Pachychoroid disease: review and update

Eye, vol. 39, pp. 819–834

Abstract

The pachychoroid disease spectrum is a phenotype characterized by alterations in choroidal vasculature which result in outer retinal and choriocapillaris damage and visual loss. The presence of pachyvessels is one of the key features of the pachychoroid phenotype. Recent imaging studies suggest that pachyvessels may form because of choroidal venous congestion in one or more quadrants. The formation of intervortex anastomosis may function as a compensatory mechanism to dissipate the increased venous pressure, while outflow obstruction has been hypothesized to occur at the site of the vortex vein exiting the sclera. This review aims to summarize recent imaging findings and discuss evolution in the understanding of pathogenesis of the pachychoroid disease spectrum. We have summarized notable treatment trials in central serous chorioretinopathy and polypoidal choroidal vasculopathy and included an update of the current diagnostic and management strategies of the entities that are part of the pachychoroid disease spectrum.

Authors 10

1. Chui Ming Gemmy Cheung corresponding

   SingHealth · Singapore National Eye Center · SingHealth Duke-NUS Academic Medical Centre · Singapore Eye Research Institute · Duke-NUS Medical School

   Affiliation as printed

   Duke-NUS Medical School, Singapore, Singapore. Gemmy.cheung.c.m@singhealth.com.sg

   Singapore Eye Research Institute, Singapore, Singapore. Gemmy.cheung.c.m@singhealth.com.sg

   Singapore National Eye Centre, Singapore, Singapore. Gemmy.cheung.c.m@singhealth.com.sg

   Singapore National Eye Centre, Singapore, Singapore

2. Kunal K. Dansingani

   University of Pittsburgh Medical Center

   Affiliation as printed

   Department of Ophthalmology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

3. Hideki Koizumi

   University of the Ryukyus

   Affiliation as printed

   Department of Ophthalmology, Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan

4. Timothy Y. Y. Lai

   Chinese University of Hong Kong · Chinese University of Hong Kong, Shenzhen

   Affiliation as printed

   Department of Ophthalmology and Visual Sciences, The Chinese University of Hong Kong, Hong Kong, China

5. Sobha Sivaprasad

   Moorfields Eye Hospital NHS Foundation Trust · NIHR Moorfields Biomedical Research Centre

   Affiliation as printed

   Moorfields Clinical Research Facility, NIHR Biomedical Research Centre, Moorfields Eye Hospital NHS Foundation Trust, London, UK

6. Camiel J. F. Boon Aachen Department of Ophthalmology, Leiden University Medical Center

   Leiden University Medical Center · Amsterdam University Medical Centers

   Affiliation as printed

   Amsterdam University Medical Centers, Amsterdam, the Netherlands

   Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands

7. Elon H. C. van Dijk Aachen Department of Ophthalmology, Leiden University Medical Center

   Leiden University Medical Center

   Affiliation as printed

   Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands

8. Jay Kumar Chhablani

   University of Pittsburgh Medical Center

   Affiliation as printed

   Department of Ophthalmology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

9. Won Ki Lee

   Nune Eye Hospital

   Affiliation as printed

   Nune Eye Hospital, Seoul, South Korea

10. K. Bailey Freund

    Vitreous Retina Macula Consultants of New York · New York University

    Affiliation as printed

    Department of Ophthalmology, NYU Grossman School of Medicine, New York, NY, USA

    Vitreous Retina Macula Consultants of New York, New York, NY, USA