Journal of Ocular Biology
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Editorial
Multifocal Polypoidal Choroidal Vasculopathy in Caribbean and African- Americans
Eric M. Shrier*
Department of Opthalmology, SUNY-Downstate School of Medicine,
Brooklyn, NY, USA
*Address for Correspondence: Eric M. Shrier, Department of Opthalmology, SUNY Downstate Medical Center,
Brooklyn, NY 11203, USA, E-mail: emshrier@gmail.com
Submission: 28 October 2019;
Accepted: 11 November 2019;
Published: 14 November 2019
Copyright: © 2019 Shrier EM, et al. This is an open access article
distributed under the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium,
provided the original work is properly cited.
Editorial
Polypoidal Choroidal Vasculopathy (PCV) is a relatively
uncommon form of exudative Age-Related Macular Degeneration
(ARMD) first described in 1990 by Yannuzzi et al. [1]. It presents as
multiple, branching networks of small, polyp-like clusters, or larger
identifiable characteristic polyp-like out-pouchings. Manifestations
include lipid exudation, serosanguinous pigment epithelial
detachments and sub-retinal hemorrhage. Indocyanine-Green
(ICG) angiography has been said to be the necessary for delineating
the polypoidal vascular abnormalities, but recently hi-resolution
spectral domain Ocular Coherence Tomography (OCT) has been
demonstrated to perhaps be diagnostic as well [2]. Recently it has
been associated with a pachychoroid [3].
The vast majority of cases noted and reported on have involved
Asians (in whom it is a very common form of exudative AMD) and
Caucasians [4-8]. We have examined, imaged and treated a different
ethnic and underserved population of Caribbean and African-
Americans who display a different form of PCV. It is characterized by
its multifocal nature, prominent hypertrophic pigmentary scarring,
abundant circinate lipid exuation, and large polyps. Just two such cases
have been reported in the literature in 2016 [9], Both of which were
males. Furthermore, a thickened, pachychoroid is usually associated
with PCV. We reported a unique case of PCV ARMD in the setting
of drusen, and bilateral, chronic hypertrophically pigmented retinochoroidal
folds [10].
PCV genetic studies have been conducted in populations
of Asians and have found some putative linkage [11]. Since our
population is of a different ethnic background, and phenotype is so
different from that seen in Asians and Caucasians, I speculate that the
incident genotype may be diverse. It is unfortunate that this ethnic
group has been underrepresented in prior observational studies and
clinical trials, perhaps because of it rarity.
We are actively collating our cases (several hundred) that we
have seen in Brooklyn over the course of the last 15 years in hope
of highlighting the unique variant of PCV seen in male and female
Caribbean and African-Americans. They are distinctly different that
cases seen in Asians, which have been reported in abundance. The
polyps are often quite large and almost always multifocal over the
course of time. The late appearance is reminiscent of a white-dot
syndrome, Serpiginous Retinochoroidopathy. Some cases look like
a scarified version of Serpiginous, sometimes known pejoratively
as “Black Serpiginous” by some, due to the great abundance of
hypertrophic-black scarring of the retina. I do not think our PCV cases represent “Black” Serpiginous retinochoidopathy, as there
is no prior or coincident ocular inflammation. Our cases respond
well to anti-VEGF intravitreal injection, owing to their pathological
vasculogenic nature. It may come to pass that our observations will
lead to a better understanding of ethno-racial differences that exist in
PCV. We may ascertain new genetic markers. Further, its distinct
patterns may lead to a change in classification of PCV, and addition of
a category that may come to be known as Multifocal PCV. There are
some late-cases that are so striking that they may be aptly considered
as “Black” Polypoidal Choroidal Vasculopathy.