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Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 190-192

Papilledema associated unilateral juxtafoveal choroidal neovascularization in a patient of intracranial chondrosarcoma

Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission14-Apr-2021
Date of Acceptance16-Aug-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Gitanjli Sood
Department of Ophthalmology, All India Institute of Medical Sciences (AIIMS), Rishikesh - 249 203, Uttarakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_860_21

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Skull base chondrosarcomas are rare intracranial neoplasms. Although peripapillary choroidal neovascularization (CNV) has infrequently been noted in papilledema secondary to other pathologies, juxtafoveal CNV following intracranial chondrosarcomas have never been reported earlier. We report a case of a 50-year-old lady with operated intracranial chondrosarcoma who presented with progressive diminution of vision in the right eye. Detailed ophthalmic evaluation and ancillary investigations confirmed the diagnosis of papilledema associated juxtafoveal CNV in the right eye, and she received two intravitreal bevacizumab injections with guarded outcome. We have also described the probable differentials of the current case and highlighted the need for a high index of suspicion among neurologists as well as ophthalmologists for this rare but vision-threatening complication.

Keywords: Bevacizumab, chondrosarcoma, choroidal neovascularization, juxtafoveal CNV, papilledema

How to cite this article:
Samanta R, Sood G, Kumawat D, Saraswat N, Agrawal A, Garai A. Papilledema associated unilateral juxtafoveal choroidal neovascularization in a patient of intracranial chondrosarcoma. Indian J Ophthalmol Case Rep 2022;2:190-2

How to cite this URL:
Samanta R, Sood G, Kumawat D, Saraswat N, Agrawal A, Garai A. Papilledema associated unilateral juxtafoveal choroidal neovascularization in a patient of intracranial chondrosarcoma. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:190-2. Available from: https://www.ijoreports.in/text.asp?2022/2/1/190/334984

Intracranial chondrosarcomas are extremely rare tumors, seen in approximately one in 1000 intracranial neoplasms.[1] The skull base is involved only in 2% of all chondrosarcomas.[2] The majority of the skull base chondrosarcomas are low-grade and well-differentiated. It is most commonly seen in the age group of 40–70 years without any significant gender predilection. Initial presentation may be asymptomatic and incidentally diagnosed or may have varied features, including headache, diplopia, tinnitus, auditory dysfunctions, cranial nerve deficits, and others, depending upon tumor location and surrounding compression effect secondary to the tumor.[1],[3] In the current report, we have presented an unusual ocular complication in a middle-aged female diagnosed and treated for intracranial chondrosarcoma.

