10.5005/jp-journals-10020-1022 Sandhya Hegde et al CASE REPORT
Bilateral Ocular Ischemic Syndrome Following Radiotherapy for Hodgkin’s Lymphoma Sandhya Hegde, Pukhraj Rishi, Mohammed Salman Kazi
ABSTRACT We report a case of bilateral ocular ischemic syndrome in a middle aged female 7 years following radiotherapy for Hodgkin’s lymphoma involving the cervical lymph nodes. On presentation she had very early features suspicious of ocular ischemic syndrome which were confirmed with a carotid Doppler showing nearly absent circulation through the carotid system due to a radiation induced stenosis. We highlight the importance of regular screening in patients receiving radiotherapy in view of its life- threatening consequences. Keywords: Ocular ischemia, Carotid Doppler, Hodgkins lymphoma. How to cite this article: Hegde S, Rishi P, Kazi MS. Bilateral Ocular Ischemic Syndrome Following Radiotherapy for Hodgkin’s Lymphoma. World J Retina Vitreous 2012;2(1): 14-17. Source of support: Nil Conflict of interest: None declared
microaneurysms. Fluorescein angiogram showed delayed choroidal and arterial filling, presence of a leading edge, microaneurysms and staining of both arteries and veins in late frames (Fig. 1). She was suspected to have ocular ischemic syndrome and advised for an electroretinogram. A delayed 30 Hz flicker implicit time, considered as a marker for retinal ischemia was recorded as 32.0 and 36.5 ms in right and left eye respectively confirming bilateral retinal ischemia with the left eye being more severely affected2 (Fig. 2). A subsequent carotid color Doppler ultrasonography revealed 100% stenosis in both common carotids as well as the right external and internal carotid arteries along with 99% stenosis in left external and internal carotid arteries (Fig. 3). There was no evidence of intimal plaques. The flow was normal in the vertebral arteries which most likely compensated for the vascular supply to the brain and avoided any neurological symptoms. She was referred to a cardiovascular surgeon and has been lost to follow-up since then.
INTRODUCTION DISCUSSION Ocular ischemic syndrome1 is a manifestation of a systemic disease presenting with signs of global ocular ischemia, such as microaneurysms, arteriolar attenuation, venular dilatation, prominent episcleral vessels, rubeosis of unknown cause or silent uveitis. We report an interesting case who presented with bilateral ocular ischemia due to total occlusion of the entire carotid arterial trunk.
Ocular ischemic syndrome is characterized by chronic ocular arterial hypoperfusion with the obstruction of flow
CASE REPORT A 66-year-old female presented to us with gradual bilateral loss of vision for the past 5 months. She was diagnosed elsewhere to have metabolic syndrome with diabetes mellitus, hypertension, hyperlipidemia causing retinopathy. She gave a past history of Hodgkin’s lymphoma 7 years back for which she had received chemotherapy and radiotherapy. The patient did not have any details of the treatment received except that radiation was given over the neck on both sides. She had been in total remission since then and had received no treatment till date. Her best corrected visual acuity was 6/12, N10 and 6/36, N18 respectively in right and left eyes. Intraocular pressure was 9 mm Hg in both eyes. Anterior segment evaluation was normal with early cataract changes. Fundus examination showed mild arteriolar attenuation with midperipheral
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Fig. 1: Fundus fluorescein angiogram of both eyes in early arteriovenous phase (above) showing midperipheral microaneurysms with sludging of the blood column. Fundus fluorescein angiogram of both eyes in late phase (below) showing staining of all arteries and veins
JAYPEE
WJORV Bilateral Ocular Ischemic Syndrome Following Radiotherapy for Hodgkin’s Lymphoma Ganzfeld Summary Rod response
Maximal response
Oscillatory potentials
Cone response
Field view
Field view 30 Hz Flicker
Fig. 2: ERG of both eyes showing reduced and delayed scotopic and photopic responses with 30 Hz implicit times more than 30 msec in both eyes suggestive of ischemic changes
World Journal of Retina and Vitreous, January-April 2012;2(1):14-17
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Sandhya Hegde et al
