Download Central retinal vein occlusion with secondary cilioretinal artery

Taiwan Journal of Ophthalmology 2 (2012) 151e154
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Case report
Central retinal vein occlusion with secondary cilioretinal artery occlusion treated
with intravitreal bevacizumab (Avastin)
Yu-Chien Chung a, Shih-Jen Chen a, b, *, Shih-Yun Lee a, Fenq-Li Lee a, b, Sui-Mei Lee a, b
a
b
Department of Ophthalmology, Taipei Veterans General Hospital, Taipei, Taiwan
School of Medicine, National Yang-Ming University, Taipei, Taiwan
a r t i c l e i n f o
a b s t r a c t
Article history:
Received 17 May 2012
Received in revised form
6 August 2012
Accepted 30 August 2012
Available online 2 October 2012
Two young male patients experienced acute visual disturbance in one eye, with central scotoma showing
central retinal vein occlusion with cilioretinal artery occlusion (CLRAO). Optical coherent tomography
(OCT) revealed inner retinal edema at areas of retinal infarction. An intravitreal injection of Avastin was
administered to these two patients with improvement of symptoms within 4e8 weeks. These two cases
suggested that OCT may help detect CLRAO by showing inner retinal edema, and demonstrated that
intravitreal injection of Avastin may improve venous engorgement and retinal hemorrhage, as well as
circulation of cilioretinal artery, thus shortening the disease course.
Copyright Ó 2012, The Ophthalmologic Society of Taiwan. Published by Elsevier Taiwan LLC. All rights
reserved.
Keywords:
bevacizumab (Avastin)
central retinal vein occlusion
cilioretinal artery occlusion
1. Introduction
Central retinal vein occlusion (CRVO) is a frequent primary
vascular disorder of the retina with vein blockage in the optic nerve
head. Some eyes with CRVO, at its onset, may have associated cilioretinal artery occlusion (CLRAO). Oosterhuis in 19681 and then
Hayreh in 19712 first reported this condition. The pathogenesis of
CLRAO in patients with CRVO was unknown. In one study, retinopathy resolved in 70% of the eyes with nonischemic CRVO and
CLRAO in a median time of 8 months.3
2. Case reports
2.1. Case 1
A healthy 38-year-old man presented with two episodes of
transient blackout in the left eye for 1 day prior to coming to our
clinic. On ophthalmic examination, visual acuity was 6/6 in both
eyes, and visual field (VF) revealed cecocentral scotoma in the left
eye. Dilated fundus examination showed venous engorgement of
four quadrants, disc hemorrhage, and sectoral retinal whitening
over the territory of one artery, with optical coherent tomography
* Corresponding author. Department of Ophthalmology, Taipei Veterans General
Hospital, Shih-Pai Road, Taipei 112, Taiwan.
E-mail address: [email protected] (S.-J. Chen).
(OCT) showing inner retinal edema (Fig. 1). Fluorescein angiography (FA) disclosed pulsatile filling of the artery with area of
hypoperfusion above the fovea (Fig. 2). Carotid angiography
revealed no thrombus or filling defect in the carotid or vertebral
artery as well as in the ophthalmic or central retinal artery. Other
systemic evaluation, including blood chemistry, inflammatory
markers (erythrocyte sedimentation rate (ESR), C-reactive protein
(CRP)), blood coagulation profile, and autoimmune markers, were
normal. The obstructed retinal artery was suspected to be a cilioretinal artery with secondary obstruction by increased intramural
pressure due to CRVO.
Intravitreal injections of Avastin (1.25 mg/0.05 mL) and triamcinolone (0.25 mg/0.05 mL) were given on the day of presentation,
and the patient was treated with two more intravitreal injection of
Avastin (1.25 mg/0.05 mL) in the following 2 months.
In the following months, although the retinal whitening did not
improve, the venous engorgement and artery circulation delay
ameliorated with smaller scotoma on VF 1 month later. The artery
circulation normalized with persistent distal ischemic area 2 months
later. However, atrophy of inner retina was noted on OCT (Fig. 1).
2.2. Case 2
A 22-year-old man had newly diagnosed hypertension for 2
weeks prior to onset of ocular symptoms, and was on medical
treatment. He presented to our clinic with gray vision in the left eye
for 2 weeks. On ophthalmic examination, the best-corrected visual
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Y.-C. Chung et al. / Taiwan Journal of Ophthalmology 2 (2012) 151e154
Fig. 1. Color fundus picture, OCT, and visual field of Case 1 (A) at presentation, and (B) 4 and (C) 8 weeks after intravitreal Avastin injection. The color fundus picture showed
ameliorated venous engorgement and retinal whitening. Vertical OCT showed inner retinal swelling at the location of CLRAO (arrows) without macular edema, which regressed
with inner retinal atrophy 8 weeks after therapy. Visual fields revealed gradual improvement of central scotoma. CLRAO ¼ cilioretinal artery occlusion; OCT ¼ optical coherent
tomography.
acuity was 6/6 in both eyes, with an intraocular pressure of
18/17 mmHg. Anterior segment was normal. Dilated fundus
examination revealed disc swelling with retinal hemorrhage and
faint retinal infarction in the left eye. OCT on the same day showed
no macular edema but inner retinal swelling in the upper portion of
fovea. VF demonstrated a central scotoma compatible with the
location of retinal infarction (Fig. 3). One week later, he was
administered an intravitreal injection of Avastin (1.25 mg/0.05 ml).
