Polypoidal Vasculopathy

Polypoidal choroidal vasculopathy has been described a peculiar hemorrhagic disorder of the macula, posterior uveal bleeding syndrome, and multiple recurrent retinal pigment epithelium detachments in African-American women [26–29]. Yannuzzi et al. gave the disorder the name idiopathic polypoidal choroidal vasculopathy (IPCV). IPCV presents with multiple, recurrent, detachments of the RPE (serous and hemorrhagic), secondary to leakage and bleeding fromchoroidalvascularabnormalities.Classically thought of a distinct entity separate from neovascular AMD, some consider IPCV a subtype of AMD. Besides, similar macular appearance of the two entities may share common risk factors and even genetic abnormalities [30].

Pearl

Although more common in darkly pigmented individuals, IPCV is not only a disease of pigmented females. Consider the diagnosis in cases which lack drusen, have orange-red lesions with peripapillary location, and have multiple hemorrhagic PEDs with a prominent exudative component. Also consider the diagnosis in cases poorly responsive to anti-VEGF therapy.

Originally, IPCV was reported exclusively in females of darkly pigmented races, though recent reports include Caucasian males [31–33]. Female cases still outnumber male ones by a ratio of approximately 5:1. African Americans and Asians are at higher risk of developing IPCV [34]. It remains unclear if IPCV represents a variant of AMD within a different population, or if the conditions are unrelated. IPCV is usually diagnosed in patients who are between the ages of 50 and 65. The two entities can be difficult to distinguish and indocyanine green angiography (ICGA) is essential in making the diagnosis in difficult cases. An IPCV study group attempted to define the condition with a scheme based on fundus examination, ICG

findings, or both. ‘Definite’ cases were determined if at least one of the following criteria were met: Protruding elevated orange-red lesions observed

by fundus examination

Characteristic polypoidal lesions seen on ICGA “Probable” cases were determined if at least one

of the following criteria were met:

Only an abnormal vascular network is seen in ICG.

Recurrent hemorrhagic and/or serous detachments of the RPE were observed (Fig. 3.9) [35].

Although clinically IPCV may appear similar to AMD in that patients develop multiple RPE detachments, bilateral disease, IPCV is not linked to drusen, it appears reddish (as opposed to greygreen), and does not develop significant fibrous proliferation and disciform scarring as seen in late wet AMD [29, 34]. In contrast to AMD, patients with IPCV have a relapsing-remitting course with long-term preservation of good vision with 50% of eyes having had a favorable visual outcome at two years without treatment [34]. FA can assist in the diagnosis though ICG is the better diagnostic modality to differentiate this entity from AMD. ICGA better highlights the choroidal circulation due to its longer wavelength, which can penetrate the RPE. On ICGA, there is a cluster of leaking polypoidal choroidal vessels usually in the peripapillary area seen in the early phases.

Response to treatment also differs among patients with exudative AMD and IPCV. IPCV seems to have better responses to photodynamic therapy, but a poorer response to the anti-VEGF therapy [36].

Diagnosis

AMD is a clinical diagnosis made by the physician following a comprehensive examination of a patient. Patients with nonexudative (dry) AMD in one or both eyes or unilateral exudative AMD may have no complaints and may be detected on a routine ophthalmic exam. Symptomatic patients with dry AMD usually present with complaints of gradual dulling of vision, which may require brighter lights or magnifying lenses to help with

activities of daily living. Vision loss may be severe in cases of advanced dry AMD with central geographic or nongeographic atrophy. Despite having good central vision, patients with significant atrophy often have difficulty reading due to central and paracentral scotomas not enabling them to read an entire line of small print or even an entire letter of large print. They also may have difficulty seeing at night or in conditions of low lighting [37, 38]. Patients with wet AMD may complain of distorted vision with wavy lines, central or paracentral scotomas, rapid onset visual loss, or they may not notice any changes [39].

Diagnosis of AMD should include a detailed medical history to evaluate for predisposing conditions such as hypertension, smoking, and family history. This is not only important for the diagnosis of AMD, but also is useful in cases with a diagnostic dilemma to narrow the differential diagnosis. Dilated fundus examination should

be performed and a stereoscopic viewing method such as slit-lamp biomicroscopy with a contact or noncontact lens is essential.

The clinical hallmarks of AMD are drusen. Drusen can be easily recognizable as subretinal yellow material, though drusen may also be very subtle and overlooked. Although the finding of isolated small drusen with distinct borders (hard drusen) in an elderly patient is not worrisome to the clinician, the findings should be documented and followed. Isolated drusen do not account for significant vision loss [10]. Drusen are dynamic and can spontaneously disappear resulting in overlying atrophy of the RPE [40]. Clinically when drusen are either larger than 63 m(mu)m, associated with other changes such as RPE hyperand hypopigmentation, with vision loss below 20/30, or when soft drusen begin to coalesce, the dialogue should be started with the patients concerning the diagnosis of dry AMD. Macular changes in AMD