Rare Case of Polypoidal Choroidal Vascular Angiopathy: A Case Report

Polypoidal choroidal vasculopathy (PCV) is currently recognized as a phenotype of age-related macular degeneration (AMD). PCV is also known as” posterior uveal bleeding” and is believed to be a type of choroidal neovascularization, PCV often shows several unique clinical manifestations which appears to be different from typical neovascular AMD (tAMD). The primary abnormality involves the choroidal circulation, and the characteristic lesion is an inner choroidal vascular network of vessels ending up as an aneurysmal bulge or outward projection, visible clinically as a reddish orange, spheroid, polyp-like structure. The usual age of onset of polypoidal CNV is between 50 and 65 years with an average of 60.1 years. Here an unusual case of Polypoidal choroidal vasculopathy in 32 years male with history non-resolving enteric fever is being presented and pathogenesis, clinical features, investigation findings and management are discussed. Introduction Polypoidal choroidal vasculopathy (PCV) was first time described by Yannuzzi LA et al in 1982.Various names were given like” posterior uveal bleeding syndrome” or” multiple recurrent retinal pigment epithelium detachments”. Indocyanine green angiography (ICG) and optical coherence tomography (OCT) is the imaging modality of choice in assessing the PVC. Diagnosis is based on ICG and confirmed with fundus characteristics and OCT findings. The primary abnormality involves the choroidal circulation and the characteristic lesion is the inner choroidal vascular network of vessels ending, in an aneurysmal dilatation which appears clinically as a reddish orange, spheroid, polyp-like structure. The natural course of the disease often have a remitting-relapsing course, and clinically, it is associated with chronic, multiple, recurrent serosanguineous detachments of the retinal pigment epithelium and neurosensory retina with long-term preservation of good vision. However, the classification of PCV as distinct subtype of exudative age-related macular degeneration (AMD) easily differentiated from other diseases and other subtypes of choroidal neovascularization associated with AMD is still a great challenge. Polypoidal choroidal vasculopathy has also been described in different pathological conditions including central serous chorioretinopathy, circumscribed choroidal hemangioma; melanocytoma of the optic nerve; pathological myopia and staphyloma; or choroidal osteoma. It behaves like a neovasculopathy occurring in a variety of different diagnoses than a distinct abnormality of the inner choroidal vasculature IPCV is characterized by the presence of recurrent serosanguineous PEDs and neurosensory retinal detachments (NSRD). The vascular abnormality underlying the disorder appears to be in the inner choroid. Dilated networks of vessels terminating in aneurysmal dilatations or “polyps” can be observed on ICG angiography. Polypoidal lesions can originate from various different region such as the peripapillary region, macula, or peripheral areas. On histopathological evaluation, various cases of IPCV showed extensive fibrovascular proliferation in the subretinal space and within Bruch's membrane, and a marked lymphocytic infiltration with both B and T cells. Although laser photocoagulation appears to be very effective in preserving visual acuity in patients with IPCV, but there are also chances of spontaneous resolution of PEDs and NSRDs to occur. Case Report A 32year male presented with sudden deterioration of vision for last two months in left eye which was associated with mild ocular pain. His visual acuity in right eye was 6/6 and in left eye was 6/12 on Snellen’s chart. On examination anterior segment was within normal limit. On dilated fundus examination with indirect ophthalmscope and 78D slit lamp biomicroscopy, right eye was normal but in left eye diffuse soft confluent drusen in polypoidal pattern was present near macula with hard exudates and some calcified drusen present all over posterior pole which was gradually increasing. He denied any history of trauma but patient had history of typhoid for 4 times in last 3 months, but no other ocular complaints. Fundus photos were taken at presentation and then at 15 days interval, it shows gradual increase in size of lesions, which is associated with gradual diminution of vision up to 6/24 in 1 month duration. Kamya Sharma et al. Rare case of polypoidal choroidal vascular angiopathy: A case report International Journal of Ocular Oncology and Oculoplasty, April-June, 2016;2(2):138-141 139 Fig. 1: Fundus photograph on day one showing