American Journal of Ophthalmology Case Reports 16 (2019) 100548

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American Journal of Ophthalmology Case Reports

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Case report

Presumed atypical peripapillary Vogt-Koyanagi-Harada disease T

a,∗ a a
Julio Zaki Abucham-Neto , Andressa Passos Masson , Priscila Alves Nascimento ,
Aline Alves Barbosa Ferraza, Eduardo Cunha de Souzab
a
Medical Faculty of the ABC - Faculdade de Medicina do ABC, Av. Príncipe de Gales, 821, Santo André, SP, Brazil
b
University of Sao Paulo Hospital of Clinics - Universidade de São Paulo, Av. Dr. Enéas Carvalho de Aguiar, 255, São Paulo, SP, Brazil

A R T I C LE I N FO A B S T R A C T

Keywords: Purpose: To describe a case of bilateral presumed atypical Harada disease with sequential, not simultaneous,
Serous retinal detachment involvement of the peripapillary retina (subretinal fluid) in a healthy patient with no systemic complaints.
Vogt-Koyanagi-Harada Observation: A 35-year-old healthy white man presented with sudden paracentral visual loss in the left eye. His
medical history was unremarkable. However, he reported a similar episode 20 months earlier in the right eye
that was associated with macular serous retinal detachment. The right eye showed evidence of reactive peri-
papillary atrophy and pigmentary alteration in the macula. Optical coherence tomography scans of the posterior
left eye segment revealed a diffuse thickened choroid, papillomacular subretinal exudate and discontinuity of the
ellipsoid layer with suggestion of vitreous cellularity. Autofluorescence imaging of the left eye showed peri-
papillary hyperautofluorescence. A fluorescein angiogram revealed progressive staining and pooling of the
peripapillary retina with corresponding retinal vasculitis. Indocyanine green angiography revealed multiple
hypocyanescent lesions with an area of hypercyanescence temporal to the disc. Rheumatologic evaluation and
laboratory tests were all negative. Chest tomography was normal. Considering the apparent absence of infectious
diseases, the patient was started on 60 mg/day prednisone. After 8 days, visual acuity improved to 20/250,
improving to 20/20 vision six months after a slow steroid wean.
Conclusion: We believe our case represented a variant of the Vogt-Koyanagi-Harada syndrome in an atypical
situation, because the patient fulfilled the presumed criteria. Furthermore, the findings of clinical and com-
plementary examinations led to this nosological entity to the exclusion of others.
Importance: The point of this case is to alert ophthalmologists to the existence of this atypical presentation of the
disease so that it should be included among the differential diagnoses of pathologies that present with these
findings.

1. Introduction had reported a similar episode 20 months earlier in the right eye that
was associated with macular serous retinal detachment (Fig. 1-a and 1-
Serous retinal detachment represents a diagnostic challenge because b). At that time, the exam of the left eye showed no abnormalities
of its etiological diversity. Inflammatory and infectious diseases should (Fig. 1-c), and the results of systemic and laboratory investigations,
be considered in order to direct appropriate clinical management.1 including a study of the cerebrospinal fluid, were normal. He was di-
We describe a case of bilateral presumed atypical Harada disease agnosed elsewhere with an optic disc pit maculopathy in the right eye.
with sequential, not simultaneous, involvement of the peripapillary Laser treatment associated with a pneumatic retinopexy was performed
retina with subretinal fluid, in a healthy patient with no systemic and this was believed to have resulted in complete regression of the
complaints. retinal detachment (Fig. 1-d). By the time we first examined the patient,
visual acuity was 20/25 in both eyes. Extrinsic ocular motility, pupil-
1.1. Case report lary reflexes and anterior segment biomicroscopy were normal in both
eyes. Right eye retinography revealed evidence of reactive peripapillary
A 35-year-old healthy white man presented with sudden paracentral atrophy and pigmentary alteration in the macula (Fig. 2-a). In the left
visual loss in the left eye. His medical history was unremarkable. He eye, retinography revealed peripapillary serous retinal detachment

∗
Corresponding author. Department of ophthalmology of Faculdade de Medicina do ABC, Avenida Príncipe de Gales, 821, Santo André, ZIP CODE: 09060-650, SP,
Brazil.
E-mail address: [email protected] (J.Z. Abucham-Neto).

https://doi.org/10.1016/j.ajoc.2019.100548
Received 28 June 2018; Received in revised form 20 August 2019; Accepted 22 August 2019
Available online 27 August 2019
2451-9936/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/BY-NC-ND/4.0/).
J.Z. Abucham-Neto, et al. American Journal of Ophthalmology Case Reports 16 (2019) 100548

Fig. 1. (a–b): Serous retinal detachment shown on OCT (yellow arrows), mainly
in the lower macula, and vitreous cellularity (red arrows) seen 20 months
earlier.(c): No abnormalities was seen on OCT of the left eye at the same time.
(d): Resolution of serous retinal detachment after treatment. Peripapillary
atrophy secondary to laser treatment (yellow circle).