  Case Report Top

A 50-year-old female presented at the ophthalmology clinic with progressive diminution of vision in the right eye (RE) for the last six months, aggravated over the past four weeks. Ocular examination revealed best-corrected visual acuity of 20/400 in RE and 20/20 in the left eye (LE). Intraocular pressures and anterior segment evaluation were unremarkable in both eyes. Fundus [Figure 1]a and [Figure 1]b showed bilateral optic disc edema (ODE; grade-3 in RE and grade-2 in LE according to modified Frisen scale) with tortuous peripapillary vessels. Additionally, RE [Figure 1]a. revealed a yellowish-gray subretinal lesion involving the papillomacular bundle region with surrounding exudation and retinal surface wrinkling. Past medical history revealed a diagnosis of occipital chondrosarcoma two years back. Brain-MRI with contrast at that time showed a destructive lytic lesion (3.1 × 2.8 × 3.9 cm) involving the right occipital condylar region with involvement of the jugular fossa causing encasement of the right internal jugular vein. It was extending from the right carotid space anteriorly to right cerebello-medullary cistern posteriorly and partially encased the origin of the 9th, 10th, and 11th nerve. She underwent surgical resection with far-lateral skull base approach for the same two years ago. A review of old records showed bilateral normal visual acuity and fundus except papilledema at that time. No history of diabetes or hypertension was elicited. Her recent systemic evaluation including neurological examination was noncontributory. RE fundus fluorescein angiography [Figure 1]c, [Figure 1]d, [Figure 1]e revealed a juxtafoveal classic choroidal neovascularization (CNV). Optical coherence tomography (OCT) of macula showed a subretinal hyperreflective material with intra and subretinal fluid in RE [Figure 1]f and normal foveal contour in LE [Figure 1]g. Peripapillary retinal nerve fiber layer (RNFL) thickening (RE > LE) was noted in both eyes on OCT [Figure 1]h and [Figure 1]i. However, there was no peripapillary choroidal thickening in either eye. She refused further indocyanine green angiography at this moment. Repeat MRI of the brain with craniovertebral junction [Figure 2]a, [Figure 2]b, [Figure 2]c revealed bone destructing enhancing mass lesion (2.1 × 1.4 × 1.7 cm) in the region of right petro-clival fissure extending to the posterior part of the petrous bone and right occipital condyle. Whole-body PET scan revealed a focal metabolically active lesion in the right occipital condylar region with calcifications without any evidence of other metabolically active lesions elsewhere in the body. Although the repeat neuroimaging was suggestive of residual disease in the right skull base, neurosurgeons advised her conservative management with periodic evaluation. Working diagnosis of RE classic juxtafoveal CNV secondary to chronic papilledema from intracranial chondrosarcoma was made. Subsequently, she received two intravitreal bevacizumab injections (1.25 mg/0.05 ml) at monthly intervals. Follow-up at 6-months [Figure 3]a and [Figure 3]b revealed a mild decrease in ODE bilaterally (grade-2 in RE and grade-1 in LE) and scarred CNV with resolution of hard exudates in RE. OCT macula [Figure 3]c and [Figure 3]d showed the absence of any intra/subretinal fluid in RE and normal contour in LE. OCT RNFL [Figure 3]e and [Figure 3]f showed a mild decrease in peripapillary RNFL thickness in both eyes. She was kept under close follow-up with a stable BCVA of 20/200 in RE and 20/20 in LE.
Figure 1: (a) RE fundus at presentation showing disc edema, juxtafoveal CNV (white arrow) associated with macular and peripapillary exudation (yellow arrows). (b) LE fundus showing mild disc edema. (c-e) RE FFA revealing juxtafoveal lacy hyperfluorescence in the early phase (C, white arrow) with intensification in the A–V phase (d) and dye leakage in the late phase (e) suggestive of a classic CNV. OCT of RE (f) showing hyperreflective subretinal membrane (white arrow) with mild intra and subretinal fluid and LE (g) showing normal contour. OCT RNFL (h and i) showing increased peripapillary RNFL thickness in both eyes

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Figure 2: Post-operative contrast-enhanced MRI brain with cranio-vertebral junction showing (a) enhancing soft tissue mass in right petroclival region (red arrow) and post-operative changes in right occipital region in T1-weighted images; (b) hyperintense lesion in right petroclival region (white arrow) in T2-weighted images and (c) widening of the hypoglossal canal (red arrow). The right side of the brain is shown in the left side of the scan

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Figure 3: Fundus picture (a) and OCT macula (c) of RE at six months showing juxtafoveal organized scar (white arrow) and resolution of exudates without any intra or subretinal fluid. Fundus picture (b) and OCT macula (d) of LE showing normal foveal contour at six months. OCT RNFL (e and f) showed a mild decrease in peripapillary RNFL thickness in both eyes as compared to the presentation

