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July 2018 Case Reports
of dengue hemorrhagic fever is unreported in published
literature. This report established that it was not dengue
virus per se that caused the panophthalmitis but a secondary
infection by B. cereus. Plausible hypothesis is secondary
endogenous endophthalmitis leading to panophthalmitis
caused by dengue‑induced septicemia. Microorganisms after
crossing the blood ocular barrier enter the uveal tract or retinal
circulation, lodge in small capillaries, and establish a septic
focus in the retina that can later break into the vitreous. If a
large septic embolus passes through the central retinal artery
and disseminates throughout the retina, retinal necrosis and
ischemia may occur thus allowing microorganisms to quickly
invade the vitreous and further into anterior segment.[5]
Patients with dengue who present with endophthalmitis or
panophthalmitis should be aggressively managed, and
samples (aqueous/vitreous or eviscerated material) should be
subjected to standard microbiological tests to look for offending
organisms and tailor therapy accordingly.
Conclusion
We present a hitherto unreported case of culture‑positive
unilateral panophthalmitis in a patient with serology‑positive
dengue hemorrhagic fever. Ophthalmologists need to be aware
of all the possible ocular complications in patients with such
a scenario, be vigilant of sight‑threatening complications and
treat aggressively.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Central retinal arterial occlusion in a
patient with pyoderma gangrenosum
Koushik Tripathy, Shahana Mazumdar, Barsha Sarma
A  74‑year‑old male presented to us with a history of vision loss for
36 hours in the right eye (RE). The RE had a visual acuity of hand
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DOI:
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*****
Department of Vitreoretina and Uvea, ICARE Eye Hospital and
Postgraduate Institute, Noida, Uttar Pradesh, India
Correspondence to: Dr. Koushik Tripathy, Department of
Vitreoretina and Uvea, ICARE Eye Hospital and Postgraduate
Institute, E3A, Sector‑26, Noida ‑ 201 301, Uttar Pradesh, India.
E‑mail: drkoushiktripathy@icarehospital.org
Manuscript received: 08.01.18; Revision accepted: 25.03.18
1019
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1. Gupta P, Jain C, Aggarwal A, Gupta SC. Dengue fever presenting
with macular hemorrhages. Retin Cases Brief Rep 2011;5:213‑8.
2. Koh YT, Sanjay S. Characteristics and ophthalmic manifestations
of the classic dengue fever epidemic in Singapore (2005‑2006). Asia
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3. Lim WK, Mathur R, Koh A, Yeoh R, Chee SP. Ocular manifestations
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4. Teoh SC, Chan DP, Laude A, Chee C, Lim TH, Goh KY. Dengue
chorioretinitis and dengue‑related ophthalmic complications. Ocul
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5. Ng AW, Teoh SC. Dengue eye disease. Surv Ophthalmol
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8. Saranappa SB, Sowbhagya HN. Panophthalmitis in dengue fever.
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9. Sriram S, Kavalakatt JA, Pereira Ad, Murty S. Bilateral
panophthalmitis in dengue fever. Ann Trop Med Public Health
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10. Thein TL, Ng EL, Yeang MS, Leo YS, Lye DC. Risk factors for
concurrent bacteremia in adult patients with dengue. J Microbiol
Immunol Infect 2017;50:314‑20.
11. Rishi E, Rishi P, Sengupta S, Jambulingam M, Madhavan HN, Gopal L,
et al. Acute postoperative Bacillus cereus endophthalmitis mimicking
toxic anterior segment syndrome. Ophthalmology 2013;120:181‑5.
12. Cowan CL Jr., Madden WM, Hatem GF, Merritt JC. Endogenous
Bacillus cereus panophthalmitis. Ann Ophthalmol 1987;19:65‑8.
movements. The fundus revealed a pale retina, cattle tracking in
the retinal vessels, and a cherry‑red spot at the macula. The patient
was a known case of pyoderma gangrenosum (PG) and had
received intravenous methylprednisolone and cyclophosphamide
at the onset of visual symptoms. An emergency anterior chamber
paracentesis was performed following unsuccessful attempts of
ocular massage. The patient improved to 6/9 in the RE 4 months
after paracentesis. The patient had an aggressive course of PG, for
which he needed a combination of oral steroid, immunomodulator
therapy and biologicals. An association between central retinal
arterial occlusion and PG has not been reported before, according
to the best of authors' knowledge.