Two weeks later, FA disclosed a minimal hypoperfusion patch
superior to the fovea with mild artery and venous delay in the left
eye (Fig. 2). One month later, OCT showed atrophy of inner retina
and VF showed ameliorated central scotoma in the left eye (Fig. 3).
3. Discussion
Hayreh reported a case series of 38 patient having CRVO with
CLRAO from 1974 to 1999.3 Most reported cases of nonischemic
CRVO with CLRAO were young adults (mean 45.8 16.0 years;
range, 19e80 years), compared to those of CRVO alone that
increased with age.4 Nearly all patients had disc edema with
hemorrhage at initial visit, and all eyes had variable degrees of
venous engorgement during acute phase. Generally, FA showed
only transient hemodynamic block and not the typical CLRAO.
Without intervention, resolution time of retinopathy in nonischemic CRVO was mostly 8 months (mean 42 101.0 months;
median, 8.1 months; range, 0.7e472.3 months). In our cases,
improved retinopathy could be observed after administration of
intravitreal Avastin, with resolution at 1e2 months of follow-up. To
our knowledge, this is the first report of treatment of CRVO with
CLRAO with intravitreal Avastin.
Two hypotheses have been proposed for the pathogenesis of
CRVO with CLRAO.5,6 The first hypothesis is the development
of CLRAO secondary to the raised capillary pressure caused by
CRVO.7e12 The second hypothesis suggests a primary reduction in
perfusion pressure of the cilioretinal and retinal arteries, leading to
decreased retinal circulation and subsequent venous stasis and
thrombosis.8,9,11 In our cases, venous engorgement alleviated after
intravitreal Avastin treatment, with subsequent improvement of
retinal infarction. The clinical picture supported the first hypothesis.
Retinal vein occlusion leads to an increased expression of
vascular endothelial growth factors (VEGFs) in retina and retinal
pigment epithelium and an increased release into the vitreous,
causing neovascularization and vascular hyperpermeability with
subsequent breakdown of the blooderetina barrier.13 Intraocular
VEGF is elevated in patients with both ischemic CRVO and nonischemic CRVO.14 The possible role of anti-VEGF agents in these two
patients with nonischemic CRVO, nonmacular edema, but CLRAO is,
first, to decrease the hyperpermeability status of retinal vein
induced by venous stasis, which, hopefully, will decrease the
edema surrounding the occluded vein and thus improve the
circulation of the corresponding or nearby artery. Another role of
Avastin injection is to decrease the inflammation. A recent study
has shown that Avastin can not only prevent the upregulation of
VEGF, but also decrease the proinflammatory cytokine.15 Nonischemic CRVO in young healthy patients had been suggested to be
associated with inflammatory reaction with secondary occlusion.13
Y.-C. Chung et al. / Taiwan Journal of Ophthalmology 2 (2012) 151e154
153
Fig. 2. Color fundus picture, OCT, and visual field of Case 2 (A) at presentation, and (B) 1 week and (C) 4 weeks after intravitreal Avastin injection. Arrows point to the area of retinal
infarction in the fundus picture and OCT. The inner retina became atrophy at 4 weeks, as shown by the OCT. Visual fields showed gradual improvement of scotoma. OCT ¼ optical
coherent tomography.
Fig. 3. (A) Baseline FA of Case 1 and (B) 2-week follow-up FA of Case 2. Both images showed area of hypoperfusion above the fovea as well as the retinal hemorrhage, disc swelling,
venous congestion, and leakage. FA ¼ fluorescein angiography.
Hence, Avastin was worth a trial in these two patients in order to
improve the arterial as well as venous circulation, and the results
seemed promising. Larger case series are needed to confirm this
hypothesis of treatment effects.
Unlike CRVO with macular edema, inner retina swelling, as
demonstrated by OCT, was the feature of CRVO complicated with
CLRAO. In Case 2, with faint retinal infarction in the fundus because
of the mild symptoms and delayed visit (2 weeks after onset),
significant inner retinal edema was still demonstrated by OCT. OCT
is a valuable tool for CRVO not only for the evaluation of macular
edema but also for the differential diagnosis of combined artery
occlusion.
In conclusion, nonischemic CRVO with disc hemorrhage may
obscure the detection of secondary CLRAO. OCT may help in the
detection of CLRAO by revealing the presence of inner retinal
edema. Intravitreal injection of Avastin may improve venous
engorgement and retinal hemorrhage as well as circulation of cilioretinal artery.
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