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Idiopathic polypoidal choroidal vasculopathy in a patient with atrophic age related macular degeneration
  1. NOEMI LOIS
  1. Retina Service, Ophthalmology Department, Aberdeen Royal Infirmary, Foresterhill, Aberdeen, AB25 2ZN, Scotland, UK
  1. noemilois{at}aol.com

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Editor,—Since the initial description by Stern and colleagues in 1985,1 the clinical entity now known as idiopathic polypoidal choroidal vasculopathy (IPCV)2 has been increasingly recognised. Although it was initially described in black, middle aged, hypertensive women,1 it is now widely accepted that IPCV can affect men and women of any race, and may represent a significant proportion of patients with age related macular degeneration (AMD).34

In this report, the case of a patient with atrophic AMD and evidence of IPCV is presented. To my knowledge, the co-existence of IPCV and atrophic AMD has not been previously reported.

CASE REPORT

An 80 year old white woman presented complaining of sudden deterioration in vision in her left eye. Her ocular history was remarkable for atrophic AMD. Visual acuity was measured at 6/6 in the right eye and counting fingers in the left eye. On fundus examination of the right eye diffuse soft confluent drusen, some calcified, and geographic atrophy (GA) were detected (Fig 1A). In the left, the most striking feature was the presence of marked and diffuse cystoid macular oedema (CMO), and a serosanguineous pigment epithelial detachment (PED) associated with large amounts of hard exudates (Fig 1B). Soft and calcified drusen and GA were also present. Fluorescein angiography (FA) disclosed diffuse pooling of dye in the macula in the left eye, and an area of hyperfluorescence corresponding to the PED (Fig 2A). Window defects corresponding to areas of atrophy were detected in both eyes. On indocyanine green angiography (ICG) a choroidal vascular network of polypoidal structures was observed in the left eye (Fig 2B, C).

Figure 1

Fundus examination of both eyes disclosed soft confluent and calcified drusen and areas of geographic atrophy (A and B). In the left eye, a neurosensory detachment of the macula and a serosanguineous pigment epithelial detachment surrounded by extensive hard exudation were also present (B, arrowheads).

Figure 2

On fluorescein angiography pooling of dye at the macula was observed in late phases (A). An area of bright hyperfluorescence with late staining, corresponding to the serosanguineous pigment epithelial detachment, was also present (A). On indocyanine green angiography, a network of dilated channels terminating in aneurysmal dilations was observed in early frames of the angiogram (B), and leakage from this vascular network was detected in late frames (C). Laser photocoagulation was applied to cover the vascular network shown in B (inset). Following laser treatment, resolution of the neurosensory detachment of the macula and pigment epithelial detachment was noted on fundus examination and on fluorescein angiography (D). Closure of the polypoidal vessels was confirmed by indocyanine green angiography (E).

After informed consent was obtained, focal laser photocoagulation using an argon laser was applied to polypoidal vessels. The parameters used were a laser power of 200 mW, an exposure time of 0.2 seconds, and a spot size of 200 μm. This resulted in resolution of the CMO and PED (Fig 2D) on FA, closure of the choroidal vascular network on ICG (Fig2B, E), and on a subjective improvement in vision 2 weeks following laser treatment.

COMMENT

IPCV is characterised by the presence of recurrent serosanguineous PEDs and neurosensory retinal detachments (NSRD).1-5 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.2-5 Polypoidal lesions may arise from the peripapillary region,12 macula,5 or peripheral areas.4 Histopathological evaluation of a case 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.6 Although laser photocoagulation appears to be very effective in preserving visual acuity in patients with IPCV, spontaneous resolution of PEDs and NSRDs can also occur.5

The patient described in this report had evidence of atrophic AMD. However, the diagnosis of IPCV was suspected by the presence of a marked NSRD, extensive and diffuse hard exudates, and a serosanguineous PED. Since it was not clear whether GA was involving the fovea in the left eye, laser treatment was applied in an attempt to achieve resolution of subretinal fluid and hard exudates and in the hope that an associated visual improvement will occur. Rapid resolution of all subretinal fluid was noted and, more spectacularly, resolution of the serosanguineous PED, distantly located from the treated area, was also observed 2 weeks after laser treatment. Although no objective improvement in visual acuity was measured, the patient perceived a gain in vision after the treatment.

Although probably rare, IPCV can occur in patients with atrophic AMD. A high index of suspicion may be required to establish the diagnosis in these cases.

References

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