Retinal Neovascularization in Posterior Polar Annular Choroidal Dystrophy

Bommakanti, Nikhil; Besirli, Cagri G; Zhao, Peter

doi:10.1001/jamaophthalmol.2022.5269

Cited by 1 publication

(3 citation statements)

References 8 publications

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“…Bommakanti and colleagues reported a PPACD case followed up for over 5 years. During this period, the patient developed posterior subcapsular cataract, cystoid macular edema, and retinal neovascularization [8]. This instance indicates that the progression of PPACD might comprehend complications common to various retinal dystrophies, highlighting the need for diligent and longer follow-up and potentially revising prognostic expectations for this condition [2,8].…”

Section: Introductionmentioning

confidence: 92%

“…Full-field ERG in PPACD typically shows predominately diffused depressed responses, not solely restricted to the atrophic regions [3,6,8]. However, there is a diminished response in both scotopic and photopic a-wave and b-wave amplitudes, alongside a decrease in the 30 Hz flicker response, and oscillatory potentials with a prolongation of implicit time [1,6].…”

Section: Full-field Electroretinographymentioning

confidence: 99%

“…A possible variant of the disease known as posterior polar hemispheric choroidal dystrophy (PPHCD) is characterized by similar atrophic lesions spreading along a single retinal vascular arcade following a hemispheric configuration [3]. Long-term data are limited with only two cases reporting a follow-up with the patient for over five years [2,8]. The case reported by Sone and colleagues showed progression of fundus lesions and consequent visual field deterioration suggesting the possible progressive nature of the disease [2].…”

Section: Introductionmentioning

confidence: 99%

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Posterior Polar Annular Choroidal Dystrophy: Genetic Insights and Differential Diagnosis in Inherited Retinal Diseases

Ruggeri,

Ciancimino,

Guillot

et al. 2024

CIMB

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Posterior polar annular choroidal dystrophy (PPACD) is a rare ocular disorder and presents as symmetric degeneration of the retinal pigment epithelium (RPE) and the underlying choriocapillaris, encircling the retinal vascular arcades and optic disc. This condition distinctively preserves the foveal region, optic disc, and the outermost regions of the retina. Despite its distinct clinical presentation, due to the infrequency of its occurrence and the limited number of reported cases, the pathophysiology, and the genetic foundations of PPACD are still largely uncharted. This review aims to bridge this knowledge gap by investigating potential genetic contributors to PPACD, assessing current findings, and identifying genes that warrant further study. Emphasis is also placed on the crucial role of multimodal imaging in diagnosing PPACD, highlighting its importance in understanding disease pathophysiology. By analyzing existing case reports and drawing comparisons with similar retinal disorders, this paper endeavors to delineate the possible genetic correlations in PPACD, providing a foundation for future genetic research and the development of targeted diagnostic strategies.

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Section: Introductionmentioning

confidence: 92%