Key words: Anti-phospholipid antibody, central retinal
arterial occlusion, cherry‑red spot, ischemic tolerance of retina,
paracentesis
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which allows others to remix, tweak, and build upon the work non‑commercially,
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For reprints contact: reprints@medknow.com
Cite this article as: Tripathy K, Mazumdar S, Sarma B. Central retinal arterial
occlusion in a patient with pyoderma gangrenosum. Indian J Ophthalmol
2018;66:1019-21.
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1020 Indian Journal of Ophthalmology Volume 66 Issue 7
Central retinal arterial occlusion (CRAO) is an ophthalmic
emergency and early treatment is crucial. Arterial occlusions
have been reported in association with atherosclerosis,
hypercoagulable states, and various inflammatory disorders
including systemic lupus erythematosus, polyarteritis nodosa,
Behçet’s disease, Wegener’s granulomatosis, Crohn disease,
and Churg–Strauss syndrome. We report a patient with
pyoderma gangrenosum (PG) who developed CRAO and
underwent anterior chamber (AC) paracentesis after 36 hours
of symptom onset.
Case Report
A 74‑year‑old male presented to us with a history of sudden
onset vision loss for 36 hours in the right eye (RE). The
best‑corrected visual acuity (BCVA) was hand movements
with accurate projection of rays in the RE and 6/6p in the left
eye  (LE). The RE showed relative afferent pupillary defect.
Intraocular pressure was 14 mmHg in the RE and 12 mmHg
in the LE. Both eyes had posterior‑chamber intraocular lens.
There were no cells in the AC of either eye. RE had a pale
retina, cattle tracking in the retinal vessels, and a cherry‑red
spot at the macula [Fig. 1a]. LE showed few small drusen.
Spectral‑domain optical coherence tomography (SDOCT,
RTvue, Optovue Inc., California, USA) revealed an increased
reflectivity of the inner retina  [Fig.  2a]. The patient was a
known case of PG for 1 year. He was given intravenous
methylprednisolone (IVMP, 125 mg) and cyclophosphamide
a b
c d
e f
Figure 1: (a) At presentation, there was whitened retina, cherry‑red
spot at macula, and cattle tracking of blood columns. (b) One day
after paracentesis, some apparent clearing of retinal pallor nasal to
the fovea was noted. Superotemporal cattle‑tack appearance had
disappeared.  (c‑e) The fluorescein angiogram at 51 s, 61 s, and
8  min 21 s showed no filling of the inferotemporal retinal vessels.
(f) At 4th month, mild optic disc pallor was noted.
500 mg at the onset of the visual decline by the rheumatologist,
considering it a manifestation of inflammatory phenomenon
in PG. The patient had a very aggressive/uncontrolled
course of systemic disease and required multiple hospital
admissions due to the same. Two weeks before the onset
of visual symptoms, he had received IVMP 500 mg and
cyclophosphamide 500 mg for PG. A clinical diagnosis of
bilateral pseudophakia and CRAO in the RE was made.
An emergency AC paracentesis was performed following
unsuccessful attempts of ocular massage. On the 1st day after
paracentesis, the BCVA of RE was 6/36. The superotemporal
cattle‑track appearance had disappeared [Fig. 1b]. The fundus
fluorescein angiogram did not show any obvious cilioretinal
artery. In the RE, the inferotemporal vessels did not fill up
even at 8 min [Fig. 1c‑e]. Echocardiography did not detect
any clots or vegetation. The carotid Doppler demonstrated
eccentric calcific plaque in the right internal carotid artery
with <30% stenosis. Electrocardiogram was unremarkable.
Hemoglobin was 11.8  gm/dl and total leukocyte count was
10,700/mm3. Erythrocyte sedimentation rate (ESR) was 33 mm
in the 1st h and C‑reactive protein (CRP) was 21.9 mg/dl. There
was no clinical evidence of giant cell arteritis. Blood pressure
was 130/86 mmHg and the fasting blood sugar was 101 mg/dl.