trial 6 revealed abnormal carotid Doppler in 22% and abnormal ocular Doppler in 16% of the survivors. The relative risk of stroke is nearly doubled in these survivors after a median gap of 9 years. Long-term survivors of nasopharyngeal carcinoma postradiotherapy also have shown similar incidence of carotid stenosis.7 Carotid disease may be picked up clinically based on symptoms of transient ischemic attacks and amaurosis fugax or on regular screening for carotid bruit on auscultation and stenosis on Doppler scan. Our patient was in remission for 7 years and had not had a carotid Doppler during this time nor did she experience such symptoms. This could be the reason for such advanced stenosis and bilateral ocular ischemic changes. Also the presence of collaterals from the external carotid and vertebral arteries could delay the presentation of ocular ischemia while her systemic diseases could contribute toward worsening of ischemic changes. A prompt referral might allow for timely surgical intervention and life saving treatment in such cases. Carotid end arterectomy has been accepted as choice of treatment in symptomatic stenosis >70% with 81% reduction in chances of futher TIA’s.8 It can cause 80% increase in retinal flow with visual stabilization. Carotid artery stenting has also proven beneficial especially in cases with stenosis. Fig. 3: Carotid color Doppler study showing absence of blood flow in both common and external carotid arteries with minimal flow noted in left internal carotid artery (red color)
being commonly at the level of carotid artery. The reduction of flow is usually unilateral and commonly due to an atherosclerotic plaque. Diabetes mellitus, hypertension, hyperlipidemia contribute further to the carotid obstruction.3 Radiation therapy to the neck nodes is known to cause endothelial damage leading to fibroblastic deposition and luminal stenosis involving carotid artery which over long term can cause severe compromise of carotid flow. Cervical radiation therapy is a modality of treatment for initial stages of Hodgkin’s lymphoma and show very good response when combined with chemotherapy in specific histological types. Hodgkin’s lymphoma can have bimodal age of presentation with children of 15 to 25 years also getting affected. Due to the recent modalities of treatment, survival rates of these patients has increased several fold and is around 88% at present.4 This allows for long-term effects of the therapeutic modalities to cause various manifestations. Radiotherapy is known to have effects on the vascular system with higher incidences of coronary, subclavian and carotid arteries in that order.5 A 5 years
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CONCLUSION An ophthalmologist can play an important role in early diagnosis and referral of cases with ocular ischemic syndrome. A high index of suspicion with knowledge of variable presentations of this syndrome may be helpful in patients having undergone radiotherapy for head and neck cancers. REFERENCES 1. Brown GC, Magargal LE. The ocular ischemic syndrome. Clinical, fluorescein angiographic and carotid angiographic features. Int Ophthalmol 1988;11:239-51. 2. Kjeka O, Bredrup C, Krohn J. Photopic 30 Hz flicker electroretinography predicts ocular neovascularization in central retinal vein occlusion. Acta Ophthalmol Scand 2007 Sep;85(6): 640-43. 3. Mizener JB, Podhajsky P, Hayreh SS. Ocular ischemic syndrome. Ophthalmology 1997;104:859-64. 4. Gustavsson A, Osterman B, Cavallin-Stahl E. A systematic overview of radiation therapy effects in Hodgkin’s lymphoma. Acta Oncol 2003;42:589-604. 5. Hull MC, Morris CG, Pepine CJ, Mendenhall NP. Valvular dysfunction and carotid, subclavian, and coronary artery disease in survivors of Hodgkin lymphoma treated with radiation therapy. JAMA 2003;290:2831-37. JAYPEE
WJORV Bilateral Ocular Ischemic Syndrome Following Radiotherapy for Hodgkin’s Lymphoma 6. Elerding SC, Fernandez RN, Grotta RC, Lindberg RD, Causay LC, McMurtrey MJ. Carotid artery disease following external cervical irradiation. Ann Surg 1981 Nov;194(5): 609-15. 7. Lam WW, Yuen HY, Wong KS, Leung SF, Liu KH, Metreweli C. Clinically underdetected asymptomatic and symptomatic carotid stenosis as a late complication of radiotherapy in Chinese nasopharyngeal carcinoma patients. Head and Neck 2001 Sep; 23(9):780-84. 8. North American symptomatic carotid endarterectomy trial collaborators. Beneficial effect of carotid endarterectomy in symptomatic patients with high grade carotid stenosis. N Engl J Med 1991;325:445-53.
ABOUT THE AUTHORS Sandhya Hegde (Corresponding Author) Fellow, Department of Vitreo-retina, Sankara Nethralaya, Shri Bhagwan Mahavir Vitreoretinal Services, 18 College Road, Chennai Tamil Nadu, India, e-mail:
[email protected]
Pukhraj Rishi Consultant, Department of Vitreo-retina, Sankara Nethralaya, Chennai Tamil Nadu, India
Mohammed Salman Kazi Fellow, Department of Vitreo-retina, Sankara Nethralaya, Chennai Tamil Nadu, India
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