(Fig. 2-b). Autofluorescence imaging in right eye revealed peripapillary

hypoautofluorescence and in the left eye there was peripapillary hy-
perautofluorescence. Fluorescein angiogram (FA) in the left eye re-
vealed progressive staining and pooling of the peripapillary retina with
corresponding retinal vasculitis (Fig. 2-c). Indocyanine green angio-
graphy (ICGA) revealed multiple hypocyanescent lesions with an area
of hypercyanescence temporal to the disc (Fig. 2-c). Spectral-domain
optical coherence tomography (OCT-SD) scans through the posterior
left eye segment revealed a diffuse thickened choroid, papillomacular
subretinal exudate and discontinuity of the ellipsoid layer with sug- Fig. 2. (a): Reactive peripapillary atrophy and pigmentary alteration in the
gestion of vitreous cellularity (Fig. 2-d). There was no evidence of pits macula of right eye. (b): Changes observed in the left eye at the onset.
in either eye. Laboratory tests were required at that time. Peripapillary serous detachment in the left eye (yellow arrow). (c): Overlap of
During this period, the patient presented with worsening vision in FA (peripapillary image) on ICGA (peripheral image): Peripapillary hyper-
the left eye, and an increased area of serous detachment reaching the fluorescence observed on FA in an early stage leakage with perivascular
staining (yellow arrow). On ICGA, a more extensive area of leakage was ob-
fovea could be seen on retinography (Fig. 2-e), red-free, auto-
served. Note “dark dots” (red arrow) and “stromal vessels” (blue arrow). (d):
fluorescence and infra-red. The OCT-SD showed an increase in the sub-
The yellow arrow indicates the degeneration of photoreceptors and presence of
retinal fluid, affecting the foveal zone, in addition to the increase of the septum with accumulation of subretinal fluid. The image also shows a thick-
amorphous substance and the vitreous cellularity (Fig. 2-f). ened choroid on OCT-EDI. (e): Evolution after a few days, showing an increased
Laboratory tests, including hemogram, erythrocyte sedimentation area of serous detachment reaching the fovea (retinography, yellow arrows
rate, C-reactive protein, rheumatoid factor and antinuclear antibodies delimitates the area of subretinal fluid). (f): OCT showing an increase in the
were normal. We excluded the following infectious diseases: HIV, sy- subretinal fluid, affecting the foveal zone: amorphous substance (yellow arrow)
philis, toxoplasmosis, cytomegalovirus, herpes simplex, herpes zoster and vitreous cellularity (red arrow). (g): Improved appearance of the macula on
and Bartonella henselae. Chest tomography was normal. Considering the retinography after treatment completion. (h): Improvement of serous detach-
apparent absence of infectious diseases, the patient was started on 60 ment on OCT-SD. The regeneration stage of the papillomacular bundle photo-
receptors (yellow arrow) after treatment with oral steroid.
mg/day prednisone. After 8 days, visual acuity improved to 20/250,
improving to 20/20 vision six months after slow steroid wean. This
improvement could be demonstrated on retinography (Fig. 2-g), OCT- heterogeneous group of pathologies that could be responsible.
SD (Fig. 2-h) and infra-red. Infectious causes, including syphilis and tuberculosis, were ex-
cluded due to negative serologies. Chest tomography showed no ab-
normalities, making the possibility of sarcoidosis highly unlikely.
2. Discussion
Ocular ultrasonography and neuroimaging showed no signs sug-
gestive of scleritis, tumor or lymphoma that were not consistent with
These fundoscopic findings in a previously-healthy young white
the FA and ICGA.
man revealing rapidly progressive low visual acuity suggested a