The ESR and CRP of the patient remained high in follow‑up at
1 month (82 mm in the 1st h, 113.2 mmHg), 2nd month (101 mm
in the 1st h, 63.3 mg/dl), and at 7th month (63 mm in the 1st h,
29  mg/dl). One month after the onset of CRAO, the skin
lesions increased and the appetite of the patient reduced with
increased inflammatory markers (ESR and CRP). In the course
of disease, the patient showed good response to steroids,
partial‑to‑good response to antitumor necrosis factor agents,
poor response to mycophenolate mofetil, and combination
of cyclophosphamide and steroid was considered to be less
useful. Good response to cyclosporine was noted but it had
b
Figure 2:  (a) The optical coherence tomography at presentation
showed hyperreflectivity of inner retinal layers. (b) At the 4th month,
the optical coherence tomography showed loss of foveal depression
and retinal thinning.
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July 2018 Case Reports
to be stopped due to acute kidney injury. He subsequently
received etanercept with some response.
The BCVA in the RE improved to 6/18 at 1 month and 6/9
at 4 months. At 4 months, mild pallor of optic disc and mild
pigmentary changes at the fovea were noted [Fig. 1f]. The
SDOCT revealed loss of foveal depression and retinal thinning
in the RE [Fig. 2b]. At the last follow‑up at 10th month, the
patient was receiving oral prednisolone, methotrexate, and
IV infliximab for PG.
Discussion
PG is a rare inflammatory disease of skin belonging to a
spectrum of neutrophilic dermatoses which also include
Behçet’s disease. Leukocytoclastic vasculitis is seen in about
40% [1] cases with PG. According to the best of authors’
knowledge, there is no prior report of an association of CRAO
with PG. PG has been reported to be associated with inferior
vena caval thrombosis in antiphospholipid syndrome,[2] portal
vein thrombosis in chronic myelomonocytic leukemia, [3]
and aortoiliac embolism[4] in a patient with acne conglobata
and myocardial infarction. A case of superficial venous
thrombosis in pregnancy has been reported which ultimately
resulted in PG lesions due to Koebner phenomena.[5] Chronic
inflammatory diseases may predispose to a hypercoagulable
state due to upregulation of procoagulant factors and reduced
anticoagulant mechanisms.[2] Furthermore, inflammation of
vessels may cause CRAO, as in giant cell arteritis. In our patient,
there was no evidence of active retinal arterial inflammation,
leukemia, venous thrombosis, or acne conglobata, and the
pathogenesis appeared to be related to arterial thrombosis.
A limitation of this report is that antiphospholipid antibody
or anticardiolipin antibody was not tested.
We, however, agree that a single case report does not
necessarily prove a cause‑effect relation of the diseases and
may even be coincidental. It has been shown by Hayreh and
Zimmerman that “massive irreversible retinal damage occurs
in CRAO lasting about 240 min.”[6] However, spontaneous
reperfusion of both the central retinal artery and lateral
posterior ciliary artery can happen as late as 4 days after
documented ischemia with a final BCVA of 20/30.[7] This is
because, unlike the experimental models, CRAO in human
may not be complete. Furthermore, there may be some
ischemic tolerance of the retina mediated by the upregulation
of neuroprotective agents and antiapoptotic factors due to
previous brief episodes of retinal ischemia.[8] Our patient
presented >24 h after the onset of sudden visual loss. We
decided not to give up and to give a trial of AC paracentesis.
Good final visual acuity (≥6/9) could be achieved in this case,
which can be noted in only 12.5% of all eyes with CRAO.[7]
However, a large series evaluating 74 patients concluded that
paracentesis did not provide additional visual gain.[9] In
our case, anatomical and visual recovery on the 1st day after
paracentesis may point toward its beneficial role. However, the
visual recovery, in this case, could also have been related to
1021
the control of inflammatory process with steroids and various
immunomodulator therapy/biologicals or the natural history
of CRAO[10] rather than AC paracentesis. High ESR and CRP at
presentation may denote an underlying activity of PG which
may have predisposed to CRAO.
Conclusion
In conclusion, we report a rare case of PG who developed
CRAO and achieved good final BCVA after paracentesis at
36 hour of onset of symptoms and management with systemic
steroids and immunomodulator/biological therapy.
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.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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8. Junk AK, Rosenbaum PS, Engel HM, Rosenbaum DM. Visual
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9. Fieß A, Cal Ö, Kehrein S, Halstenberg S, Frisch I, Steinhorst UH,
et al. Anterior chamber paracentesis after central retinal artery
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outcome. Am J Ophthalmol 2005;140:376‑91.
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