DJO
E-ISSN 2454-2784
61
Anubhav Chauhan
Department of Ophthalmology, Govt. Medical College, Nahan, District Sirmour, Himachal Pradesh, India
Summary
We report a case of a 48 year old asymptomatic male who came to us for routine ocular examination but was diagnosed with retinal vasoproliferative tumour(VPT) and angioid streak.Retinal vasoproliferative tumours(VPTs) are a rare entity and after an extensive search on the internet, this is probably and to the best of our knowledge,the first reported case of VPT in association with angioid streak. Delhi J Ophthalmol 2018;28;61-3; Doi http://dx.doi.org/10.7869/djo.362
Keywords: ocular,vasoproliferative,angioid
Introduction
Retinal VPTs are rare, benign retinal vascular tumors. They are yellowish red in appearence, and are mostly located on the inferotemporal peripheral retina.Visual acuity (VA) in patients with VPTs vary from light perception to 6/6, depending on the complications of this disease.1
Case Report
A 48 year old male presented to the department of ophthalmology for routine ocular examination. There were no complaints.There was no history of any systemic disease, trauma, previous ocular disease and surgery. Ocular examination was carried out and his visual acuity was 6/6 in both the eyes ; pupillary reactions, ocular movements, colour vision, slit lamp examination and intraocular pressure were normal bilaterally. Expecting everything to be normal, the last test in the form of fundus examination was carried out and here came the surprise. Fundus examination of the right eye revealed a whitish red elevated nodular lesion protruding above the retinal surface just inferotemporal to the macula along with angioid streak starting temporal to the optic disc but terminating medial to the macula. Fluorescein angioraphy (FA) revealed early filling in the
Figure 2: Early Phase During Fluorescein Angiography
arterial phase with increasing hyperfluorescence and late leakage. FA showed hyperfluorescent window defect for the angioid streak.The fundus of the left eye was within normal limits. Other retinal imaging facilities were not available at our centre. The clinical and fundus findings were suggestive of a vasoproliferative tumour with angiod streak.The general physical and systemic examination was normal. Investigations in the form of a complete blood count with peripheral smear,blood sugar, renal function tests,lipid profile, magnetic resonance imaging of the brain and ultrasonography of the abdomen was carried out as a number of closely related conditions to VPTs have systemic involvement as well and must be ruled out. All his investigations were within normal limits.The patient was advised consultation at a higher centre as facility for retina treatment in the form of lasers and surgery (if needed for the lesion) were not available at our centre.The patient refused for the same as he stated that he had no ocular complaint.
Figure 1: Fundus photograph
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Delhi Journal of Ophthalmology
Brief Communication
A Retinal Surprise
DJO
Brief Communication
62
Other possible associations with VPTs are Waardenburg’s syndrome, neurofibromatosis type I, systemic hypertension, and hyperlipidemia. Males and females appear to be equally affected and the mean age of presentation is the third to fourth decade of life. The differential diagnosis include retinal capillary hemangioma, cavernous hemangioma, Wyburn–Mason syndrome and choroidal hemangioma. Retinal capillary hemangioma presents between 10 to 40 years of age and these lesions have dilated tortuous feeder vessels. When present with Von Hippel Lindau hippel lindau syndrome, renal cell carcinoma, central nervous system hemangiomas, and pheochromocytomas are common associations. Retinal cavernous hemangiomas appear as a group of blood filled saccules within the inner retinal layers or on the surface of the optic disc and have been described as having a “cluster of grapes” appearance. Wyburn–Mason syndrome is characteristic, with dilated and tortuous retinal vessels extending from the optic disc to the retinal periphery. Choroidal hemangioma appears as an orange choroidal mass with indistinct margins that blend with