Clinical Manifestations Of Polypoidal Choroidal Vasculopathy

Background and ObjectivePolypoidal choroidal vasculopathy(PCV) is characterized by the polyp-like aneurysmal dilations of choroidal vascular networks and the presence of typical hyper-fluorescent nodules in the early phase of indocyanine green angiography (ICGA). Subretinal hemorrhage or fluid accumulation, persistent leakage, recurrently serous pigment epithelial detachment(PED) or serous neurosensory detachment are the clinical manifestations of PCV, and can cause serious and permanent vision loss. Age-related macular degeneration(AMD), as the leading cause of irreversible blindness in elderly people in the world, its prevalence among Asian populations aged 40 to 79 is estimated at 6.8% and is dramatically increasing year by year. As some common clinical manifestations and molecular genetic determinants have been identified between PCV and wet AMD(wAMD), most ophthalmologists originally used to recognize it as a phenotype of wAMD instead of take them as two separate clinical entities. With the development of diagnostic techniques in PCV, however, PCV has been more frequently distinguished as a differential diagnosis among generalized wAMD patients, especially in Asian populations while it is relatively lower in white patients. Based on the unique clinical characteristics of PCV which are apparently different from typical wAMD as well as distinct natural course and quite disparate response to the treatment like anti-vascular endothelial growth factor(VEGF), there have been warranted reasons to regard PCV as a distinct abnormality distinctly different from typical wAMD.The morbidity and clinical features of PCV is quite different in ethnicity and regionalism. When comparing with white populations, the proportion of PCV among generalized wAMD patients gets remarkably higher with an uprising tendency in pigmented populations, particularly in Asians, which shows representative clinical features as well. Nevertheless, no epidemiology or clinical study for PCV patients from Shandong province and surrounding regions has been reported in previous reports of Chinese PCV patients to date. We aim to investigate the demographic features and clinical manifestations of PCV patients from Shandong province and its surrounding regions, which may offer significant help in precise diagnosis and estimation of prognosis of PCV. ICGA, as the gold standard in the diagnosis of PCV, not only can define the position of the polypoidal lesion, but also evaluate its activity by the leakage of ICG According to the meticulously observation and the summary of the features of the polypoidal lesion on ICGA, we attempt to detect the new predictor for the evaluation of pathogenetic condition and the prognosis of PCV with an in-depth exploration.MethodsWe retrospectively reviewed the medical records of 246 eyes of 194 consecutive outpatients who visited the Department of Ophthalmology, Shandong Provincial Hospital affiliated to Shandong University with a preliminary diagnosis of generalized wAMD from 11 August 2011 to 28 January 2015. All the patients underwent routine eye examination, color fundus photograph (FP), fluorescein angiography (FFA) and indocyanine green angiography (ICGA). Analysed the patients that eventually diagnosed with PCV of their epidemiological features, FP, FFA and ICGA.Results246 eyes from 194 eligible patients were included in our study. Among them, 87(44.8%) patients were diagnosed with PCV and 107(55.2%) patients were diagnosed with typical AMD. In PCV patients, there was a male predominance with 50(57.5%) men and 37(42.5%) women, the average age was 66.4 (range, 41-92).20(23.0%) patients were bilaterally affected. Of the 107 eyes(87 patients) of PCV,70 eyes(65.4%) belonged to hemorrhagic PCV. Based on fundus examination, the commonest clinical fundus feature was hemorrhagic pigment epithelial detachment (PED) (55eyes,51.4%). This was followed by sub-retinal orange-red bulging lesions (49eyes,45.8%) and serous PED (25eyes,23.4%). Drusen was observed in 8(7.5%) eyes. Protracted gray scars were present in 3 (2.8%) eyes. In the 67 unaffected fellow eyes of patients with unilateral PCV,39 (58.2%) eyes are in normal situation. Atrophy in the macular area was shown in 10 (14.9%) patients. Drusen was shown in 8 (11.9%) patients. Macular scar was present in 4 (6.0%) patients. Sub-retinal hemorrhage and vitreous hemorrhage of other causes were seen in the rest of the unaffected eyes (6 eyes,9.0%) of PCV patients. On FFA,11 (10.3%) PCV cases presented minimally classic-type CNV lesions appeared by leakage presentation was visible. On ICGA, polyps with branching vascular networks (BVN) could be shown in 60 (56.1%) eyes. The location of the polyps were found in the macular, peripapillary, arcade and midperipheral area in 83 (77.6%) eyes,24 (22.4%) eyes,12 (11.2%) eyes and 3(2.8%) eyes, respectively. The average number of polypoidal lesions was 2.71±1.79 (ranged from 1 to 8). The mean area of the solitary polyp and the biggest one of multiple polyps was 0.52±0.35mm2,0.49±0.37mm2 respectively.ConclusionIn our study, among patients with PCV in Shandong and surrounding areas, we had found a predominance of male, unilateral, with hemorrhagic PED and sub-retinal orange-red bulging lesions as the most common manifestations which used to appear at the macular locations. On ICGA, the mean area of PCV with single polyp was larger than the biggest one of PCV with multiple polyps. The application of the number and the area of polyp lesion on ICGA as a prognostic predictor still needed to be further investigated in the evaluation of pathogenetic condition and the prognosis of PCV in the near future.