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  Discussion Top

ODE-associated CNV is an uncommon occurrence. Although peripapillary-CNV (PCNV) has been described in the literature in ODE patients, especially with idiopathic intracranial hypertension, juxtafoveal CNV has not been previously reported in papilledema secondary to occipital chondrosarcoma.[4] We hypothesize that the etiopathogenesis of CNV in this case may be similar to PCNV. Transmitted raised intracranial tension at the optic nerve head level might have led to initial disruption of the peripapillary Bruch's membrane followed by subsequent ingrowth of neo-vessels from underlying choroidal vasculature.[5],[6] Further, axoplasmic stasis induced hypoxia aggravated the CNV.[5] The CNV might have started in the peripapillary area and then progressed temporally toward the juxtafoveal region. An OCT-angiography at the early stage could have documented an initial peripapillary hyperintense vascular network. The other probable causes of juxtafoveal CNV include myopia, exudative age-related macular degeneration, polypoidal choroidal vasculopathy, trauma, and past choroiditis lesions. However, the absence of myopia, drusens, pigmentary changes, subretinal hemorrhage, inflammatory chorioretinitis in the affected or fellow eye ruled out the above causes of CNV. Although idiopathic CNV and peripapillary pachychoroid syndrome are other probable differentials, papilledema was considered as the likely primary etiology as there was relatively more ODE in the affected RE.

Visual abnormalities in ODE can range from asymptomatic or transient blurring in early stages to marked diminution in late and chronic stages due to optic atrophy or CNV. Although asymptomatic extrafoveal-CNV may be observed only with medical or surgical lowering of intracranial pressure, juxtafoveal active CNV needs treatment. Ozgonul et al.[7] have shown successful resolution of IIH-associated PCNV in a subset of patients with only medical management of IIH and suggested intravitreal anti-vascular growth factor (anti-VEGF) injections only in vision-threatening and/or persistent cases. Resolution of CNV and improvement in vision with anti-VEGF injections in PCNV patients have been demonstrated by Figueroa et al.[8] as well. Despite two anti-VEGF injections, visual improvement was guarded in this patient due to delayed presentation and scar formation. Neurologists are the primary caregivers in most of such patients and hence they must exercise a high index of suspicion and promptly seek opinion of ophthalmologists for detailed evaluation.

  Conclusion Top

Juxtafoveal CNV may be associated with intracranial malignancy related papilledema. Thus neurologists must be cognizant about this less common but potentially sight threatening complication of papilledema. Early diagnosis and prompt treatment with anti-VEGF agent may lead to favourable outcome in such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Awad M, Gogos AJ, Kaye AH. Skull base chondrosarcoma. J Clin Neurosci 2016;24:1-5.  Back to cited text no. 1
Rosenberg AE, Nielsen GP, Keel SB, Renard LG, Fitzek MM, Munzenrider JE, et al. Chondrosarcoma of the base of the skull: A clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 1999;23:1370-8.  Back to cited text no. 2
Bloch O, Parsa AT. Skull base chondrosarcoma: Evidence-based treatment paradigms. Neurosurg Clin N Am 2013;24:89-96.  Back to cited text no. 3
Wendel L, Lee AG, Boldt HC, Kardon RH, Wall M. Subretinal neovascular membrane in idiopathic intracranial hypertension. Am J Ophthalmol 2006;141:573-4.  Back to cited text no. 4
Morse PH, Leveille AS, Antel JP, Burch JV. Bilateral juxtapapillary subretinal neovascularization associated with pseudotumor cerebri. Am J Ophthalmol 1981;91:312-7.  Back to cited text no. 5
Tewari A, Shah GK, Dhalla MS, Shepherd JB. Combination photodynamic therapy and juxtascleral triamcinolone acetonide for the treatment of a peripapillary choroidal neovascular membrane associated with papilloedema. Br J Ophthalmol 2006;90:1323-4.  Back to cited text no. 6
Ozgonul C, Moinuddin O, Munie M, Lee MS, Bhatti MT, Landau K, et al. Management of peripapillary choroidal neovascular membrane in patients with idiopathic intracranial hypertension. J Neuroophthalmol 2019;39:451-7.  Back to cited text no. 7
Figueroa MS, Noval S, Contreras I. Treatment of peripapillary choroidal neovascular membranes with intravitreal bevacizumab. Br J Ophthalmol 2008;92:1244-7.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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