RETINA QUIZ

Uncertain "Something"
Confounds Diagnosis 


Mark T. Dunbar, O.D.


A 48-year-old black female of Haitian descent presented for an evaluation of blurred vision in her left eye. She was examined about 2 years earlier by a different doctor, who told her there was "something" in the left eye. She didn't recall specifically what the doctor had told her.

Her medical history was significant for hypercholesterolemia, which has been untreated. Best-corrected visual acuity was 20/20 O.D. and 20/30 O.S. Amsler grid testing was normal O.D., and showed slight central metamorphopsia O.S.

A dilated fundus exam of the right eye was normal. The left eye showed a clear vitreous. The optic nerves appeared healthy with a moderate-sized cup O.U. Of significance in the left eye was the presence of extensive exudate involving much of the posterior pole (figure 1). A pigment epithelial detachment (PED) was present superiorly with a ring of exudate surrounding it. Temporal to the macula, there was an area of scarring with more exudate extending inferiorly. The retinal vessels and the periphery were normal. Fluorescein angiography confirmed the PED superiorly in addition to showing scattered areas of focal leakage.

The left eye shows extensive exudate involving much of the posterior pole. An exudative ring surrounds a superior pigment epithelial detachment.


Quiz

  1. What is the cause of the reduced acuity in the left eye?
    a. Scarring.
    b. Ischemia.
    c. Edema.
    d. Amblyopia.

  • What is the etiology of all the exudate?
    a. Retinal telangiectasis.
    b. Choroidal neovascularization.
    c. Elevated cholesterol.
     d. Polyp-like choroidal vessels.

  • What is the correct diagnosis for this condition?
    a. Coats' syndrome.
    b. Resolving central retinal vein occlusion.
    c. Age-related macular degeneration
    d. Idiopathic polypoidal choroidal vasculopathy (IPCV).

  • What is the best management for this patient?
    a. Laser photocoagulation.
    b. Photodynamic therapy (PDT).
    c. Submacular surgery.
    d. Observation.

Get the Answers


Discussion
This patient has idiopathic polypoidal choroidal vasculopathy (IPCV), a condition that was first described as recently as 1982 by Lawrence Yannuzzi.1

Over the years, the pathogenesis of this condition has remained enigmatic. As a result, it has been classified by other names, including "posterior uveal bleeding syndrome" and "multiple serosanguineous retinal pigment epithelial detachment syndrome." IPCV was finally settled upon because the primary abnormality involved the choroidal circulation.

Also, the characteristic lesion is an inner choroidal vascular network of vessels ending in an aneurysmal bulge or outward projection. These vessels may appear clinically as a reddish-orange, spheroidal, polyp-like structure.

IPCV frequently has its origin from the peripapillary choroidal circulation, but polypoidal lesions arising from the macular choroidal circulation have been identified.2 Either form is associated with multiple, recurrent, serosanguineous detachments of the RPE and neurosensory retina, secondary to leakage and bleeding from these peculiar choroidal vascular lesions.

Additionally, vitreous hemorrhage and fibrous scarring may develop throughout the posterior pole as patients develop episodic leakage and bleeding. This patient manifests many of these characteristics. The extensive exudate probably results from leakage of the polypoidal vascular channels, which are subtle, but appear as a reddish-orange discoloration slightly superior within the PED and inferotemporal to the macula.

Also present is a serosanguineous PED superior to the macula, and retinal pigment epithelial scarring further temporal from previous leakage bouts. Macular edema in the form of retinal thickening from the exudate causes the reduced acuity.

IPCV is more common than you might think. As you'd expect, AMD is the most common misdiagnosis of IPCV. In one study, 13 patients (8%) of 167 consecutive patients diagnosed with AMD actually had IPCV.3

How do you make the distinction between AMD and IPCV?
IPCV tends to occur predominately in darker pigmented patients, typically ages 50-65.4 Initially, it was thought that IPCV occurred predominately in young black females, but we now know that this condition is not exclusive to blacks. It also occurs in Asians and Hispanics.

In fact, this predisposition for more pigmented races to develop IPCV contrasts greatly with AMD, which tends to develop more frequently in the less-pigmented elderly population. Based on this alone, the diagnosis of CNVM from AMD in a pigmented patient should arouse suspicion that the patient actually may have IPCV.

Interestingly, this patient also fits the demographic features that characterize this condition. There are also obvious funduscopic differences between AMD and IPCV. IPCV tends to be more unilateral, and although bilateral cases do occur, there is a marked absence of drusen.

The vascular lesions in IPCV lie within the choroid and usually are located in the peripapillary area. They can vary in size and often have tubular or polypoidal components. Often, these can be visible on fluorescein angiography; they pool with fluorescein but do not leak. Progression tends to occur based on enlargement of a focal process, rather than confluency of multicentric lesions. When a cluster of aneurysmal polypoidal vessels develops beneath a PED, it will appear as a reddish-orange subretinal mass.

Choroidal neovascularization in AMD is associated with drusen, and tends to grow from the choroid into the sub-RPE space. The vessels lack the polypoidal components you see with IPCV and tend to locate more in the macular or paramacular region. Fluorescein angiography exhibits this as late leakage of the dye.

Management

What is the best management of IPCV? We still don't understand the natural course of this disorder. For some patients who develop vision loss as a result of sersosanguineous detachments of the RPE and neurosensory retina, laser photocoagulation to the vessels has been successful. However, that success depends upon the extent and location of the IPCV.

A newer treatment that has received considerable attention for the treatment of CNVM associated with AMD is photodynamic therapy (PDT). PDT may be an ideal treatment for IPCV because the drug verteporfin has an affinity for vascular lesions.

In PDT, a non-thermal laser activates a dye (verteporfin) that collects within the vascular channels, resulting in platelet activation and subsequent thrombosis and occlusion of the polypoidal choroidal vessels within the treated area.

This treatment has not been studied extensively in IPCV; however, it may preserve or restore vision in these patients better than laser photocoagulation does.

Unfortunately for our patient, PDT was not an option because the FDA had not yet approved the treatment when we saw her. u


  1. Yannuzzi LA. Idiopathic polypoidal choroidal vasculopathy. Presented at the Macula Society Meeting; February 5, 1982; Miami.
  2. Moorthy RS, Lyon AT, Rabb MF, et al. Idiopathic polypoidal choroidal vasculopathy of the macula. Ophthalmology 1998;105:1380-1385.
  3. Yannuzzi LA, Wong DW, Sforzolini BS, et al. Polypoidal choroidal vasculopathy and neovascularized age-related macular degeneration. Arch Ophthalmol 1999;117(11):1503-1510.
  4. Yannuzzi LA, Ciardella A, Spaide RF, et al. The expanding clinical spectrum of idiopathic polypoidal choroidal vasculopathy. Arch Ophthalmol 1997;115:478-485.

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November 15, 2000
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