Delhi Journal of Ophthalmology

Pachychoroid Spectrum Disorders: A Review of Clinical Features and Management

Devesh Kumawat1, Amber Bhayana2, Vinod Kumar2
1Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, India
2Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Editor-in-Chief, Delhi Journal of Ophthalmology, Dr R.P.Centre, AIIMS.


Corresponding Author:

Vinod Kumar MS FRCS
Assistant Professor
Dr. Rajendra Prasad Centre for Ophthalmic Sciences,
All India Institute of Medical Sciences,
New Delhi - 110029, India
Email id: drvinod_agg@yahoo.com


Received: 07-SEP-2019 Accepted: 28-SEP-2019 Published Online: 03-DEC-2019
DOI: https://dx.doi.org/10.7869/djo.471

Abstract
Pachychoroid disorders are an entity distinct from age-related macular degeneration and constitute four major variants along a continuum- pachychoroid pigment epitheliopathy, central serous chorioretinopathy (CSCR), pachychoroid neovasculopathy (PNV), and polypoidal choroidal vasculopathy (PCV). The common characteristics include presence of dilated outer choroidal vessels (pachyvessels), choriocapillaris and retinal pigment epithelium (RPE) attenuation with or without increased choroidal thickness in a focal area or diffuse in topography. Whether choroidal congestion and hyperpermeability is the primary event with consequent choriocapillaris atrophy or choriocapillaris attenuation occurs primarily with resultant compensatory outer choroidal vascular dilatation, remains poorly understood. Chronic choroidal inflammation and ischemia predispose to angiogenic factor release which together with overlying RPE-Bruch’s disruption leads to type 1 choroidal neovascular membrane (CNV) formation. Pachychoroid disorders occur in younger population, does not have multiple soft drusen, and have distinct genetic polymorphisms and favourable treatment response when compared to the age-related macular degeneration. Systemic associations are known to occur with pachychoroid disorders. The treatment is required in cases of CSCR with chronic subretinal fluid and PNV or PCV with CNV and macular exudation. While acute cases of CSCR resolves spontaneously, diffuse epitheliopathy often requires photodynamic therapy or focal laser photocoagulation depending upon the site of RPE leakage. Pachychoroid disorders with exudation from CNV respond to three monthly intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections followed by pro-re-nata treatment. The presence of polyps and branched vascular network in PCV often requires photodynamic therapy instead or in addition to anti-VEGF therapy. Significant advances have occurred in the understanding of this novel spectrum with the help of indocyanine green angiography and optical coherence tomography angiography. However, there remain several areas of uncertainty including the pathogenesis and superiority of the various treatment approaches. Specific therapies aimed at the pathological changes occurring in the pachychoroid disease are the need of future.

Keywords :Pachychoroid, pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, multimodal imaging