularisation (MNV), small red area with associated retinal exudate or which is also known as posterior uveal haemorrhage subretinal fluid accumulation.
A unique "string-of-pearls" pattern is PCV is identified in women and men of all races.
formed when feeder arteries attach to the RPE layer In Asian population, 2050% of cases of nAMD are of the fibrovascular pigment epithelial detachment PCV type.
In white people less than 5% of cases of (PED) and create a network of polypoidal lesions.
MNV are PCV type.
6 One study found that between This pattern is characterised by PCV.
20% and 60% of ICGA cases had polypoidal When new blood vessels sprout through Bruch's choroidal vasculopathy (PCV).
In contrast, the membrane into the sub-retinal pigment epithelium percentage of PCV is much lower, ranging from 8% (RPE) to 13% in cases when ICGA was performed, 8-9 There is a hereditary component to PCV risk factors, (MNV) Published by: INAVRS https://w.
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Table 1.
Polypoidal Choroidal Vasculopathy Diagnosis Criteria.
AuPolypoidal Choroidal Vasculopathy Diagnostic Criteria Japanese Study Group Definite PCV:
Guidelines A Elevated orange-red lesions on fundus examination, and/or Polypoidal lesions on ICGA A Probable PCV:
Only abnormal BVN seen on ICGA or recurrent hemorrhagic or serous A RPE detachments, or both, without features of definite PCV EVEREST criteria .
ased on Focal hyperfluorescent lesions appearing before 6 minutes on ICGA, plus at least confocal scanning laser 1 of the following:
A BVN on ICGA A Pulsatility on dynamic ICGA A Nodular appearance when ICGA viewed stereoscopically A Hypofluorescent halo on ICGA A Orange subretinal nodule on color photograph A Associated massive submacular hemorrhage BVN: branching vascular network.
ICGA: indocyanine green angi- ography.
PCV: polypoidal choroidal RPE: retinal pigment epitheliumAy.
and both PCV and conventional AMD share systemic sub-RPE risk factors such cigarette smoking, , obesity, and characterises hemorrhagic PCV can happen with or 9,11 without the presence of exudative alterations.
15 In inflammatory indicators in the blood.
10,12 Some this case, the presentation is likely an exudative PCV research shows a higher prevalence of dyslipidemia C-reactive, in patients with PCV compared to other subtypes of Polypoidal choroidal vasculopathy (PCV) can be age-related macular degeneration (AMD), and diagnosed by funduscopy with reddish-orange dyslipidemia has been linked to increased severity or subretinal nodules, serous retinal detachment, many recurrence of PCV due to its role in promoting hard exudates, or severe haemorrhage.
On optical vascular instability.
coherence tomography (OCT), the retinal pigment In the pathophysiology of PCV, there is localized epithelium (RPE) appears as dome-shaped or choroidal thickening at the site of the disease, thumb-like elevations, some of which may have caused by abnormally dilated veins in the Haller internal polyps and others do not.
The double-layer layer, known as pachyvesselsDue to the presence of sign, consisting of hyperreflective tissue separating these pachyvessels, the Sattler and choriocapillaris Bruch's membrane from hyperreflective RPE, is layers that are located above the Bruch's membrane- RPE complex become thinner and eventually bleed.
pachychoroid characteristics, which are enlarged The subfoveal area and overall choroidal thickness is blood vessels, may be present in Haller's layer.
thicker in PCV patients.
14 Different forms of polypoidal choroidal vasculopathy (PCV) manifest in different ways.
The lack of subretinal or intraretinal fluid as well as haemorrhage is a hallmark of quiescent PCV.
Lipid exudation, neurosensory retinal thickening, pigment epithelial detachment (PED), subretinal fluid, or a mix of these traits is found in exudative PCV, but haemorrhage is not.
The Finally.
