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One Minute Ophthalmology
Bilateral pale posterior pole sans pain:
A “hard” sell
Case
A 30‑year‑old Asian man presented with metamorphopsia in left eye since
1 month. He was diagnosed with peripapillary serpiginous choroiditis elsewhere
and advised intravenous methylprednisolone. His past medical history was
unremarkable. On examination, visual acuity was 20/15 in both eyes. Anterior
chamber was quiet and did not show any reaction in either eye.
What Is Your Next Step?
A. Start intravenous methylprednisolone
B. High‑resolution chest computed tomography (CT) to rule out tuberculosis
C. Mantoux test
D. Dilated fundus evaluation.
Findings
Fundus evaluation showed peripapillary reddish‑white lesion bilaterally, with
well‑defined irregular geographic borders forming pseudopodia and sparing
the fovea  [Fig.  1a]. Ultrasound scan showed high‑reflective plaque echoes in
peripapillary area of both eyes suggestive of calcification  [Fig.  1c]. CT orbit
showed hyperdense lesion suggestive of calcification [Fig. 1e]. Optical coherence
tomography (OCT) showed choroidal lesion causing shadowing with subretinal
fluid  (SRF)  [Fig.  1d]. Fundus fluorescein angiography  (FFA) of both eyes
showed early intense hyperfluorescence with staining [Fig. 1b]. Indocyanine
green angiography did not show any features of active choroiditis or choroidal
neovascular membrane (CNVM). In the absence of CNVM and the tumor edge
being very close to fovea, it was decided to observe the condition rather than
consider anti‑  vascular endothelial growth factor  (VEGF) or photodynamic
therapy. In such eyes with SRF, anti‑VEGF is the preferred treatment in the
presence of CNV, while observation is the norm without CNV. At 1 and 3 months
follow‑up, both fundi were stable without CNVM.
Diagnosis
Bilateral choroidal osteoma
Correct Answer: D.
a
d There are no conflicts of interest.
b 3. Znaor  L, Medic  A, Karaman  K. Serpiginous‑like choroiditis as sign of intraocular
c e
Figure 1: Fundus showed peripapillary reddish-white lesion bilaterally,
with well-defined irregular geographic borders forming pseudopodia and
sparing the fovea (a). Fundus fluorescien angiography showed early intense
hyperfluorescence with staining (b). Indocyanine green angiography did not show
any features of active choroiditis or CNVM. Ultrasound showed high-reflective
plaque echoes suggestive of calcification (c). Optical coherence tomography
showed choroidal lesion causing shadowing, with subretinal fluid (d). CT orbit
showed hyperdense lesion suggestive of calcification (e)
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DOI:
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© 2019 Indian Journal of Ophthalmology | Published by Wolters Kluwer - Medknow
Fairooz P. Manjandavida, Bangalore, India
Carol L. Shields, Philadelphia, USA
Discussion
Choroidal osteoma is a benign tumor of choroid composed of mature bone.
Usually choroidal osteoma is found in young, healthy women in the second
or third decade of life and unilateral in 75% of cases.[1] Mostly patients present
with symptoms of blurring and metamorphopsia. The tumor is usually
juxtapapillary or peripapillary and varies from deep pale yellow to orange
red (thinned out retina) color with well‑defined scalloped or distinct geographic
margins with blunt pseudopod‑like projections and branching “spider” vessels
on tumor surface, and may show SRF or hemorrhage which usually signifies
CNVM.[2] The lesion may decalcify over time and occurs in nearly 50% of eyes
by 10 years. Management options include laser photocoagulation, transpupillary
thermotherapy, anti‑VEGFs, photodynamic therapy, or combination of these
therapies. Asymptomatic or stable osteoma can be observed. Indications
for treatment of such eyes include the presence of active CNVM or calcified
extrafoveal tumor. In such eyes with SRF, anti‑VEGF is the preferred treatment
in the presence of  CNVM, while observation is the norm without CNV.
Serpiginous choroiditis (geographic helicoid peripapillary choroidopathy) is
bilateral, chronic, progressive, and recurrent inflammatory disease affecting the
retinal pigment epithelium and inner half of choroid. It starts in juxtapapillary or
peripapillary region and progresses centrifugally from the disc to involve the macula
area.[3] As the lesions resolve, retinal pigment epithelium and choroidal degeneration
begin, leading to fibrous scarring and pigment hyper or hypoplasia. Patients present
with symptoms such as central or paracentral scotomas, metamorphopsia, visual
field loss, and blurred vision. CNVM may develop over time.
In our case, the clinical appearance mimicked peripapillary serpiginous
choroiditis. However, investigations such as ultrasound and CT helped to
differentiate between these two conditions. The other differential diagnoses
to consider are juxtapapillary  (postuveitic) CNVM, and organized subretinal
hemorrhage (polypoidal choroidal vasculopathy).
Patients with suspected bilateral peripapillary serpiginous choroiditis should
have a dilated fundus examination and ancillary investigations such as FFA,
OCT, and ultrasonography.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms.
In the form, the patient’s parents have given their consent for the patient’s image
and other clinical information to be reported in the journal. The patient’s parents
understand that the patient’s name and initials will not be published and due
efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
References
1. Kadrmas EF, Weiter JJ. Choroidal osteoma. Int Ophthalmol Clin 1997;37:171‑82.
2. Shields CL, Shields JA, Augsburg JJ. Choroidal osteoma. Surv Ophthalmol 1988;33:17‑27.
tuberculosis. Med Sci Monit 2011;17:CS88‑90.
Ramya Appanraj, Vinay S Kumar, Pukhraj Rishi,
Jyotirmay Biswas1
Shri Bhagwan Mahavir Vitreoretinal Services, 1Department of Uvea,
Sankara Nethralaya, 18, College Road, Chennai - 600 006,
Tamil Nadu, India
Correspondence to: Dr. Pukhraj Rishi,
Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya,
18 College Road, Chennai ‑ 600 006, Tamil Nadu, India.
E‑mail: docrishi@yahoo.co.in
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Cite this article as: Appanraj R, Kumar VS, Rishi P, Biswas J. Bilateral pale
posterior pole sans pain: A “hard” sell. Indian J Ophthalmol 2019;67:582.