2
J.Z. Abucham-Neto, et al. American Journal of Ophthalmology Case Reports 16 (2019) 100548

Table 1 found in APMPPE, multifocal choroiditis and panuveitis, there were no

Diagnostic criteria for Vogt-Koyanagi-Harada disease.4 typical angiographic or fundoscopic patterns of any spectrum of these
Complete Vogt-Koyanagi-Harada disease (criteria 1 to 5 must be present) pathologies, as seen in this case.
1. No history of penetrating ocular trauma or surgery preceding the initial onset of Although the diagnosis of Vogt-Koyanagi-Harada syndrome (VKHS)
uveitis. requires bilateral ocular involvement, there have been reports in the
2. No clinical or laboratory evidence suggestive of other ocular disease entities. literature of unilateral and bilateral non-simultaneous involvement.2,3
3. Bilateral ocular involvement (a or b must be met, depending on the stage of disease
when the patient is examined).
Typical findings of VKHS that are found in FA (such as pin points) were
A. Early manifestations of disease. not detected in our patient. Furthermore, a circumscribed type of
(1) There must be evidence of a diffuse choroiditis (with or without anterior peripapillary serous retinal detachment that slowly advanced to the
uveitis, vitreous inflammatory reaction, or optic disk hyperemia), which may macula in both eyes is an atypical presentation of Harada disease as
manifest as one of the following:
well. Our patient initially presented with peripapillary serous retinal
(a) Focal areas of subretinal fluid, or
(b) Bullous serous retinal detachments. detachment in the left eye, eighteen months after a similar event in the
(2) With equivocal fundus findings; both of the following must be present as well: right eye that had been misdiagnosed as a papilla pit. We believe both
(a) Focal areas of delay in choroidal perfusion, multifocal areas of pinpoint eyes suffered from the same syndromic condition, but in distinct per-
leakage, large placoid areas of hyperfluorescence, pooling within subretinal iods, because the clinical presentation and findings from the com-
fluid, and optic nerve staining (listed in order of sequential appearance) by
plementary exams were similar. This observation reinforces the prob-
fluorescein angiography, and
(b) Diffuse choroidal thickening, without evidence of posterior scleritis by able diagnosis of VKHS, with bilateral ocular involvement occurring
ultrasonography. non-simultaneously.3
B. Late manifestations of disease. The diagnostic criteria for VKHS were published in 2014 (Table 1).
(1) History suggestive of prior presence of findings from 3A, and either both (2)
In this case report, the patient met the criteria for the probable form,4
and (3) below, or multiple signs from (3):
(2) Ocular depigmentation (either of the following manifestations is sufficient): because trauma and previous ocular surgeries were excluded, as were
(a) Sunset glow fundus, or other ocular and systemic pathologies.2,4
(b) Sugiura sign.
(3) Other ocular signs: 3. Conclusion
(a) Nummular chorioretinal depigmented scars, or
(b) Retinal pigment epithelium clumping and/or migration, or
(c) Recurrent or chronic anterior uveitis. We believe our case represented a variant of the VKHS in an atypical
4. Neurological/auditory findings (may have resolved by time of examination). situation, because the patient fulfilled the presumed criteria.
A. Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the Furthermore, the findings of clinical and complementary examinations
neck and back, or a combination of these factors; headache alone is not sufficient
led to this nosological entity to the exclusion of the others.
to meet definition of meningismus, however), or
B. Tinnitus, or
It is important to promptly diagnose atypical VKHS, so that early
C. Cerebrospinal fluid pleocytosis. therapy can be directed in order to improve visual prognosis.
5. Integumentary finding (not preceding onset of central nervous system or ocular Patient consent: Written consent to publish this case has not been
disease). obtained.
A. Alopecia, or
This report does not contain any personal identifying information.
B. Poliosis, or
C. Vitiligo.
Incomplete Vogt-Koyanagi-Harada disease (criteria 1 to 3 and either 4 or 5 Acknowledgements and disclosures
must be present)
1. No history of penetrating ocular trauma or surgery preceding the initial onset of
Funding
uveitis, and
2. No clinical or laboratory evidence suggestive of other ocular disease entities, and
3. Bilateral ocular involvement. No funding or grant support.
4. Neurologic/auditory findings; as defined for complete Vogt-Koyanagi-Harada
disease above, or Conflicts of interest
5. Integumentary findings; as defined for complete Vogt-Koyanagi-Harada disease
above.
Probable Vogt-Koyanagi-Harada disease (isolated ocular disease; criteria 1 to 3 The following authors have no financial disclosures: Abucham-Neto,
must be present) J.Z; Ferraz, A.A.B; Masson, A.P; Nascimento, P.A; Souza Cunha, E.
1. No history of penetrating ocular trauma or surgery preceding the initial onset of Authorship: All authors attest that they meet the current ICMJE criteria
uveitis.
for Authorship.
2. No clinical or laboratory evidence suggestive of other ocular disease entities.
3. Bilateral ocular involvement as defined for complete Vogt-Koyanagi-Harada
disease above. Acknowledgements

None.
Some diseases belonging to the group of white dots syndrome
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serpiginous choroiditis. Chao et al.1 described a case of multiple eva- manifestation in a hepatitis C carrier. J Ophthalmic Inflamm Infect. 2012;2(4):235–237.
4. Read RW, Holland GN, Rao NA, et al. Revised diagnostic criteria for Vogt-Koyanagi-
nescent white dots syndrome (MEWDS) with similar characteristics to
Harada disease: report of an international committee on nomenclature. Am J
those found in our case, including thickening of the choriocapillaris and Ophthalmol. 2001;131(5):647–652.
serous detachment observed on optical coherence tomography.1 How-
ever, despite the presence of early hypofluorescent lesions in FA, also