The presence of polypoidal dilatations of the choroidal circulation, along with or without feeder and draining veins, can be detected by Indocyanine green angiography (ICGA) as hyperfluorescent Occult neovascularisation (NV) is the most common way that fluorescein angiography (FA) detects PCV, however a conventional NV pattern can be seen in few cases.
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A worse visual prognosis may be indicated in cases Chronic papilledema may present with optic of PCV that present as classic CNV on FA.
The disc pallor, reduced swelling from axonal loss, gliosis inability of FA to detect polyps and other sub-RPE .
carring of retinal fiber.
, optociliary shunts, and structures limits its use in PCV diagnosis.
In PCV, refractile bodies.
In papilledema, the forward shift of OCT-A gives a two-dimensional picture of aberrant the optic nerve head and surrounding peripapillary vasculature, just like ICGA.
In most cases.
OCT-A can tissues can cause compression or ischemia.
Chronic detect the branching vascular network (BVN), flattening of the posterior globe, along with however it is far less effective than ICGA at abnormalities in the choroidal vessels, may have identifying polyps.
ICGA.
FA, and OCT-A are not contributed to the onset of PCV in this case.
3,18,19 The performed in our case, but the diagnosis made patient, with left sphenoid meningioma and early based on the diagnosis criteria (Table .
When compared to other forms of macular neovascularisation (MNV), polypoidal choroidal vasculopathy (PCV) often responds less favourably to anti-VEFG treatment.
After a year, the visual anti-VEFG combination therapy with photodynamic therapy (PDT) are outstanding, on par with those from normal neovascular AMD, according to the EVERESTII and PLANET trials.
Therefore, for patients experiencing symptoms of PCV, both approaches are considered effective initial therapies.
Signs of increased intracranial pressure (ICP) include papilledema, which manifests as fuzzy and raised optic disc borders.
ICP can be caused by tumours within the brain, infections, haemorrhages, or blockage of the cranial ventricular drainage When papilledema is associated with visual lossAiusually as a result of progressive damage to the retinal nerve fibres that narrows the visual fieldsAior, less frequently, with macular exudates, neovascularizationAicommon in long-term casesAi the diagnosis becomes much more difficult.
16,17 It is believed that pressure-induced distortion at the border of Bruch's membrane near the optic disc causes choroidal neovascularisation (CNV) to occur in chronic papilledema.
This anatomic weakening, combined with hypoxia from axonal swelling and subsequent vascular perfusion changes, promotes The presence of an extra-axial cavernous tumor, specifically a left sphenoid meningioma, likely contributed to the development of PCV.
The tumor-induced compression of the orbital cavity and optic nerve led to chronic posterior globe flattening and increased intracranial pressure, which could compromise the choroidal circulation and structural integrity of the posterior segment.
This mechanical stress, combined with chronic ischemia and inflammation, may have triggered vascular remodeling and the formation of the polypoidal lesions characteristic of PCV.
The cooccurrence of dyslipidemia in this patient further exacerbates vascular instability, increasing the risk of neovascularization and disease progression.
The observed clinical featuresAidome-shaped polyps in both eyes and compressive optic neuropathyAi conditions .
, local tumor effects, and choroidal vascular pathology in PCV development.
CONCLUSION
Polypoidal choroidal vasculopathy (PCV) is a rare condition that describes a choroidal artery BVN and polypoidal dilations, both of which impact the choroidal vasculature.
Possible contributors to the development of PCV in this case include intrinsic posterior globe flattening.
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It is possible that an intracranial tumour produced Cheung CMG.
Lai TYY.
Ruamviboonsuk P, the chronic papilledema.
Either monotherapy or Chen SJ.
Chen Y.
Freund KB.
Gomi F.
Koh AH, combo therapy with anti-VEFG can stabilise visual Lee WK.
Wong TY.
Polypoidal Choroidal acuity and balance polyp regression.
Vasculopathy:
Definition.
Pathogenesis.
Diagnosis.
Management.
REFERENCES