the surrounding choroid.3 The term ‘reactionary retinal glioangiosis’ has also been used for VPTs. Patients tend to present with visual loss, floaters, photopsia as VPTs complications include macular oedema, exudative retinal detachment, neovascular glaucoma and epiretinal membranes.4 The histopathological feature of VPTs reveals a mix of vascular and glial proliferation. The glial component usually exhibits fusiform and rather uniform cells. Vascular component shows dilated vessels with mural hyalinization and occasional thrombosis.VPTs exhibit feeder vessels, although with less dilation and tortuosity than that seen on retinal capillary hemangioma, its major differential diagnosis.5
Figure 2: Mid Phase During Fluorescein Angiography
Figure 2: Late Phase During Fluorescein Angiography
Discussion
VPTs are classified into primary (idiopathic) or secondary, based on related ophthalmic findings. Primary VPTs tend to be solitary, small while secondary VPTs are more often multifocal, bilateral, and are believed to be a result of various ocular diseases like Retinitis pigmentosa,Coats disease, previous retinal detachment repair, Toxoplasmosis, Aniridia, Congenital hypertrophy of retinal pigment epithelium, and Retinopathy of prematurity. Both primary and secondary VPTs occur inferotemporally or inferiorly in the fundus. VPTs have classic peripheral location, indistinct margins, and color.2
Volume 28
P-ISSN 0972-0200
Tests for VPTs include echography, FA, and Optical coherence tomogram. FA reveals early filling in the arterial phase with increasing hyperfluorescence and late leakage. Various treatment modalities are available including cryotherapy, laser photocoagulation, photodynamic therapy, brachytherapy, surgical resection, intravitreal injections, and immunomodulators.6 Angioid streaks are crack like dehiscences in Bruch’s membrane and are associated with diseases like hemochromatosis, diabetes mellitus, sickle cell hemoglobinopathies, pseudoxanthoma elasticum, hemolytic anaemia, neurofibromatosis, paget’s disease, Ehlers–Danlos syndrome, Sturge–Weber syndrome and hyperphosphatemia. Patients with angioid streaks are generally asymptomatic, unless complicated by traumatic Bruch’s membrane rupture or macular choroidal neovascularization. Typically, angioid streaks have a ‘window defect’ FA due to atrophy of RPE adjacent to them.Treatment modalities include laser photocoagulation, macular translocation surgery, transpupillary thermotherapy, photodynamic therapy and anti-vascular endothelial growth factor.7
Number 4
April - June 2018
DJO
E-ISSN 2454-2784
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1. Huang YM, Chen SJ. Clinical characters and treatments of retinal vasoproliferative tumors. Taiwan J Ophthalmol 2016; 6:85-8. 2. Shields CL, Kaliki S, Dahmash SA, Rojanaporn D, Shukla SY, Reilly B, et al. Retinal Vasoproliferative Tumors Comparative Clinical Features of Primary vs Secondary Tumors in 334 Cases. JAMA Ophthalmol 2013; 131:328-34. 3. Turell ME, Singh AD. Vascular Tumors of the Retina and Choroid: Diagnosis and Treatment. Middle East African Journal of Ophthalmology 2010; 17:191-200. 4. Damato B. Vasoproliferative retinal tumour. Br J Ophthalmol 2006; 90:399–400. 5. Marback EF, Guerra RL, Maia Junior ODO, Marback RL. Retinal vasoproliferative tumor. Arq Bras Oftalmol 2013; 76:2003. 6. Venkatesh R, Gurav P, Dave PA, Roy S. Retinal meteor. Indian J Ophthalmol 2017; 65:879-81. 7. Georgalas I, Papaconstantinou D, Koutsandrea C, Kalantzis G, Karagiannis K, Georgopoulos G, et al. Angioid streaks, clinical course, complications,and current therapeutic management. Ther Clin Risk Manag 2009; 5:81-9.
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Cite This Article as: Chauhan A, Parmar YS. A Retinal Surprise. Acknowledgements: We would like to thank our patient. Conflict of interest: None declared Source of Funding: None Date of Submission: 24 December 2017 Date of Acceptance: 02 February 2018
Address for correspondence Anubhav Chauhan, MS
Senior Resident, Department of Ophthalmology, Govt. Medical College, Nahan, District Sirmour, Himachal Pradesh, India. Email id:
[email protected]
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